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BMC Anesthesiol. 2024 Oct 14;24(1):370. doi: 10.1186/s12871-024-02749-6.
ABSTRACT
OBJECTIVE: Currently, the primary surgical treatment for pseudomyxoma peritonei (PMP) is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). The perioperative period is frequently accompanied by severe pain. Erector spinae plane block (ESPB) can enhance analgesia for abdominal surgery. The purpose of this study was to compare the analgesic effects of bilateral multiple-injection ESPB in patients with PMP.
METHODS: Fifty patients with PMP were randomly divided into two groups: the ESPB combined with general anesthesia group (Group E) and the general anesthesia alone group (Group C). Prior to the induction, patients in Group E underwent ESPB at the T7 and T11 levels. The primary outcome was the visual analog scale (VAS) scores during rest at 6 h post-extubation. Secondary outcomes included intraoperative and postoperative opioid consumption, time for first rescue analgesia, frequency distribution of rescue analgesia, incidence of nausea and vomiting, adverse events associated with ESPB.
RESULTS: The Visual Analogue Scale (VAS) scores in Group E were significantly lower compared to Group C at immediate post-extubation (1.6 ± 0.9 vs. 2.4 ± 1.2, P = 0.008), and at 2 (1.9 ± 1.2 vs. 3.2 ± 1.1, P < 0.001), 4 (2.4 ± 1.5 vs. 3.7 ± 1.0, P = 0.001), and 6 h (2.7 ± 1.1 vs. 3.8 ± 1.4, P = 0.004) post-extubation during rest. Similarly, the VAS scores in Group E were significantly lower than those in Group C at immediate post-extubation (3.0 ± 1.4 vs. 4.6 ± 1.2, P < 0.001), and at 2 (3.8 ± 1.7 vs. 4.9 ± 1.4, P = 0.019), 4 (3.5 ± 1.3 vs. 5.3 ± 1.5, P < 0.001), and 6 h (3.9 ± 1.8 vs. 4.9 ± 1.3, P = 0.004) post-extubation during movement. In Group E, the intraoperative remifentanil administration (2319.3 ± 1089.5 vs. 2984.6 ± 796.1, P = 0.017) and the amount of rescue analgesia within 2 h post-extubation (0 vs. 4, P = 0.037) were significantly less than in Group C, and the first rescue analgesia time was shorter as well (231.4 ± 147.5 vs. 668.8 ± 416.7, P < 0.001).
CONCLUSION: Compared to general anesthesia alone, bilateral multiple-injection ESPB with 0.2% ropivacaine can enhance analgesia and reduce opioid administration in patients with PMP. However, the duration of analgesia with ESPB is relatively short due to the low concentration of the local anesthetic used.
TRIAL REGISTRATION: Chinese Clinical Trial Registry, ChiCTR2300069504, 20/03/2023.
PMID:39402455 | DOI:10.1186/s12871-024-02749-6
Genes Chromosomes Cancer. 2024 Oct;63(10):e23270. doi: 10.1002/gcc.23270.
ABSTRACT
Low-grade appendiceal mucinous neoplasia (LAMN) represents a relatively rare tumor of the appendix typically diagnosed incidentally through appendectomy for acute appendicitis. In cases where perforation occurs, mucinous content may disseminate into the abdominal cavity, leading to the development of pseudomyxoma peritonei (PMP). The primary objective of this study was to elucidate the molecular characteristics associated with various stages of LAMN and PMP. DNA was extracted from LAMN, primary PMPs, recurrent PMPs, and adenocarcinomas originating from LAMN. The subsequent analysis involved the examination of mutational hotspot regions within 50 cancer-related genes, covering over 2800 COSMIC mutations, utilizing amplicon-based next-generation sequencing (NGS). Our findings revealed activating somatic mutations within the MAPK-signaling pathway across all tumors examined. Specifically, 98.1% of cases showed mutations in KRAS, while one tumor harbored a BRAF mutation. Additionally, GNAS mutations were identified in 55.8% of tumors, with no significant difference observed between LAMN and PMP. While LAMN rarely displayed additional mutations, 42% of primary PMPs and 60% of recurrent PMPs showed additional mutations. Notably, both adenocarcinomas originating from LAMN showed mutations within TP53. Furthermore, 7.7% (4/52) of cases exhibited a potentially targetable KRAS G12C mutation. In four patients, NGS analysis was performed on both primary PMP and recurrent PMP/adenocarcinoma samples. While mutations in KRAS and GNAS were detected in almost all samples, 50% of recurrent cases displayed an additional SMAD4 mutation, suggesting a notable alteration during disease progression. Our findings indicate two key points: First, mutations within the MAPK pathway, particularly in KRAS, are evident across all tumors, along with a high frequency of GNAS mutations. Second, progression toward PMP or adenocarcinoma is associated with an accumulation of additional mutations within common oncogenic pathways.
PMID:39400480 | DOI:10.1002/gcc.23270
J Surg Oncol. 2024 Oct 13. doi: 10.1002/jso.27913. Online ahead of print.
ABSTRACT
Cytoreductive surgery with HIPEC has definite application to the management of selected patients with peritoneal metastases. Patients who profit most have a complete cytoreductive surgery. Higher-grade tumors such as colorectal cancer, gastric cancer, and ovarian malignancy are benefited by CRS and HIPEC only under limited circumstances. High-grade tumor invades subperitoneal lymphatics where HIPEC is not effective. Options to traditional HIPEC for treatment of invasive intraabdominal malignancies with peritoneal metastases must be explored.
PMID:39400331 | DOI:10.1002/jso.27913
Radiol Case Rep. 2024 Sep 28;19(12):6565-6573. doi: 10.1016/j.radcr.2024.08.158. eCollection 2024 Dec.
ABSTRACT
Appendiceal mucinous neoplasms, a rarity comprising less than 1% of all cancers, present intricate challenges in clinical management, and their incidence is on the rise. Notably, these neoplasms tend to metastasize intraperitoneally, leading to peritoneal carcinomatosis and concurrent accumulation of mucinous material, resulting in pseudomyxoma peritonei. Due to its spectrum of presentation, the classification of the appendiceal mucinous neoplasms remains a controversial subject with a range of management from a simple appendicectomy to a complex hyperthermic intraperitoneal chemotherapy (HIPEC). A 42-year-old Chadian male presented to the hospital with a sudden onset of right lower abdominal pain radiating to the inguinal region for 24 hours, associated with nausea and vomiting. The abdomen was distended and ascitic. Laboratory investigations revealed anemia, leukocytosis, hypernatremia, hypokalemia, elevated ESR, high CEA marker, and normal CA19-9. An abdominopelvic CT with contrast demonstrated extensive ascites and cystic masses in the liver, and pancreas with soft tissue thickening of the cecum; however, the appendix is not well-delineated. Patient was managed with chemotherapy and HIPEC followed by removal of all the affected parts. Nodules of the peritoneum and liver were submitted for histopathological analysis and a final diagnosis of pseudomyxoma peritonei of primary appendicular origin was established. This case highlights a case of extensive pseudomyxoma peritonei of appendicular origin managed aggressively by HIPEC and multiple resections of the involved organs. Prognosis of such a case is determined by the grade of the appendiceal tumor and the extent of invasion.
PMID:39391033 | PMC:PMC11465061 | DOI:10.1016/j.radcr.2024.08.158
Abdom Radiol (NY). 2024 Oct 5. doi: 10.1007/s00261-024-04595-z. Online ahead of print.
ABSTRACT
Peritoneal malignancies encompass a diverse range of tumors originating within the peritoneum, including primary tumors such as mesothelioma and primary serous peritoneal carcinoma or secondary tumors resulting from the spread of cancers from gastrointestinal, gynecological, and extra-abdominal sources. The traditional approach of palliative care for these malignancies is being replaced by a multimodal strategies that integrates surgery with systemic or intraperitoneal chemotherapy. Notably, cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy has shown significant improvements in survival rates. Imaging is crucial in the multidisciplinary management of these tumors, aiding in diagnosis, staging, restaging, and monitoring therapy response. It is also vital for appropriate patient selection, using the acronym "PAUSE", which involves assessing tumor burden via the peritoneal carcinomatosis index, evaluating patients pre- and post-therapy, detecting complications following therapy, and predicting treatment outcomes. This review explores the imaging manifestations of peritoneal malignancies, distinguishing them from various mimics, and underscores the importance of imaging modalities such as CT, MRI, PET/CT, and PET/MRI in effective decision-making and management.
PMID:39368001 | DOI:10.1007/s00261-024-04595-z
J Surg Oncol. 2024 Oct 3. doi: 10.1002/jso.27850. Online ahead of print.
ABSTRACT
BACKGROUND AND OBJECTIVES: Few preclinical models of pseudomyxoma peritonei (PMP) have been developed, probably due to the tumor's low incidence and its peculiar characteristics of slow growth. Therefore, there is a need to develop more refined PMP models that better reflect its characteristics. The aim of the study is to develop a culture strategy to generate organoid models derived from PMP patient samples.
METHODS: We followed a strategy based on combinatorial culture conditions that include the different factors essential for PMP growth and that mimic the microenvironment present in the patients.
RESULTS: We cultured PMP samples in the presence of the various factors produced by the niche environment of PMP. We obtained 12 PMP organoid models, each of which grows under specific culture conditions. PMP-derived organoids show long-term expansion capacity and reproduce the genetic landscape and histological phenotype of the tumor of origin.
CONCLUSION: The organoids we developed faithfully reproduce the key features of PMP disease and will allow us to understand the biology of PMP. With them, we will be able to identify key regulatory networks that support PMP progression, providing a platform for multilevel preclinical testing, identify novel diagnostic biomarkers, and generate novel targets for patient treatments.
PMID:39360464 | DOI:10.1002/jso.27850
Ann Surg Oncol. 2024 Sep 26. doi: 10.1245/s10434-024-16254-0. Online ahead of print.
ABSTRACT
BACKGROUND: International consensus on classifications of appendiceal mucinous neoplasms (AMNs) and associated pseudomyxoma peritonei (PMP) have been carefully made but clinicopathological associations supporting decision making remain scarce.
OBJECTIVE: This study aimed to assess interdependence between AMNs and PMP and provide directions for clinical management.
METHODS: This two-center retrospective cohort study reviewed patients with PMP treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy between 2005 and 2021. The primary objective was to reassess histopathologic grade of AMNs and PMP according to the Peritoneal Surface Oncology Group International classification and to establish its interdependence. Secondary outcomes were recurrence rate, PMP grade progression, ovarian involvement, and overall survival (OS).
RESULTS: Of 105 patients included, 78 (74.3%) had low-grade AMNs as the primary tumor, 8 (7.6%) had high-grade AMNs, 7 (6.7%) had mucinous adenocarcinoma (MAC), 1 (0.9%) had MAC with signet ring cells (SRC), and 11 (10.5%) had unidentified tumors. Overall, 11 patients (10.5%) had no PMP, 21 (20.0%) had acellular mucin, 56 (53.3%) had low-grade PMP, 12 (11.4%) had high-grade PMP, and 5 (4.8%) had PMP-SRC. In 11 cases (13.3%), AMNs and matching PMP grade differed. Over a 16-year follow-up, recurrence occurred in 31.8%, with three cases showing histopathologically changed PMP. Ovarian involvement was observed in 43/65 females (66.2%). Median OS was 13.8 years, and 5-year OS rates were 100%, 74.4%, 44.4%, and 20% for acellular mucin, low-grade PMP, high-grade PMP and PMP-SRC, respectively (p < 0.001).
CONCLUSIONS: AMN histology does not always reflects its associated PMP grade, while PMP grade strongly influences survival. Ovarian involvement and recurrent PMP showing unchanged histopathological features are common.
PMID:39327362 | DOI:10.1245/s10434-024-16254-0
J Obstet Gynaecol Res. 2024 Sep 25. doi: 10.1111/jog.16085. Online ahead of print.
ABSTRACT
BACKGROUND: The most common subtype of borderline ovarian tumors in Asia is mucinous borderline ovarian tumors (mBOTs). Intraoperative distinction from mucinous carcinoma can be difficult. Despite the indolent behavior of mBOTs, recurrence or metastases may occur. The objectives of this study were to determine the oncological outcomes of mBOTs and the risk factors for their recurrence.
RESULTS: This retrospective study enrolled patients with mBOTs treated or referred to our institution between January 2005 and December 2019. Histological reviews of the recurrent cases (primary and recurrent or metastatic tumors) were performed. Patients with other tumor subtypes, pseudomyxoma peritonei, or no in-house operation were excluded. Two hundred thirty-two patients were diagnosed with mBOTs. The median follow-up was 52 months. Six patients (2.58%) had tumor recurrence or metastasis. The risk factors for recurrence were a ruptured tumor, residual tumor after an operation, high serum CA19-9 level, and stage of the disease. The recurrence rates of fertility-sparing and radical surgery were not significantly different. Detailed surgical staging, intraepithelial carcinoma, and microinvasion were also not associated with disease recurrence.
CONCLUSIONS: mBOTs have an excellent prognosis. Currently, fertility-sparing surgery is the standard treatment, showing no significant difference in oncological outcomes compared to radical surgery. Patients with risk factors should be closely monitored.
PMID:39323179 | DOI:10.1111/jog.16085
Cureus. 2024 Aug 23;16(8):e67554. doi: 10.7759/cureus.67554. eCollection 2024 Aug.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a rare disease caused by primary mucinous neoplasms. Here, we describe a case where a large ovarian tumor was initially removed laparoscopically, followed by an appendectomy. The patient was diagnosed with PMP arising from an ovarian mucinous borderline tumor with a KRAS mutation. Treatment included bevacizumab-containing chemotherapy, resulting in complete remission.
PMID:39310419 | PMC:PMC11416708 | DOI:10.7759/cureus.67554
Gynecol Oncol Rep. 2024 Aug 18;55:101488. doi: 10.1016/j.gore.2024.101488. eCollection 2024 Oct.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is a clinical syndrome characterised by intraperitoneal accumulation of mucus due to mucinous neoplasia. It is a rare condition affecting 1-2 per million individuals per year. The majority of PMP arises from a ruptured mucinous appendiceal tumour, with infrequent occurrences from other primary gastrointestinal tumours and mucinous ovarian tumours. PMP arising from a mature ovarian teratoma is a rare entity, with limited case reports in the literature. Given the infrequent and sporadic occurrences of these tumours, little is known about the tumour behaviour and prognosis.
CASE SERIES AND LITERATURE REVIEW: Herein, we report six cases of PMP arising from a mature ovarian teratoma who were treated with primary cytoreductive surgery (CRS), with one case of recurrence. Literature review identified 21 cases from 12 manuscripts. Nineteen patients were treated with CRS alone, with two patients receiving adjuvant hyperthermic intraperitoneal chemotherapy (HIPEC). Follow up data were variably reported, with no recurrence in 20 patients during their follow up of 5-54 months. One patient reported to have died of disease at 49 months.
CONCLUSION: Despite the lack of high-quality evidence and limitations of small case series, our review indicates that close surveillance after CRS could be considered as the preferred treatment over more morbid CRS and HIPEC, with HIPEC reserved for patients who recur or progress after CRS.
PMID:39308901 | PMC:PMC11415951 | DOI:10.1016/j.gore.2024.101488
Int J Surg Case Rep. 2024 Oct;123:110329. doi: 10.1016/j.ijscr.2024.110329. Epub 2024 Sep 21.
ABSTRACT
INTRODUCTION: Mucinous appendiceal tumor is an exceptionally rare and indolent epithelial neoplasm characterized by the production of mucin within the appendix. Here we present a rare case of a large, asymptomatic mucinous appendiceal tumor discovered incidentally during repair of a paraumbilical hernia.
CASE PRESENTATION: A 73-year-old man with a complex medical history presented with epigastric pain, nausea, vomiting, and constipation. Diagnosed with a strangulated paraumbilical hernia. During hernia repair surgery, a low-grade mucinous appendiceal tumor was accidentally discovered. Post-operative monitoring over 18 months, including colonoscopy and CT scans, showed no recurrence.
DISCUSSION: Appendiceal primary tumors, though rare, can originate from neuroendocrine or epithelial cells. Epithelial tumors, including mucinous adenocarcinoma, produce mucin, potentially leading to pseudomyxoma peritonei, characterized by mucinous ascites and abdominal swelling. These tumors are often incidentally discovered during surgery, as symptoms are nonspecific, resembling acute appendicitis or causing abdominal distension. Diagnosis requires histopathology, revealing mucin accumulation and irregular glandular structures. Treatment typically involves cytoreductive surgery with hyperthermic intraperitoneal chemotherapy to manage the condition effectively.
CONCLUSION: This case emphasizes the critical need for intraoperative vigilance and histopathological analysis in detecting appendiceal mucinous tumors during abdominal surgery, ensuring accurate diagnosis and favorable outcomes.
PMID:39307026 | PMC:PMC11440772 | DOI:10.1016/j.ijscr.2024.110329
Clin Biochem. 2024 Sep 18;133-134:110827. doi: 10.1016/j.clinbiochem.2024.110827. Online ahead of print.
ABSTRACT
BACKGROUND AND AIMS: Pseudomyxoma peritonei (PMP) is a rare malignancy that lacks a highly sensitive and specific biomarker for its diagnosis. Identifying reliable serum markers is crucial for improving the diagnostic accuracy and management of PMP. This study aims to explore the diagnostic value of serum growth differentiation factor 15 (GDF-15) in patients with PMP.
MATERIAL AND METHODS: We carried on a 1:1 matched case-control study. 44 patients with PMP hospitalized in Aerospace Center Hospital were recruited as cases, and 44 sex- and age-matched apparently healthy participants were selected as controls. The serum GDF-15 concentrations were tested using an ELISA method. The diagnostic value of GDF-15 in PMP patients was assessed by receiver operating characteristic (ROC) curve analysis.
RESULTS: The median serum GDF-15 level in PMP patients was 1192.77 (843.03-1879.06) pg/mL, notably higher than that in healthy controls [533.27 (410.46-641.47) pg/mL] (P<0.001). The area under the curve (AUC) of serum GDF-15 for PMP diagnosis was 0.907, the optimal diagnostic threshold value was 644.58 pg/mL, the sensitivity was 93.18 %, and the specificity was 77.27 %. The AUC of GDF-15 combined with carbohydrate antigen 125 (CA125) was larger than that of GDF-15 alone (P=0.027), and the sensitivity and specificity achieved 86.36 % and 95.45 %. GDF-15 levels showed a significant correlation with age (P=0.042), with younger PMP patients exhibiting notably lower concentrations of GDF-15 compared to older patients.
CONCLUSION: Serum GDF-15 could become a new marker for the PMP diagnosis. The combination of GDF-15 and CA125 demonstrated superior diagnostic performance for PMP compared to GDF-15 alone, achieving a sensitivity of 86.36% and a specificity of 95.45%.
PMID:39304173 | DOI:10.1016/j.clinbiochem.2024.110827
Ann Surg Oncol. 2024 Sep 17. doi: 10.1245/s10434-024-16188-7. Online ahead of print.
NO ABSTRACT
PMID:39289246 | DOI:10.1245/s10434-024-16188-7
Diagn Microbiol Infect Dis. 2024 Aug 14;110(4):116496. doi: 10.1016/j.diagmicrobio.2024.116496. Online ahead of print.
ABSTRACT
We aimed to present a case of two mesocolonic hydatid cysts that mimicked the presentation of peritoneal pseudomyxoma. Hydatidosis is a zoonotic parasitic infection caused by the cestode Echinococcus spp., whose larval stage affects various organs. The present case describes a 40-year-old male patient who presented with severe lower abdominal pain and was diagnosed with acute appendicitis. The patient underwent an appendectomy and was later referred to an oncology surgery clinic because of imaging findings suggestive of peritoneal pseudomyxoma or carcinomatosis. A video-assisted laparoscopic procedure revealed two cysts and microscopic findings confirmed hydatid cysts. The patient was from a hydatidosis-endemic region of southern Brazil. This case highlights the diagnostic challenges and the need for a multidisciplinary approach and careful histopathological analysis in patients with complex abdominal conditions. This also demonstrates the importance of disseminating knowledge about this condition and its management.
PMID:39244842 | DOI:10.1016/j.diagmicrobio.2024.116496
Eur J Surg Oncol. 2024 Aug 14;50(12):108568. doi: 10.1016/j.ejso.2024.108568. Online ahead of print.
ABSTRACT
INTRODUCTION: To improve care for patients with colorectal peritoneal metastases (CRC-PM) or pseudomyxoma peritonei (PMP), the Dutch CRS-HIPEC quality registry was initiated in 2019. The aims are to describe the development and content of this registry and to give insight into the data collected during the first years.
MATERIALS AND METHODS: The registry is an observational cohort in the Netherlands. All patients with CRC-PM or PMP who intend to undergo cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) from 6 hospitals are included. Clinical data and outcomes (including hospital variation) were analyzed.
RESULTS: In 2019-2022, 889 patients were included in the CRS-HIPEC quality registry: 749 (84 %) with CRC-PM and 140 (16 %) with PMP. Peritoneal metastases were diagnosed synchronously in 51 % of CRC-PM patients and in 94 % of PMP patients. In patients undergoing complete CRS, the median peritoneal cancer index was 8 (IQR 4-13) for CRC-PM and 15 (IQR 6-26) for PMP. Complete cytoreduction was achieved in 639 CRC-PM patients (97 %) and 108 PMP patients (82 %). HIPEC was mainly performed with mitomycin C (CRC-PM: 94 %, PMP: 92 %). Major postoperative complications (Clavien-Dindo grade ≥3) occurred in 148 CRC-PM patients (22 %) and 30 PMP patients (23 %) with 90-day mortality rates of 2 %. In CRC-PM, differences between hospitals were observed regarding proportions of diagnostic laparoscopies/laparotomies, (neo)adjuvant treatment, ostomy formations and re-admissions.
CONCLUSION: The CRS-HIPEC quality registry provides insight into the outcomes of CRS-HIPEC and enables clinical auditing and observational cohort studies aiming to improve treatment outcomes for patients with CRC-PM and PMP.
PMID:39241538 | DOI:10.1016/j.ejso.2024.108568
Cureus. 2024 Aug 3;16(8):e66067. doi: 10.7759/cureus.66067. eCollection 2024 Aug.
ABSTRACT
Appendiceal mucocele is a rare disease that can sometimes mimic acute appendicitis or be discovered accidentally during surgeries. The clinical presentation of appendiceal mucocele is observed as lumen distension due to mucin accumulation. This condition has both benign and malignant underlying etiologies, which can be confirmed by histopathological examination. Acute presentation of appendiceal mucocele is rare and mostly resembles the symptoms of acute appendicitis. The treatment of appendiceal mucocele is crucial due to the risk of pseudomyxoma peritonei caused by the spread of mucus, mucocele perforation, or the presence of malignancy such as mucinous carcinoma. Surgical resection, either appendicectomy, typhlectomy, or sometimes right hemicolectomy, is the recommended management approach. This is a case of a 74-year-old male with pain in the abdomen as the major presenting complaint. He had a palpable right iliac fossa mass. The diagnosis of appendiceal mucocele was made by contrast-enhanced computed tomography, which was later confirmed by histopathology. The patient underwent surgical resection and was doing well at the three-month follow-up.
PMID:39229429 | PMC:PMC11368574 | DOI:10.7759/cureus.66067
Asian J Surg. 2024 Aug 29:S1015-9584(24)01829-3. doi: 10.1016/j.asjsur.2024.08.093. Online ahead of print.
NO ABSTRACT
PMID:39214806 | DOI:10.1016/j.asjsur.2024.08.093
Semin Diagn Pathol. 2024 Sep;41(5):213-221. doi: 10.1053/j.semdp.2024.08.005. Epub 2024 Aug 16.
ABSTRACT
Appendiceal adenocarcinoma (ApAC) is a rare malignancy, comprising less than 1 % of all gastrointestinal tumors. The current World Health Organization classifies ApAC as mucinous or nonmucinous. Mucinous ApAC are composed of pools of mucin lined by cells with low- and high-grade cytology and areas of infiltrative invasion. Nonmucinous ApAC histologically resemble conventional colorectal adenocarcinomas and have a worse prognosis than their mucinous counterpart. Unfortunately, the nomenclature and histologic classification of ApAC, specifically the mucinous subtype, has changed several times throughout the years, contributing to diagnostic confusion for pathologists. The treatment for mucinous ApAC differs from that of other appendiceal mucinous neoplasms, thus accurate diagnosis is key to patient management and outcome. This review discusses the current classification and staging of ApAC with a particular emphasis on the mucinous subtype and peritoneal disease, as these areas are the most challenging for practicing surgical pathologists.
PMID:39214725 | DOI:10.1053/j.semdp.2024.08.005
J Gastrointest Surg. 2024 Aug 28:S1091-255X(24)00579-1. doi: 10.1016/j.gassur.2024.08.010. Online ahead of print.
ABSTRACT
BACKGROUND: Low-grade appendiceal mucinous neoplasms (LAMNs) can progress to pseudomyxoma peritonei (PMP). The incidence and risk factors for recurrence are unclear, and there is a lack of consensus on the need and duration of surveillance imaging.
METHODS: Patients at the Mayo Clinic in Rochester, Minnesota, with a histologic diagnosis of LAMNs confined to the appendix and limited involvement of the right lower quadrant from 1992 to 2023 were included. Associations between recurrence and risk factors were assessed using Kaplan-Meier curves and Cox proportional hazards regression.
RESULTS: A total of 125 patients with LAMNs underwent abdominal imaging surveillance for a median of 51.2 months (IQR, 26-92). Of note, 5 patients (4%) recurred, all of which were PMP. Overall, the 5- and 10-year cumulative recurrence incidence rates were 3% and 6%, respectively. The median time to recurrence was 24 months (IQR, 23-87). Only LAMNs limited to the right lower quadrant and LAMN tumor size of <2 cm were associated with recurrence (P < .05). The 5- and 10-year cumulative recurrence risks were 12% and 30%, respectively, for the 21 patients with either risk factor. Only 1% of patients without these 2 risk factors developed a recurrence at 10 years.
CONCLUSION: The overall recurrence risk of LAMN after resection is low. Appendectomy is sufficient for LAMN. Select patients with acellular mucin confined to the right lower quadrant and a tumor size of <2 cm are at higher risk of recurrence. Thus, long-term surveillance is recommended for high-risk individuals. Conversely, LAMNs without risk factors can be safely observed expectantly.
PMID:39209560 | DOI:10.1016/j.gassur.2024.08.010
J Surg Oncol. 2024 Aug 29. doi: 10.1002/jso.27842. Online ahead of print.
ABSTRACT
While a rare entity, peritoneal pseudomyxoma treatment evolves. Decision-making criteria improve with imaging development and exploratory laparoscopy. Surgery remains at the core of the therapeutic strategy whatever disease progression. Complete cytoreduction plus hyperthermic intraperitoneal chemotherapy (HIPEC) is standard of care. Iterative cytoreduction or debulking is sometimes justified. Intraperitoneal chemotherapy modalities change with early postoperative HIPEC or pressurized intraperitoneal aerosol chemotherapy. Systemic or local treatment such as new chemo/immuno-therapies or BromAc should improve outcomes. Expertise and multicentric cooperation are more than ever needed.
PMID:39206531 | DOI:10.1002/jso.27842
Ann Surg Oncol. 2024 Nov;31(12):7978-7986. doi: 10.1245/s10434-024-16013-1. Epub 2024 Aug 27.
ABSTRACT
BACKGROUND: Decision regret is an emerging patient reported outcome. The aim of this study was to assess the incidence of regret in patients with appendiceal cancer (AC) who underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC).
PATIENTS AND METHODS: An anonymous survey was distributed to patients through the Appendix Cancer and Pseudomyxoma Peritonei (ACPMP) Research Foundation. The Decision Regret Scale (DRS) was employed, with DRS > 25 signifying regret. Patient demographics, tumor characteristics, postoperative outcomes, symptoms (FACT-C), and PROMIS-29 quality of life (QoL) scores were compared between patients who regretted or did not regret (NO-REG) the procedure.
RESULTS: A total of 122 patients were analyzed. The vast majority had no regret about undergoing CRS-HIPEC (85.2%); 18 patients expressed regret (14.8%). Patients with higher regret had: income ≤ $74,062 (72.2% vs 44.2% NO-REG; p = 0.028), major complications within 30 days of surgery (55.6% vs 15.4% NO-REG; p < 0.001), > 30 days hospital stay (38.9% vs 4.8% NO-REG; p < 0.001), a new ostomy (27.8% vs 7.7% NO-REG; p = 0.03), >1 CRS-HIPEC procedure (56.3% vs 12.6% NO-REG; p < 0.001). Patients with worse FACT-C scores had more regret (p < 0.001). PROMIS-29 QOL scores were universally worse in patients with regret. Multivariable analysis demonstrated > 30 days in the hospital, new ostomy and worse gastrointestinal symptom scores were significantly associated with regret.
CONCLUSIONS: The majority of patients with AC undergoing CRS-HIPEC do not regret undergoing the procedure. Lower income, postoperative complications, an ostomy, undergoing > 1 procedure, and with worse long-term gastrointestinal symptoms were associated with increased regret. Targeted perioperative psychological support and symptom management may assist to ameliorate regret.
PMID:39192009 | DOI:10.1245/s10434-024-16013-1
BMJ Case Rep. 2024 Aug 22;17(8):e260759. doi: 10.1136/bcr-2024-260759.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a rare neoplastic condition characterised by gelatinous ascites, which generally arise from mucin-producing appendiceal tumours. Presentation is variable but requires prompt recognition to ensure appropriate specialist management due to risk of malignancy.A male in his 40s presented with a 1-day history of sudden onset, non-migratory abdominal pain, worse in the right iliac fossa. He had no significant medical history nor known drug allergies. Examination revealed right iliac fossa peritonism and blood tests revealed raised inflammatory markers. CT scan showed a right-sided abdominal collection. Intraoperatively, a diagnostic laparoscopy was performed, which revealed extensive mucin in the abdominal cavity. This was washed out and a laparoscopic appendectomy was performed; histopathology confirmed PMP from the ruptured appendix.
PMID:39179264 | DOI:10.1136/bcr-2024-260759
Eur J Surg Oncol. 2024 Oct;50(10):108600. doi: 10.1016/j.ejso.2024.108600. Epub 2024 Aug 10.
ABSTRACT
INTRODUCTION: Low-grade appendiceal mucinous neoplasms (LAMNs) are classified as non-perforated (pTis, pT3) or perforated (pT4), and considered precursors of pseudomyxoma peritonei (PMP). This study aims to quantify the risk of developing PMP from pTis and pT3 LAMNs.
MATERIALS AND METHODS: Retrospective analysis of a prospectively collected database identified LAMN patients referred to a specialist centre from 2004 to 2019. pT4 LAMNs and other appendix tumours were excluded. All patients had specialist review of their pathology, operation note, and a CT scan (at least 6 weeks post-operatively). Surveillance CTs were then performed at 6, 12, 24, 36, 48, & 60 months, with tumour markers (CEA, CA19-9, CA125).
RESULTS: 193 pT3/pTis LAMN patients were included (pTis = 153, pT3 = 40). Median follow-up = 6.45 (3.91-22.13) years, M:F ratio = 1:1.57, and median age = 57 (23-83) years. Initial surgery included: appendicectomy (67 %), appendicectomy + visceral resection (6 %), and right hemicolectomy (27 %). R1 resections were identified in 5/193 patients (2.5 %). 3 R1 patients underwent re-operation (2 caecal pole excision and 1 ileocecectomy), none of which had residual tumour. 8/193 patients (4 %) were lost to follow up. None of the remaining 185 developed PMP.
CONCLUSION: This is the largest reported series of pTis/pT3 LAMNs with standardised follow-up in the literature. LAMNs correctly classified as pT3/pTis (after careful specialist review of pathology, operation note, and a baseline post-operative CT) have negligible risk of developing PMP and should have low intensity surveillance. If completely excised, further surgery is not indicated. R1 resections should be considered on an individual basis at a specialist centre.
PMID:39167862 | DOI:10.1016/j.ejso.2024.108600
Ann Surg Oncol. 2024 Aug 20. doi: 10.1245/s10434-024-16044-8. Online ahead of print.
NO ABSTRACT
PMID:39164601 | DOI:10.1245/s10434-024-16044-8
Surg Oncol. 2024 Oct;56:102123. doi: 10.1016/j.suronc.2024.102123. Epub 2024 Aug 17.
ABSTRACT
INTRODUCTION & OBJECTIVES: Treatment of PMP consists of appendectomy, cytoreductive surgery (CRS) and HIPEC. Right-sided hemicolectomy is necessary only when PMP is high grade, given the lymphatic invasion risk. To date, no single preoperative factor was identified as predictive of PMP grade.
MATERIALS & METHODS: Preoperative factors of a prospective cohort study on PMP were retrospectively analyzed, in order to identify situations linked with high or low grade appendiceal PMP. The main outcome was PMP grade on definitive histology after CRS.
RESULTS: n = 105. In univariate analysis, the grade of the appendiceal tumor, systematically reviewed in an expert center, showed an OR of 25.00 (95 % CI: 3.30-189.27; p = 0.001) and an NPV of 93.75 [85.36, 100]. Peritoneal biopsy demonstrated an OR of 19.80 (95 % CI: 2.30-170.71; p = 0.002) and a PPV of 90 [71.41, 100]. In multivariate analysis, these two factors remained significantly associated with PMP grade.
CONCLUSION: Whenever appendiceal tumor is low grade on preoperative histology, the colon has to be spared unless completeness of CRS is compromised, which is a high-grade feature in fact. In case of high grade appendiceal tumor and/or peritoneal biopsy, right-sided hemicolectomy is warranted. If no histology is available preoperatively, adapt to intraoperative lesions as no preoperative factors seem to be predictive.
PMID:39163796 | DOI:10.1016/j.suronc.2024.102123
Cureus. 2024 Jul 18;16(7):e64790. doi: 10.7759/cureus.64790. eCollection 2024 Jul.
ABSTRACT
A urachal remnant is a disorder resulting from a disturbance in the closure process of the urachus. A 55-year-old man was referred to our hospital for treatment of gallstones. The computed tomography scan revealed a cystic mass in the lower abdomen connecting to the urinary bladder. The preoperative diagnosis was a urachal cyst. Simultaneous laparoscopic cholecystectomy, mass resection, and urachectomy were performed. The mass on the cranial side of the urinary bladder was located on the median umbilical ligament. Both were resected and removed, along with the umbilicus. The postoperative course was uneventful. The histopathological diagnosis was urachal mucinous cystadenoma. There is no sign of a recurrence. A complete resection without damage is especially important for mucinous tumors of the urachal remnant because the injury to the tumor may lead to the development of pseudomyxoma peritonei. Only seven cases of mucinous cystadenoma of the urachal remnant were reported in English literature, and only one of these was treated with laparoscopic surgery. In our case, complete resection was possible by taking advantage of the magnifying effect of laparoscopic surgery. Furthermore, we are able to provide very clear intraoperative images and specimen photographs, which we believe will be useful for readers. Laparoscopic surgery will be beneficial when treating similar cases in the future. However, it should be kept in mind that a safe resection requires careful and meticulous technique.
PMID:39156387 | PMC:PMC11330288 | DOI:10.7759/cureus.64790
Int J Cancer. 2024 Aug 15. doi: 10.1002/ijc.35137. Online ahead of print.
ABSTRACT
Intrabdominal dissemination of malignant mesothelioma (MM) and pseudomyxoma peritonei (PMP) is poorly characterized with respect to the stemness window which malignant cells activate during their reshaping on the epithelial-mesenchymal (E/M) axis. To gain insights into stemness properties and their prognostic significance in these rarer forms of peritoneal metastases (PM), primary tumor cultures from 55 patients selected for cytoreductive surgery with hyperthermic intraperitoneal chemotherapy were analyzed for cancer stem cells (CSC) by aldehyde dehydrogenase 1 (ALDH1) and spheroid formation assays, and for expression of a set of plasticity-related genes to measure E/M transition (EMT) score. Intratumor heterogeneity was also analyzed. Samples from PM of colorectal cancer were included for comparison. Molecular data were confirmed using principal component and cluster analyses. Associations with survival were evaluated using Kaplan-Meier and Cox regression models. The activity of acetylsalicylic acid (ASA), a stemness modifier, was tested in five cultures. Significantly increased amounts of ALDH1bright-cells identified high-grade PMP, and discriminated solid masses from ascitic/mucin-embedded tumor cells in both forms of PM. Epithelial/early hybrid EMT scores and an early hybrid expression pattern correlated with pluripotency factors were significantly associated with early peritoneal progression (p = .0343 and p = .0339, respectively, log-rank test) in multivariable models. ASA impaired spheroid formation and increased cisplatin sensitivity in all five cultures. These data suggest that CSC subpopulations and hybrid E/M states may guide peritoneal spread of MM and PMP. Stemness could be exploited as targetable vulnerability to increase chemosensitivity and improve patient outcomes. Additional research is needed to confirm these preliminary data.
PMID:39146488 | DOI:10.1002/ijc.35137
Eur J Surg Oncol. 2024 Oct;50(10):108593. doi: 10.1016/j.ejso.2024.108593. Epub 2024 Aug 8.
ABSTRACT
BACKGROUND AND AIM: Two-stage cytoreductive surgery (CRS) has been proposed as an alternative to one-stage surgery in patients who have 'extensive' pseudomyxoma peritonei (PMP) and/or are unfit for very extensive surgery, to reduce morbidity. We review current evidence on two-stage CRS focusing on patient selection, interval between procedures, extent of surgery, use of HIPEC, perioperative and oncological outcomes.
METHODS: This is a narrative review. A literature search on PubMed and Embase was performed using keywords- 'Two-stage cytoreductive surgery', 'pseudomyxoma peritonei', 'high-volume PMP', 'huge PMP', 'cytoreductive surgery', 'HIPEC', 'staged surgery' and 'extensive pseudomyxoma peritonei'.
RESULTS: Five studies reported outcomes in a total of 114 patients. The indications for two-stage CRS were: in two studies, patients undergoing an incomplete cytoreduction due to undue surgical risk were reevaluated for a second surgery during routine surveillance; severe comorbidities in one; extensive disease with PCI>28 in another and in one, only HIPEC was performed as a second procedure due to intraoperative hemodynamic instability (the two-stage procedure was performed in interest of patient's safety). Major morbidity ranged from 0 to 37.5 % (first-stage) and 25%-38.9 % (second-stage). Short term follow-up demonstrated equivalent short-term oncological outcomes compared to historical data. Long term follow-up and quality-of-life data were not available.
CONCLUSIONS: The published studies showed different interpretations and applications of the two-stage CRS concept. The reported morbidity was similar to that after single-stage CRS for extensive PMP. Though short-term survival outcomes are acceptable, long-term follow-up is needed. Planned two-stage CRS should currently be reserved for highly selected clinical situations.
PMID:39121632 | DOI:10.1016/j.ejso.2024.108593
Case Rep Oncol Med. 2024 Aug 1;2024:3066063. doi: 10.1155/2024/3066063. eCollection 2024.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a well-known entity in gastrointestinal and ovarian tumors of mucinous histology. It has important implications on prognosis depending on whether seen in conjunction with a benign or a malignant tumor. In the current report, we describe a case of PMP in a case of advanced endocervical adenocarcinoma of the cervix which was managed surgically.
PMID:39119196 | PMC:PMC11309810 | DOI:10.1155/2024/3066063
Ann Surg Oncol. 2024 Aug 8. doi: 10.1245/s10434-024-15880-y. Online ahead of print.
ABSTRACT
BACKGROUND: The role of gastrectomy to achieve complete cytoreduction (CCR) for pseudomyxoma peritonei (PMP) is controversial due to uncertain risk/benefit ratio. The outcomes of patients who gastrectomy over a twenty-year period in a high-volume unit are reported.
METHODS: All patients requiring gastrectomy to achieve CCR for appendiceal PMP between 2000 and 2020 were reviewed. Demographics, disease, operative, complication, and survival data were analysed. The first and second decades were compared.
RESULTS: A total of 2148 patients underwent CRS and HIPEC, of which 78% had CCR. Gastrectomy was performed in 7.1%. Median age was 55 years, and 52% were female. Among gastrectomy patients, 94.2% had ≥1 elevated tumour marker, and 18% had high-grade disease. Median PCI was 30, and 30% required subtotal colectomy. Clavien-Dindo III-IV complications occurred in 32%, and 90-day mortality was 1.75%. Median survival was 104 months, and 10-year OS was 47%. Comparing the two decades, total CRS cases almost tripled, with a greater proportion achieving CCR (82.2% vs. 67.8%) but fewer requiring gastrectomy (5.3% vs. 13.5%). In those who had gastrectomy, disease was more advanced (higher PCI, more high-grade disease, more colectomies) in the later period. However, on multivariable analysis, there was no difference in survival between decades. High-grade histology was the only predictor of survival.
CONCLUSIONS: Gastrectomy can achieve good long-term survival with low mortality and acceptable morbidity and should not deter surgeons from achieving CCR. However, increasing experience shows CCR can be achieved, preserving the stomach in the majority of cases through careful consideration of the anatomy and gastric blood supply.
PMID:39115650 | DOI:10.1245/s10434-024-15880-y
Cureus. 2024 Jun 29;16(6):e63435. doi: 10.7759/cureus.63435. eCollection 2024 Jun.
ABSTRACT
Mucin-secreting adenocarcinoma of the appendix is a very rare, slow-growing, mucin-producing epithelial neoplasm of the appendix. It is usually found accidentally in an appendicectomy specimen with the presentation of acute appendicitis in most patients or when there is a rupture of the primary tumor with the mucin spreading along with the tumor cells in the entire peritoneal cavity. Here we describe a case of low-grade (well-differentiated) mucin-secreting adenocarcinoma in the appendix. A 48-year-old female presented with complaints of abdominal distension with no other complaints of fever, pain, or breathlessness. Carcinoembryonic antigen levels were 44.8 ng/mL. Cytoreduction surgery of bilateral ovaries was done. The final histopathological diagnosis was reported as low-grade (well-differentiated) mucin-secreting adenocarcinoma of the appendix staged at pT4b pNx pM1c. Pseudomyxoma peritonei is a very feared complication and also, at times, the only presenting symptom where there is an accumulation of mucin in the intra-abdominal cavity due to the spread of mucin-secreting cells, which in turn causes an increase in the abdominal girth along with discomfort for the patient. The mainstay of treatment remains cytoreductive surgery along with hyperthermic intraperitoneal chemotherapy.
PMID:39077258 | PMC:PMC11285657 | DOI:10.7759/cureus.63435
Cureus. 2024 Jun 28;16(6):e63426. doi: 10.7759/cureus.63426. eCollection 2024 Jun.
ABSTRACT
Although pseudomyxoma peritonei (PMP) classically presents with profuse mucinous ascites within the peritoneal cavity, the physical manifestations of this disease exist on a spectrum, with the possibility of milder forms that lack typical findings. The authors report an indolent case of PMP diagnosed incidentally during workup and treatment for chronic cholecystitis in a 43-year-old male. This presentation of PMP was atypical due to a lack of discernible symptoms as well as uncharacteristic intraoperative findings consisting of numerous omental and pelvic adhesions with only sparse mucinous deposits. This case contributes to the growing understanding of PMP by exploring an uncharacteristic presentation of the disease with the hope that it may assist clinicians in diagnosing those cases of PMP that are more indolent and insidious in nature.
PMID:39077246 | PMC:PMC11284341 | DOI:10.7759/cureus.63426
BMJ Case Rep. 2024 Jul 22;17(7):e258364. doi: 10.1136/bcr-2023-258364.
ABSTRACT
Low-grade appendiceal mucinous neoplasm (LAMN) may culminate as a mucin-secreting disease known as pseudomyxoma peritonei (PMP). Once the diagnosis of LAMN and PMP is made, cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC) are indicated.Herein, we present a female patient in her 50s who was diagnosed with an ovarian mass for which she underwent laparoscopic oophorectomy. As the pathology of the ovary showed a tumour of gastrointestinal origin, she then underwent CRS and HIPEC with a final pathology of LAMN. Six weeks later, a mucinous lesion confined to the abdominal wall was detected on a postoperative CT. Suspected for port-site metastasis at the laparoscopic trocar site, we treated this lesion using the same principles of treatment as the intra-abdominal disease. The abdominal wall mass was surgically resected, and the cavity created was irrigated with mitomycin C. On 30 months of follow-up, the patient had no evidence of disease.
PMID:39038876 | DOI:10.1136/bcr-2023-258364
Cancer Biol Med. 2024 Jul 18;21(7):586-605. doi: 10.20892/j.issn.2095-3941.2024.0109.
ABSTRACT
Pseudomyxoma peritonei (PMP) is an indolent malignant syndrome. The standard treatment for PMP is cytoreductive surgery combined with intraperitoneal hyperthermic chemotherapy (CRS + HIPEC). However, the high recurrence rate and latent clinical symptoms and signs are major obstacles to further improving clinical outcomes. Moreover, patients in advanced stages receive little benefit from CRS + HIPEC due to widespread intraperitoneal metastases. Another challenge in PMP treatment involves the progressive sclerosis of PMP cell-secreted mucus, which is often increased due to activating mutations in the gene coding for guanine nucleotide-binding protein alpha subunit (GNAS). Consequently, the development of other PMP therapies is urgently needed. Several immune-related therapies have shown promise, including the use of bacterium-derived non-specific immunogenic agents, radio-immunotherapeutic agents, and tumor cell-derived neoantigens, but a well-recognized immunotherapy has not been established. In this review the roles of GNAS mutations in the promotion of mucin secretion and disease development are discussed. In addition, the immunologic features of the PMP microenvironment and immune-associated treatments are discussed to summarize the current understanding of key features of the disease and to facilitate the development of immunotherapies.
PMID:39026438 | PMC:PMC11271218 | DOI:10.20892/j.issn.2095-3941.2024.0109
J Cancer Res Ther. 2024 Apr 1;20(3):840-843. doi: 10.4103/jcrt.jcrt_149_22. Epub 2023 Sep 21.
ABSTRACT
BACKGROUND: Low-grade appendiceal mucinous neoplasms (LAMNs) are benign non-invasive epithelial proliferations of the appendix. These usually present clinically as mucoceles and these rarely exceed 2 cm in diameter. Lesions confined to the lumen are labelled as LAMN; however those in which mucin spreads outside the peritoneum are labeled as pseudomyxoma peritonei (PMP).
AIMS AND OBJECTIVE: A retrospective study was conducted over a period of three years and all cases of appendectomies were studied. Twelve cases of LAMN were identified, which is a diagnostic dilemma for the pathologists and clinicians.
RESULTS: LAMN was identified based on the histopathological features. Out of the 12 cases, 9 were classified as LAMN and 3 as appendiceal neoplasm with PMP. There was villous or flat proliferation of epithelial lining, loss lymphoid aggregates, and dissecting mucin within muscularis.
CONCLUSION: LAMNs are rare neoplasms of the appendix, with clinical presentation similar to acute appendicitis. Mucinous collections within the appendiceal wall should be extensively searched for mucosal changes and, if found, should prompt a careful search for pushing invasion of LAMNs. A thorough and vigilant gross examination can be of great help. Appendicectomy is the treatment of benign and grossly intact mucinous neoplasm.
PMID:39023592 | DOI:10.4103/jcrt.jcrt_149_22
Clin Cancer Res. 2024 Sep 13;30(18):4082-4099. doi: 10.1158/1078-0432.CCR-23-4072.
ABSTRACT
PURPOSE: Pseudomyxoma peritonei (PMP) is a rare and poorly understood malignant condition characterized by the accumulation of intra-abdominal mucin produced from peritoneal metastases. Currently, cytoreductive surgery remains the mainstay of treatment but disease recurrence and death after relapse frequently occur in patients with PMP. New therapeutic strategies are therefore urgently needed for these patients.
EXPERIMENTAL DESIGN: A total of 120 PMP samples from 50 patients were processed to generate a collection of 50 patient-derived organoid (PDO) and xenograft (PDX) models. Whole exome sequencing, immunohistochemistry analyses, and in vitro and in vivo drug efficacy studies were performed.
RESULTS: In this study, we have generated a collection of PMP preclinical models and identified druggable targets, including BRAFV600E, KRASG12C, and KRASG12D, that could also be detected in intra-abdominal mucin biopsies of patients with PMP using droplet digital PCR. Preclinical models preserved the histopathological markers from the original patient sample. The BRAFV600E inhibitor encorafenib reduced cell viability of BRAFV600E PMP-PDO models. Proof-of-concept in vivo experiments showed that a systemic treatment with encorafenib significantly reduced tumor growth and prolonged survival in subcutaneous and orthotopic BRAFV600E-PMP-PDX mouse models.
CONCLUSIONS: Our study demonstrates for the first time that systemic targeted therapies can effectively control PMP tumors. BRAF signaling pathway inhibition represents a new therapeutic opportunity for patients with BRAFV600E PMP who have a poor prognosis. Importantly, our present data and collection of preclinical models pave the way for evaluating the efficacy of other systemic targeted therapies toward extending the promise of precision oncology to patients with PMP.
PMID:39018564 | PMC:PMC11393541 | DOI:10.1158/1078-0432.CCR-23-4072
Gan To Kagaku Ryoho. 2024 Jun;51(6):663-665.
ABSTRACT
A 46-year-old female presented persistent right lower abdominal pain for 4 days. Computed tomography revealed an enlarged appendix with a surrounding low-attenuation mass. The patient was diagnosed with appendiceal abscess-forming appendicitis and initially treated with antibiotics. However, owing to the manifestation of nausea as a side effect, laparoscopic appendectomy was performed 3 days after the initial consultation. Intraoperative examination revealed mucinous material on the surface of the appendix and within the abdominal cavity, leading to the decision to perform an appendectomy with partial cecum resection and excision of the omentum with mucinous deposits. Pathological examination confirmed the diagnosis of a perforating low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei. The patient was subsequently referred to a specialized center for ongoing management, and at 9 months postoperatively, surveillance is being conducted. Low-grade appendiceal mucinous neoplasms can progress to pseudomyxoma peritonei through perforation; however, an optimal treatment approach has not yet been established. In particular, patients in advanced stages of the disease often require challenging management decisions. This case is reported along with a review of the literature to provide further guidance.
PMID:39009527
Ann Surg Oncol. 2024 Sep;31(9):6262-6273. doi: 10.1245/s10434-024-15646-6. Epub 2024 Jul 15.
ABSTRACT
BACKGROUND: The combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) constitutes the established standard of care for pseudomyxoma peritonei patients. However, the role of HIPEC lacks validation through randomized trials, leading to diverse proposed treatment protocols. This consensus seeks to standardize HIPEC regimens and identify research priorities for enhanced clarity.
METHODS: The steering committee applied the patient, intervention, comparator, and outcome method to formulate crucial clinical questions. Evaluation of evidence followed the Grading of Recommendations, Assessment, Development, and Evaluation system. Consensus on HIPEC regimens and research priorities was sought through a two-round Delphi process involving international experts.
RESULTS: Out of 90 eligible panelists, 71 (79%) participated in both Delphi rounds, resulting in a consensus on six out of seven questions related to HIPEC regimens. An overwhelming 84% positive consensus favored combining HIPEC with CRS, while a 70% weak positive consensus supported HIPEC after incomplete CRS. Specific HIPEC regimens also gained consensus, with 53% supporting Oxaliplatin 200 mg/m2 and 51% favoring the combination of cisplatin (CDDP) associated with mitomycin-C (MMC). High-dose MMC regimens received an 89% positive recommendation. In terms of research priorities, 61% of panelists highlighted the importance of studies comparing HIPEC regimens post CRS. The preferred regimens for such studies were the combination of CDDP/MMC and high-dose MMC.
CONCLUSIONS: The consensus recommends the application of HIPEC following CRS based on the available evidence. The combination of CDDP/MMC and high-dose MMC regimens are endorsed for both current clinical practice and future research efforts.
PMID:39008204 | DOI:10.1245/s10434-024-15646-6
North Clin Istanb. 2024 Jun 28;11(3):261-268. doi: 10.14744/nci.2023.50374. eCollection 2024.
ABSTRACT
Pseudomyxoma peritonei is a rare pathological condition characterized by mucinous tumor tissue implants on the peritoneal surface. Although the cause of Pseudomyxoma peritonei has been extensively studied, the prevailing agreement is that it stems from mucinous tumors that occur in the ovaries or appendix. The tumor tissue typically remains localized to the peritoneum and does not exhibit extraperitoneal spread. Patients with Pseudomyxoma peritonei may present with symptoms such as abdominal pain, bloating, loss of appetite, and shortness of breath. Computerized Tomography is commonly used for diagnostic purposes. The treatment of Pseudomyxoma peritonei typically involves surgical evacuation of the tumoral tissue, followed by cytoreduction and Hyperthermic Intraperitoneal Chemotherapy. While effective treatment options are available, some patients may require repeated surgeries over an extended period. This paper reports on a case study of a patient with a history of recurrent Pseudomyxoma peritonei, necessitating multiple surgical interventions over a decade. The paper concludes with a review of the relevant literature.
PMID:39005750 | PMC:PMC11237824 | DOI:10.14744/nci.2023.50374
Cancers (Basel). 2024 Jul 2;16(13):2441. doi: 10.3390/cancers16132441.
ABSTRACT
Appendiceal tumors are uncommon and, at times, discovered incidentally during histological examination. The histopathological classification of the disease is complex and has generated some controversy. The analysis of circulating tumor cells can be used for the early detection of metastatic potential. The aim of the present study was to examine the prognostic value of circulating tumor cells in patients with appendiceal tumors and peritoneal metastases. To our knowledge, this is the first study to examine CTCs in appendiceal tumors. We performed a prospective cohort study of consecutive patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy between 2015 and 2019 at a HIPEC referral center. In total, 31 patients were included in the analysis, and circulating tumor cells were detected in 15 patients (48%). CTC positivity was not associated with overall or recurrence-free survival, nor was it correlated with PCI score or histopathological grading. Surprisingly, however, CTCs were found in almost half the patients. The presence or quantities of these cells did not, on their own, predict systemic metastatic potential during the observed time, and they did not appear to significantly correlate with the oncological outcomes recorded.
PMID:39001503 | PMC:PMC11240500 | DOI:10.3390/cancers16132441
Ann Surg Oncol. 2024 Aug;31(8):5377-5389. doi: 10.1245/s10434-024-15355-0. Epub 2024 May 4.
ABSTRACT
INTRODUCTION: Appendiceal cancer (AC) excessive mucin production is a barrier to heated intraperitoneal chemotherapy (HIPEC) drug delivery. Bromelain is a pineapple stem extract with mucolytic properties. We explored bromelain treatment effects against mucinous AC in a patient-derived tumor organoid (PTO) model and an AC cell line.
PATIENTS AND METHODS: PTOs were fabricated from tumor specimens obtained from patients with AC undergoing cytoreductive surgery with HIPEC. PTOs underwent HIPEC treatment with bromelain, cisplatin, and mitomycin C (MMC) at 37 °C and 42 °C with and without bromelain pretreatment.
RESULTS: From October 2020 to May 2023, 16 specimens were collected from 13 patients with low-grade (12/16, 75%) and high-grade AC (4/16, 25%). The mucin-depleting effects of bromelain were most significant in combination with N-acetylcysteine (NAC) compared with bromelain (47% versus 10%, p = 0.0009) or NAC alone (47% versus 12.8%, p = 0.0027). Bromelain demonstrated > 31% organoid viability reduction at 60 min (p < 0.001) and > 66% in 48 h (p < 0.0001). Pretreatment with bromelain increased cytotoxicity of both cisplatin and MMC HIPEC conditions by 31.6% (p = 0.0001) and 35.5% (p = 0.0001), respectively. Ki67, CK20, and MUC2 expression decreased after bromelain treatment; while increased caspase 3/7 activity and decreased Bcl-2 (p = 0.009) and Bcl-xL (p = 0.01) suggest induction of apoptosis pathways. Furthermore, autophagy proteins LC3A/B I (p < 0.03) and II (p < 0.031) were increased; while ATG7 (p < 0.01), ATG 12 (p < 0.04), and Becline 1(p < 0.03), expression decreased in bromelain-treated PTOs.
CONCLUSIONS: Bromelain demonstrates cytotoxicity and mucolytic activity against appendiceal cancer organoids. As a pretreatment agent, it potentiates the cytotoxicity of multiple HIPEC regimens, potentially mediated through programmed cell death and autophagy.
PMID:38704503 | PMC:PMC11236891 | DOI:10.1245/s10434-024-15355-0
World J Gastrointest Surg. 2024 Jun 27;16(6):1894-1909. doi: 10.4240/wjgs.v16.i6.1894.
ABSTRACT
BACKGROUND: Despite the rarity of appendiceal tumors, research in this field has intensified, resulting in a growing number of studies and published papers. Surprisingly, no comprehensive bibliometric analysis has specifically addressed appendiceal tumors.
AIM: To offer a thorough analysis of the current landscape and future trends in appendiceal tumor research.
METHODS: In our bibliometric analysis studies, we explored the Web of Science Core Collection database. The bibliographic details of the chosen publications were automatically converted and analyzed using the bibliometric package in the R environment. Additionally, we employed VoSviewer to create cooperation network maps for countries, institutions, and authors, as well as clustering maps for keywords. Furthermore, CiteSpace, another software tool, was utilized to build dual-map overlays of journals and analyze references with citation bursts.
RESULTS: Our study included 780 English-language articles published after 2010. The number of related publications and citations has increased in the past decade. The United States leads in this area, but there is a need to improve cooperation and communication among countries and institutions. Co-occurrence analysis also revealed close collaboration among different authors. Annals of Surgical Oncology was the most influential journal in this field. Analysis of references with high co-citations and references with citation bursts, consistent with analysis of keywords and hotspots, indicated that current research primarily centers on the classification and management of appendiceal mucinous neoplasms and consequent pseudomyxoma peritonei. Despite the abundance of clinical studies, a greater number of in-depth basic research studies should be conducted.
CONCLUSION: Current research on appendiceal tumors focuses on classification and management of appendiceal mucinous neoplasms and pseudomyxoma peritonei. Enhanced collaboration and basic research are vital for further advancement.
PMID:38983319 | PMC:PMC11230007 | DOI:10.4240/wjgs.v16.i6.1894
Cureus. 2024 Jun 8;16(6):e61945. doi: 10.7759/cureus.61945. eCollection 2024 Jun.
ABSTRACT
Appendiceal mucinous neoplasms are rare and can be easily misdiagnosed as adnexal masses. Fertility is a concern in cases requiring cytoreductive surgery involving the ovaries and if hyperthermic intraperitoneal chemotherapy is considered. We present the case of a 35-year-old patient with primary infertility who was suspected to have a hematosalpinx on ultrasonography and magnetic resonance imaging (MRI) but was found to have an appendiceal mucinous neoplasm on laparoscopy. Fertility preservation was offered to this patient. Appendiceal mucinous neoplasms should be considered in the differential diagnosis of patients in their reproductive years presenting with adnexal masses. Fertility preservation should be discussed with these patients, especially when gonadotoxic treatments are planned.
PMID:38978911 | PMC:PMC11230616 | DOI:10.7759/cureus.61945
J Coll Physicians Surg Pak. 2024 Jul;34(7):790-794. doi: 10.29271/jcpsp.2024.07.790.
ABSTRACT
OBJECTIVE: To investigate the factors contributing to the blockage of perfusion tubes during hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with appendiceal pseudomyxoma peritonei (PMP) undergoing combined cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy treatment, and to construct a nomogram for predicting the risk of tube occlusion.
STUDY DESIGN: Observational study. Place and Duration of the Study: Department of Gastrointestinal Surgery, Central Hospital Affiliated to Shandong First Medical University, Jinan, China, from June 2017 to December 2023.
METHODOLOGY: Tube occlusion was defined as the inability to achieve 30 minutes of continuous unobstructed perfusion. Statistical methods such as univariate analysis, multivariate analysis, and Lasso regression were employed for data analysis.
RESULTS: The results revealed that 27% of the 383 hyperthermic intraperitoneal chemotherapy perfusion treatments resulted in the tube occlusion events. Multivariate logistic regression analysis identified age, CA-125, CA19-9, and pathological type as the independent risk factors. A nomogram predicting the tube occlusion was constructed and validated for its predictive accuracy and clinical utility.
CONCLUSION: This study successfully developed a nomogram to predict the tube occlusion risk during cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy treatment for patients with pseudomyxoma peritonei, providing valuable guidance for clinical practice and aiding in personalised treatment decisions to improve patient prognosis. However, further research is needed to validate the reliability and clinical applicability of the model, as well as to investigate the impact of tube occlusion on treatment outcomes and corresponding management strategies.
KEY WORDS: Pseudomyxoma peritonei, Cytoreductive surgery, Hyperthermic intraperitoneal chemotherapy treatment, Tube occlusion, Nomogram.
PMID:38978242 | DOI:10.29271/jcpsp.2024.07.790
Abdom Radiol (NY). 2024 Jul 8. doi: 10.1007/s00261-024-04441-2. Online ahead of print.
ABSTRACT
Cytoreductive surgery (CRS) with or without hyperthermic intraperitoneal chemotherapy (HIPEC) is the mainstay of potentially curative surgical treatment for malignancies that have spread to peritoneal surfaces. This surgical procedure is however associated with high morbidity and appropriate patient selection and planning is therefore essential. Available multimodality imaging techniques include CT with oral and intravenous contrast, MRI including use of dedicated peritoneal protocol and FDG-PET/CT. These used with the correct technique, read by specialist radiologists and discussed under the auspices of a dedicated multidisciplinary team, can help to improve outcomes. We demonstrate that imaging not only provides information about peritoneal disease burden but more importantly want to shift the reader's focus to disease distribution. Our examples highlight how imaging helps avoid futile surgery by identifying patients with disease in unfavourable sites and show the strength and limitations of the various imaging modalities. We share how MR imaging can help identify multifocal and often occult sites including widespread miliary disease. Our examples provide a comprehensive overview demonstrating how imaging can help plan surgery by identifying patients who may need splenic vaccinations, counselling for stoma, egg harvesting and input from surgeons with other specialist expertise greatly increasing likelihood of achieving complete cytoreduction.
PMID:38976054 | DOI:10.1007/s00261-024-04441-2
Ann Gastroenterol Surg. 2024 Mar 11;8(4):701-710. doi: 10.1002/ags3.12791. eCollection 2024 Jul.
ABSTRACT
BACKGROUND: Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is established in the management of pseudomyxoma peritonei (PMP), selected cases of peritoneal mesothelioma, and resectable colorectal or ovarian peritoneal metastases in Western countries. However, the efficacy and feasibility of these techniques are not well established in the Asian population, and little has been reported on long-term survival outcomes for surgically resected PMP patients.
MATERIALS AND METHODS: Retrospective analysis of a prospective database of short- and longer-term outcomes of consecutive patients who underwent CRS and HIPEC for PMP in a newly established peritoneal malignancy unit in Japan between 2010 and 2016.
RESULTS: A total of 105 patients underwent CRS and HIPEC and 57 maximal tumor debulking (MTD) for pseudomyxoma peritonei. In the CRS group, the primary tumor was appendiceal in 94 patients (90%) followed by ovarian and colorectal. Major postoperative complications occurred in 22/105 patients (21%) with one in-hospital mortality (0.9%). The 5-year overall and disease-free survival rates for the CRS group were 74.2% and 50.1%, respectively. Multivariate analysis revealed unfavorable histology to be the significant predictor of reduced overall and disease-free survival. Completeness of cytoreduction, CA19-9, and CA125 were also associated with disease-free survival.
CONCLUSIONS: This is the first report on long-term outcomes and survival analysis of CRS and HIPEC for PMP in the Asian population. CRS and HIPEC can be conducted with reasonable safety and favorable survival in a new center. Complete tumor removal and histological type are the strongest prognostic factors for both overall and disease-free survival.
PMID:38957568 | PMC:PMC11216784 | DOI:10.1002/ags3.12791
Ghana Med J. 2024 Mar;58(1):109-114. doi: 10.4314/gmj.v58i1.15.
ABSTRACT
INTRODUCTION: Mucocoele of the appendix occurs in 0.2-0.7% of people in the world without any well-defined clinical symptoms. It occurs when there is an accumulation of mucous in the lumen of the appendix.
CASE PRESENTATION: We present three cases: a 48-year-old male admitted to the emergency room with a one-day history of right iliac fossa pain. Abdominal examination was suggestive of acute appendicitis. The initial abdominal computerised tomography scan was reported as being unremarkable. At surgery, a firm tumour of the appendix was found, and a limited right hemicolectomy was done. Histopathology confirmed a mucocoele of the appendix with borderline mucinous histology.The second case is a 63-year-old man who presented with a one-year history of abdominal distension and weight loss. Previous abdominal ultrasound was suggestive of liver cirrhosis with significant ascitic fluid. Abdominal magnetic resonance imaging found an appendix mucocoele with infiltration of the omentum and scalloping of the liver surface suggestive of pseudomyxoma peritonei. A percutaneous biopsy of the omental mass confirmed metastatic mucinous adenocarcinoma of the appendix.The third case is a 68-year-old man who, during an annual medical check-up, had an incidental finding of a cystic right iliac fossa mass on ultrasound, confirmed on abdominopelvic computerised tomography scan to be an appendix mucocele. He had laparoscopic appendicectomy. The histopathological diagnosis confirmed a mucinous cystadenoma of the appendix.
CONCLUSION: Preoperative diagnosis of appendiceal mucocoele is difficult and commonly discovered intraoperatively. The prognosis is good for the histologically benign type, but it is poor when malignant or peritoneal lesions are present.
FUNDING: None declared.
PMID:38957279 | PMC:PMC11215239 | DOI:10.4314/gmj.v58i1.15
Eur J Surg Oncol. 2024 Sep;50(9):108507. doi: 10.1016/j.ejso.2024.108507. Epub 2024 Jun 25.
ABSTRACT
BACKGROUND: Obesity is a public health concern with an increasing occurrence worldwide. Literature regarding impact of obesity on results after management of peritoneal carcinomatosis is poor. Our aim was to compare postoperative and oncological outcomes after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for rare peritoneal malignancies according to the body mass index.
METHODS: All the patients managed by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for rare peritoneal malignancies (including mainly pseudomyxoma peritonei and peritoneal mesothelioma), between 1995 and 2020, were retrospectively included from the French national registry of rare peritoneal tumors.
RESULTS: 1450 patients were retrospectively included (63.5 % female, mean age 54 ± 13 years). Patients were divided into two groups according to their body mass index: non-obese (n = 1248, 86 %) and obese (n = 202, 14 %). Overall morbidity was significantly lower in non-obese patients in comparison with obese patients (n = 532/1248, 43 % vs n = 106/202, 53 %, p = 0.009). Medical and surgical morbidities were significantly lower in non-obese patients in comparison with obese patients (423/1258, 34 % vs n = 86/202, 43 %, p = 0.02 and n = 321/1248, 26 % vs n = 67/202, 33 %, p = 0.003, respectively). One-, 5- and 10-year overall survivals were similar between non-obese and obese patients (95 %, 82 % and 70 % vs 94 %, 76 % and 63 %; p = 0.1). One-, 5- and 10-year disease free survivals were similar between non-obese and obese patients (84 %, 67 % and 61 % vs 79 %, 62 % and 56 %, p = 0.1).
CONCLUSION: Obese patients have to be carefully managed after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for rare peritoneal malignancies. Some perioperative prophylactic treatments could be specifically implemented to reduce thromboembolic events, metabolic and wound complications.
PMID:38954880 | DOI:10.1016/j.ejso.2024.108507
Cureus. 2024 May 28;16(5):e61244. doi: 10.7759/cureus.61244. eCollection 2024 May.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a rare and complex clinical syndrome characterized by the accumulation of mucinous ascites within the peritoneal cavity, typically associated with mucinous tumours of appendiceal origin. Despite its rarity, PMP poses significant challenges in diagnosis and management due to its indolent yet locally aggressive nature. This comprehensive review provides insights into the diagnosis, management, and prognosis of PMP, synthesizing current evidence and emerging trends in the field. Challenges and opportunities in PMP management are discussed, along with recommendations for clinical practice emphasizing the importance of a multidisciplinary approach and specialized care. Despite ongoing challenges, advances in surgical techniques, perioperative chemotherapy, and emerging therapies offer hope for improved outcomes and quality of life for PMP patients.
PMID:38939264 | PMC:PMC11210681 | DOI:10.7759/cureus.61244
Int J Hyperthermia. 2024;41(1):2372356. doi: 10.1080/02656736.2024.2372356. Epub 2024 Jun 27.
ABSTRACT
BACKGROUND: The greater omentum is routinely resected during cytoreductive surgery (CRS), but few studies have analyzed the rationale behind this. This study aimed to assess the prevalence of omental metastases (OM) and the correlation between macroscopically suspected and microscopically confirmed OM, in patients with pseudomyxoma peritonei (PMP) or colorectal peritoneal metastases (PM).
METHOD: All patients without previous omentectomy, treated with initial CRS and hyperthermic intraperitoneal chemotherapy for PMP or colorectal PM, at Uppsala University Hospital in 2013-2021, were included. Macroscopic OM in surgical reports was compared with histopathological analyses.
RESULTS: In all, 276 patients were included. In those with PMP, 112 (98%) underwent omentectomy and 67 (59%) had macroscopic suspicion of OM. In 5 (4%) patients, the surgeon was uncertain. Histopathology confirmed OM in 81 (72%). In patients with macroscopic suspicion, 96% had confirmed OM (positive predictive value, PPV). In patients with no suspicion, 24% had occult OM (negative predictive value, NPV = 76%). In patients with colorectal PM, 156 (96%) underwent omentectomy and 97 (60%) had macroscopic suspicion. For 5 (3%) patients, the surgeon was uncertain. OM was microscopically confirmed in 90 (58%). PPV was 85% and NPV was 89%. The presence of OM was a univariate risk factor for death in PMP (HR 3.62, 95%CI 1.08-12.1) and colorectal PM (HR 1.67, 95%CI 1.07-2.60), but not in multivariate analyses.
CONCLUSION: OM was common and there was a high risk of missing occult OM in both PMP and colorectal PM. These results support the practice of routine omentectomy during CRS.
PMID:38937059 | DOI:10.1080/02656736.2024.2372356
J Med Case Rep. 2024 Jun 28;18(1):296. doi: 10.1186/s13256-024-04612-1.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management.
CASE PRESENTATION: A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases.
CONCLUSIONS: Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management.
PMID:38937808 | PMC:PMC11212264 | DOI:10.1186/s13256-024-04612-1
J Surg Oncol. 2024 Jun 27. doi: 10.1002/jso.27742. Online ahead of print.
ABSTRACT
BACKGROUND: The absolute requirement for a long-term favorable result with cytoreductive surgery for pseudomyxoma peritonei is a complete resection of all visible disease. A combination of parietal peritonectomy procedures and visceral resections is required for this to occur. The cytoreductive surgery is supplemented by hyperthermic intraperitoneal chemotherapy.
METHODS: We searched our database and secured files for patients who required a total gastrectomy and a total colectomy to achieve a complete cytoreductive surgery. Survival of low-grade mucinous neoplasm (LAMN) and mucinous appendiceal adenocarcinoma (MACA) histologies were determined. Clinical and histologic variables were assessed for their impact on survival.
RESULTS: Thirteen of 450 patients (2.9%) with LAMN histology and 14 of 186 patients (7.5%) with MACA histology had these visceral resections. Median survival of these 27 patients was 10 years. LAMN and MACA patients showed the same survival. For LAMN histology, this requirement for extensive visceral resection markedly reduced survival (p < 0.0001). For MACA, there was no adverse impact on survival (p = 0.4359). Class 4 adverse events caused reduced survival (p = 0.0014).
CONCLUSIONS: A 10-year median survival accompanies total gastrectomy plus total colectomy for advanced pseudomyxoma peritonei. Systemic chemotherapy and class 4 adverse events reduced survival.
PMID:38935844 | DOI:10.1002/jso.27742
Cureus. 2024 May 27;16(5):e61151. doi: 10.7759/cureus.61151. eCollection 2024 May.
ABSTRACT
The concurrent presentation of a low-grade appendiceal mucinous neoplasm (LAMN) and a borderline Brenner tumor (BT) of the ovary are exceedingly rare. Brenner tumors stand out as a particularly uncommon form, making up only around 5% of all benign epithelial tumors of the ovary. Among the ovarian Brenner, the borderline subtype is even rarer. Appendiceal neoplasm (LAMN) and right ovarian BT cannot be distinguished due to their anatomical position. LAMN is often an incidental finding and at later stages when left undiagnosed may lead to pseudomyxoma peritonei (PMP). This case describes a postmenopausal woman in her 50s experiencing abdominal pain and bloating for a week. Elevated carcinoembryonic antigen (CEA) levels and imaging suggested a potential right ovarian tumor. Interestingly, it revealed a unique combination of borderline Brenner tumor of the right ovary and low-grade appendiceal mucinous neoplasm.
PMID:38933633 | PMC:PMC11200205 | DOI:10.7759/cureus.61151
Front Oncol. 2024 Jun 11;14:1396265. doi: 10.3389/fonc.2024.1396265. eCollection 2024.
ABSTRACT
Low-grade appendiceal mucinous neoplasms (LAMNs) are rare and heterogeneous diseases that, despite their increased incidence, are well differentiated, tend to be painless, and histologically lack distinctive invasive features without infiltrative growth, destructive infiltration, or associated pro-fibroproliferative responses. However, the biological behaviour of these tumours is difficult to determine preoperatively or intraoperatively, and the possibility of rupture puts patients at risk for peritoneal pseudomucinous neoplasms (PMPs).Patients with low-grade appendiceal mucinous tumours and peritoneal pseudomucinous tumours experience slow disease progression and are incurable and have a high risk of recurrence, morbidity, and ultimately death, despite the reported 5- and 10-year survival rates of 50-86% and 45-68%, respectively. In this article, we report the case of a 80-year-old male with a giant low-grade appendiceal mucinous tumour associated with a peritoneal pseudomucinous tumour, and discuss the diagnostic and management strategies for giant low-grade appendiceal mucinous tumours in the context of a literature review.
PMID:38919528 | PMC:PMC11196944 | DOI:10.3389/fonc.2024.1396265
Cureus. 2024 May 20;16(5):e60664. doi: 10.7759/cureus.60664. eCollection 2024 May.
ABSTRACT
Benign multicystic peritoneal mesothelioma (BMPM), also known as multicystic peritoneal mesothelioma (MCPM), is a rare cystic neoplasm arising from the mesothelium lining of the abdominal and pelvic peritoneum. This entity has been disproportionately described in women of reproductive age. Both the etiology and pathogenesis of the condition are not well understood. Preoperative diagnosis is challenging as differentials are varied and include endometriosis, lymphangioma, pseudomyxoma peritonei, cystic adenomatoid tumor, and malignant peritoneal mesothelioma. Management options include cytoreductive surgery (CRS) with or without heated intraperitoneal chemotherapy (HIPEC). In this case report, we highlight the complexity of preoperative diagnosis, presentation, workup, treatment, and management of BMPM. We report the case of a female patient presenting with abdominal pain and imagining consistent with cystic intra-abdominal lesions. After an inconclusive percutaneous biopsy and a multi-disciplinary tumor board discussion, the patient was offered CRS with HIPEC. Intra-operative frozen section indicated benign epithelial lined cysts. CRS and HIPEC were performed. After a second opinion, the lesions were confirmed by pathology and immunohistochemistry to be BMPM. In this report, we discuss the gold standard of care for patients with BMPM to improve the disease control rate. This pathway is proposed in our study, and, thus, we conclude that BMPM should be considered in the differential diagnosis of patients presenting with symptomatic multiple intraperitoneal cystic lesions.
PMID:38899248 | PMC:PMC11186218 | DOI:10.7759/cureus.60664
Cancers (Basel). 2024 May 31;16(11):2099. doi: 10.3390/cancers16112099.
ABSTRACT
BACKGROUND: Careful macroscopic assessment of surgical scars is needed to avoid routine scar resection during cytoreductive surgery (CRS) for peritoneal metastases (PM). This study aimed to analyze the correlation between macroscopically suspected and microscopically confirmed scar metastases (SMs), and to analyze the prognostic impact of not undergoing routine scar resection.
METHOD: All patients with previous surgery, treated with CRS and hyperthermic intraperitoneal chemotherapy, for colorectal PM or pseudomyxoma peritonei (PMP), at Uppsala University Hospital in 2013-2021, were included. Macroscopic SMs in surgical reports were compared with histopathological analyses.
RESULTS: In total, 227 patients were included. Among colorectal PM patients (n = 156), SM was macroscopically suspected in 41 (26%) patients, and 63 (40%) underwent scar resection. SM was confirmed in 19 (30%). Among patients with macroscopic suspicion, 45% had confirmed SM (positive predictive value, PPV). A total of 1 of 23 (4%) patients with no macroscopic suspicion had SM (negative predictive value, NPV = 96%). Among the PMP patients (n = 71), SM was macroscopically suspected in 13 (18%), and 28 (39%) underwent scar resection, of whom 12 (43%) had SM. The PPV was 77%. Occult SM was found in 1 of 14 (NPV = 93%). Not undergoing routine scar resection did not affect recurrence-free survival (RFS, p = 0.2) or overall survival (OS, p = 0.1) in colorectal PM patients or PMP patients (RFS p = 0.7, OS p = 0.7).
CONCLUSION: Occult SM is uncommon and scar resection does not affect RFS or OS. Therefore, macroscopically benign-appearing scars can be left without resection, though resection should be performed upon suspicion or uncertainty.
PMID:38893218 | PMC:PMC11171061 | DOI:10.3390/cancers16112099
ANZ J Surg. 2024 Jun 18. doi: 10.1111/ans.19130. Online ahead of print.
NO ABSTRACT
PMID:38888277 | DOI:10.1111/ans.19130
Surg Case Rep. 2024 Jun 13;10(1):144. doi: 10.1186/s40792-024-01936-4.
ABSTRACT
BACKGROUND: Neoplasms derived from remnant appendix are rarely described, with most cases arising from the appendiceal "stump". Here, we present two surgical cases of appendiceal neoplasms derived from appendiceal "tip" remnants.
CASE PRESENTATION: The first patient was a 71-year-old man who had undergone laparoscopic appendectomy for acute appendicitis 12 years prior. During appendectomy, the appendiceal root was ligated, but the appendix was not completely removed due to severe inflammation. At the most recent presentation, computed tomography (CT) was performed to examine choledocholithiasis, which incidentally revealed a cystic lesion of approximately 90 mm adjacent to the cecum. A retrospective review revealed that the cystic lesion had increased in size over time, and laparoscopic ileocecal resection was performed. Pathology revealed no continuity from the appendiceal orifice to the cyst, and a diagnosis of low-grade appendiceal mucinous neoplasm (LAMN) was made from the appendiceal tip remnant. The patient was discharged without complications. The second patient was a 65-year-old man who had undergone surgery for peritonitis due to severe appendicitis 21 years prior. During this operation, the appendix could not be clearly identified due to severe inflammation; consequently, cecal resection was performed. He was referred to our department with a chief complaint of general fatigue and loss of appetite and a cystic lesion of approximately 85 mm close to the cecum that had increased over time. CT showed irregular wall thickening, and malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was performed. The pathological diagnosis revealed mucinous adenocarcinoma (TXN0M0) arising from the remnant appendiceal tip. The patient is undergoing follow-up without postoperative adjuvant chemotherapy, with no evidence of pseudomyxoma peritonei or cancer recurrence for 32 months postoperatively.
CONCLUSIONS: If appendicitis-associated inflammation is sufficiently severe that accurate identification of the appendix is difficult, it may remain on the apical side of the appendix, even if the root of the appendix is ligated and removed. If the appendectomy is terminated incompletely, it is necessary to check for the presence of a residual appendix postoperatively and provide appropriate follow-up.
PMID:38867137 | PMC:PMC11169428 | DOI:10.1186/s40792-024-01936-4
Innov Surg Sci. 2024 Mar 14;9(1):3-15. doi: 10.1515/iss-2023-0055. eCollection 2024 Mar.
ABSTRACT
Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of affected patients' prognoses. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are associated with favorable long-term outcomes in carefully selected patients with peritoneal metastases (PM). We aim to summarize the current results about the initial malignancies and their peritoneal spreads. The current literature has been scrutinized, and studies between 2016 and 2022 were included wherein long-term, progression-free (PFS), and overall survival (OS) data were considered relevant information. Medline, Embase, and Google Scholar have been the main sources. Hereby, we cover all the primer malignancies: gastric, ovarian, and colorectal cancers with peritoneal metastases (PM), malignant peritoneal mesothelioma, and pseudomyxoma peritonei. Examining the advances in the current peer-reviewed literature about the indications of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), target groups, risk factors, and other influencing elements, we intend to provide a complex state-of-the-art report, establishing the relevant aspects of that emerging treatment method.
PMID:38826635 | PMC:PMC11138857 | DOI:10.1515/iss-2023-0055
Cureus. 2024 May 2;16(5):e59540. doi: 10.7759/cureus.59540. eCollection 2024 May.
ABSTRACT
Appendiceal mucinous neoplasms (AMNs) are uncommon gastrointestinal tumors characterized by mucus accumulation in the appendix. Patients may complain of acute appendicitis-like symptoms with other alarming features, but approximately half of the cases of AMNs are found incidentally on imaging. Early diagnosis and management of these neoplasms are important to prevent malignant progression and complications such as bowel obstruction and pseudomyxoma peritonei. We report a case of a 28-year-old female who initially presented with vomiting and acute left lower abdominal pain radiating to the left flank. Computed tomography (CT) revealed a 1.5 mm stone in the left ureteral vesicular junction and a 2.3 x 2.4 x 5.2 cm cystic tubular mass at the base of the cecum, suspicious of an appendiceal mucocele. An elective laparoscopic appendectomy was performed on this admission, which was converted to a right hemicolectomy due to the pathologic finding of a focally high-grade AMN on intraoperative frozen specimen pathology. This report aims to provide an example of a case of an incidental AMN and how it was diagnosed and managed surgically. AMNs are rare tumors that originate from the appendix and can pose diagnostic and therapeutic challenges due to their diverse clinical presentations and variable histopathological features. The majority of cases of AMNs are discovered in middle-aged individuals (40-50 years of age) after an appendectomy is performed and examined by pathology. This case report aims to describe a rare presentation of a 28-year-old female patient with an incidental finding of AMN on a CT scan of the abdomen while being worked up for suspected nephrolithiasis. We will provide a comprehensive overview of a unique presentation of AMN, highlighting its clinical manifestations, diagnostic approach, and management strategies. We present the case of a 28-year-old female patient who presented to the emergency department with complaints of acute left lower quadrant abdominal pain radiating to the left flank and vomiting. After an initial assessment and workup, which included lab investigations and imaging, a diagnosis of unilateral hydronephrosis due to a calculus of the ureterovesical junction was made. However, there was also suspicion of an appendiceal mucocele, as evidenced by a CT scan of the abdomen and pelvis. On admission day one, under the care and management of the urology team, she passed the stone with complete resolution of the presenting symptoms. On hospital day two, she underwent an elective laparoscopic appendectomy followed by a right hemicolectomy due to findings of high-grade mucinous neoplasm on the resected frozen specimen near the base of the appendix. AMN was an incidental finding based on CT imaging and macroscopic findings, which was later confirmed by histopathological assessment and report.
PMID:38826949 | PMC:PMC11144036 | DOI:10.7759/cureus.59540
World J Gastrointest Endosc. 2024 May 16;16(5):232-236. doi: 10.4253/wjge.v16.i5.232.
ABSTRACT
We recently read with great interest a study by Zhang et al in the World Journal of Gastroenterology. In our practice, we focus specifically on examining appendiceal mucinous neoplasms (AMNs) with endoscopic ultrasound (EUS) using different scopes. AMNs are rare neoplastic lesions characterized by an accumulation of mucin inside a cystic dilatation of the appendix. Clinically, they can present as nonspecific acute appendicitis. AMNs can turn into a life-threatening condition, termed pseudomyxoma peritonei, in which the ruptured appendix causes accumulation of mucin in the abdomen. Therefore, accurate and rapid diagnosis of AMN is essential. EUS is able to confirm and stage AMNs; although, EUS examination was once limited to the rectal and anal regions due to the conventional oblique-view scopes. With the emergence of new forward-view linear echoendoscopes and instruments like EUS miniprobes and overtubes, the scope of examination is changing. Herein, we discuss the feasibility of using the curved linear array echoendoscopes to examine cecal and appendiceal orifice lesions.
PMID:38813577 | PMC:PMC11130547 | DOI:10.4253/wjge.v16.i5.232
Australas J Ultrasound Med. 2024 Mar 12;27(2):131-135. doi: 10.1002/ajum.12377. eCollection 2024 May.
ABSTRACT
A low-grade appendiceal mucinous neoplasm (LAMN) is a cystic dilatation of the appendix resulting from the accumulation of mucinous secretions caused by a luminal obstruction. Although usually benign, pseudomyxoma peritonei may occur in the event of rupture, and 10% of cases may be secondary to appendiceal cystadenocarcinoma. A LAMN is both more common and more likely to have a malignant association in women, making it an entity with which practitioners of gynaecological ultrasound should be familiar. Although not the primary aim, early pregnancy ultrasound assessments can offer the diagnostic opportunity to identify pelvic pathology. A LAMN can be identified on ultrasonography by visualisation of an adnexal mass separate to the ovary, which due to the layers of secretions has a distinctive appearance previously likened to 'onion-skin' or 'whipped-cream'. Here, we describe an incidental finding of a LAMN during an early pregnancy assessment. Practitioners of early pregnancy ultrasound should be familiar with the characteristic morphology of this rare but important finding.
PMID:38784695 | PMC:PMC11109991 | DOI:10.1002/ajum.12377
J Pak Med Assoc. 2024 May;74(5):1007-1008. doi: 10.47391/JPMA.24-40.
ABSTRACT
Psuedomyxoma peritonei is an infrequent clinical entity characterised by intraperitoneal mucinous/gelatinous ascites produced by the cancerous cells. It has been associated with gastrointestinal, gynaecological, lung and breast tumours. It is commonly asymptomatic and is most often detected incidentally on abdominopelvic imaging or laparoscopy. Higher histological grade of the tumour shows increased metabolic activity on 18F-Fluorodeoxyglucose (FDG) positron-emission tomography (PET) computed tomography (CT). It has been rarely reported in patients with sarcoma. We hereby present an interesting case of incidentally diagnosed pseudomyxoma peritonei on 18FDG PET-CT scan of a patient with soft tissue sarcoma of peripheral nerve sheath.
PMID:38783460 | DOI:10.47391/JPMA.24-40
World J Surg Oncol. 2024 May 21;22(1):134. doi: 10.1186/s12957-024-03412-7.
ABSTRACT
BACKGROUND: Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). However, tubing blockages during HIPEC treatment pose a common challenge, impeding the smooth progression of therapy. Few studies to date have explored the incidence and risk factors of tube occlusion during HIPEC in patients with appendiceal PMP, as well as its adverse impact on postoperative complications.
METHODS: From October 2017 to June 2023, a total of 80 patients with appendiceal PMP undergoing combined CRS and HIPEC were included in this study. Tubing blockage events were strictly defined, with patients experiencing blockages during HIPEC treatment allocated to the study group, while those with unobstructed perfusion were assigned to the control group. A comparative analysis was conducted between the two groups regarding post-HIPEC health assessments and occurrence of complications. Risk factors for luminal occlusion during closed HIPEC procedures were identified through univariate and multivariate analysis of data from 303 HIPEC treatments.
RESULTS: Tubing blockages occurred in 41 patients (51.3%). The study group experienced prolonged gastrointestinal decompression time (4.1 ± 3.0 vs. 2.5 ± 1.7 days, P = 0.003) and prolonged time to bowel movement (6.1 ± 2.3 vs. 5.1 ± 1.8 days, P = 0.022) compared to the control group. There was no significant difference in the incidence of complications between the two groups. The 1-year survival rate postoperatively was 97%, and the 3-year survival rate was 81%, with no association found between tubing blockage and poorer survival. Additionally, In 303 instances of HIPEC treatment among these 80 patients, tube occlusion occurred in 89 cases (89/303, 29.4%). Multivariable logistic regression analysis revealed age, diabetes, hypertension, and pathology as independent risk factors for tube occlusion.
CONCLUSION: Tubing blockages are a common occurrence during HIPEC treatment, leading to prolonged postoperative gastrointestinal functional recovery time. When patients are elderly and have concomitant hypertension and diabetes, along with a histological type of low-grade mucinous tumor, the risk of tube occlusion increases. However, this study did not find a significant correlation between tubing blockage and the incidence of postoperative complications or overall patient survival.
PMID:38769546 | PMC:PMC11107022 | DOI:10.1186/s12957-024-03412-7
Biol Proced Online. 2024 May 15;26(1):13. doi: 10.1186/s12575-024-00239-0.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare peritoneal mucinous carcinomatosis with largely unknown underlying molecular mechanisms. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the only therapeutic option; however, despite its use, recurrence with a fatal outcome is common. The lack of molecular characterisation of PMP and other mucinous tumours is mainly due to the physicochemical properties of mucin.
RESULTS: This manuscript describes the first protocol capable of breaking the mucin barrier and isolating proteins from mucinous tumours. Briefly, mucinous tumour samples were homogenised and subjected to liquid chromatography using two specific columns to reduce mainly glycoproteins, albumins and immunoglobulin G. The protein fractions were then subjected to mass spectrometry analysis and the proteomic profile obtained was analysed using various bioinformatic tools. Thus, we present here the first proteome analysed in PMP and identified a distinct mucin isoform profile in soft compared to hard mucin tumour tissues as well as key biological processes/pathways altered in mucinous tumours. Importantly, this protocol also allowed us to identify MUC13 as a potential tumour cell marker in PMP.
CONCLUSIONS: In sum, our results demonstrate that this protein isolation protocol from mucin will have a high impact, allowing the oncology research community to more rapidly advance in the knowledge of PMP and other mucinous neoplasms, as well as develop new and effective therapeutic strategies.
PMID:38750435 | PMC:PMC11094946 | DOI:10.1186/s12575-024-00239-0
Br J Radiol. 2024 Aug 1;97(1160):1405-1412. doi: 10.1093/bjr/tqae102.
ABSTRACT
Appendiceal mucinous neoplasms are rare and can be associated with the development of disseminated peritoneal disease known as pseudomyxoma peritonei (PMP). Mucinous tumours identified on appendicectomy are therefore followed up to assess for recurrence and the development of PMP. In addition, individuals who initially present with PMP and are treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) are followed up to assess for recurrence. However, despite the concerted efforts of multiple expert groups, the optimal imaging follow-up protocol is yet to be established. The purpose of this article is to review the available evidence for imaging surveillance in these populations to identify the optimum post-resection imaging follow-up protocol.
PMID:38749003 | PMC:PMC11256959 | DOI:10.1093/bjr/tqae102
ANZ J Surg. 2024 Jul-Aug;94(7-8):1324-1332. doi: 10.1111/ans.19021. Epub 2024 May 15.
ABSTRACT
BACKGROUND: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is traditionally a maximally invasive operation with a large abdominal incision and multi-visceral resections. However, to minimize abdominal wall morbidity and improve functional recovery, some centres have adopted a minimally invasive (MI) approach in select cases. The primary aim of this systematic review and meta-analysis was to assess the evidence for safety and patient selection for minimally invasive approaches to CRS and HIPEC with curative intent.
METHODS: A PRISMA-compliant systematic review was performed using three electronic databases: Ovid MEDLINE, EMBASE and Web of Science. Data regarding postoperative morbidity was meta-analysed.
RESULTS: Thirteen studies met the inclusion criteria (N = 462 MI patients), all of which were retrospective in design. Six studies included an open comparison group. Pseudomyxoma peritonei, mesothelioma and ovarian carcinoma made up the majority of cases (>90%), with a PCI < 10 listed as a prerequisite to selection across all studies. On pooled analysis there was no difference in major morbidity between MI and open groups (OR 0.52 95% CI 0.18-1.46, P = 0.33). There was one perioperative death reported in the MI group. Length of stay appeared shorter in the MI group (median range MI: 4-11 v Open: 7-13 days). Short-term recurrence and overall survival between both groups also appeared no different.
CONCLUSION: Minimally invasive CRS and HIPEC appears feasible and safe in appropriately selected patients. Clear histological stratification and longer term follow up is required to determine oncological safety, particularly in more aggressive tumours such as colorectal peritoneal metastases.
PMID:38747542 | DOI:10.1111/ans.19021
Radiol Case Rep. 2024 May 6;19(8):2929-2933. doi: 10.1016/j.radcr.2024.04.034. eCollection 2024 Aug.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific clinical manifestations, leading to a high rate of misdiagnosis. This report describes the case of a 62-year-old male patient with recurrent and metastatic PMP. The patient first experienced unexplained epigastric pain and paroxysmal abdominal pain accompanied by distension over 8 years ago. He underwent surgical interventions for the condition in other hospitals in 2015 and 2018, respectively.
PMID:38745977 | PMC:PMC11091453 | DOI:10.1016/j.radcr.2024.04.034
Eur J Surg Oncol. 2024 Jul;50(7):108395. doi: 10.1016/j.ejso.2024.108395. Epub 2024 May 9.
ABSTRACT
The study of circulating tumor DNA (ctDNA) plays a pivotal role in advancing precision oncology, providing valuable information for individualized patient care and contributing to the ongoing effort to improve cancer diagnosis, treatment, and management. However, its applicability in pseudomyxoma peritonei (PMP) remains unexplored. In this multicenter retrospective study involving 21 PMP patients, we investigated ctDNA presence in peripheral blood using three distinct methodologies. Despite mucinous tumor tissues exhibiting KRAS and GNAS mutations, ctDNA for these mutations was undetectable in blood samples. In this pilot study, circulating tumor DNA was not detected in blood when the tumor harbored mutations of known significance. In the future, a study with a larger sample size is needed to confirm these findings and to determine whether ctDNA could identify patients at risk for early recurrence and/or systemic metastases.
PMID:38735236 | DOI:10.1016/j.ejso.2024.108395
Cureus. 2024 Apr 8;16(4):e57857. doi: 10.7759/cureus.57857. eCollection 2024 Apr.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a rare intra-abdominal malignancy characterized by diffuse dissemination of mucinous tumor cells, leading to mucinous ascites. Accurate diagnosis is crucial for appropriate management. This report presents a case of a 55-year-old Lebanese male farmer initially misdiagnosed with liver cirrhosis who presented with progressive abdominal distension refractory to diuretics and dietary modifications. Paracentesis revealed a mucinous exudate, with subsequent clinical and histopathological examination confirming PMP. The patient was referred for further evaluation at a specialized center equipped for cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). This case highlights the diagnostic challenges of PMP due to its non-specific presentation, emphasizing the importance of prompt and accurate diagnosis to facilitate optimal therapeutic intervention.
PMID:38721212 | PMC:PMC11077503 | DOI:10.7759/cureus.57857
Int J Surg Case Rep. 2024 Jun;119:109716. doi: 10.1016/j.ijscr.2024.109716. Epub 2024 Apr 28.
ABSTRACT
INTRODUCTION: Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain or presenting as an acute appendicitis or being asymptomatic.
CASE PRESENTATION: We present a case of a patient presenting as an acute appendicitis with a mucocele, and then classified as HAMN. The patient was treated with initial laparoscopic approach and then conversion in laparotomy with appendectomy. Histology demonstrated a high grade appendiceal mucinous neoplasm limited to submucosa (pT3), with concomitant acute phlegmonous appendicitis. The patient was subsequently sent to a referral center where a right hemicolectomy with HIPEC was performed.
DISCUSSION: HAMN is a rare entity, only recently classified as a new kind of appendiceal mucinous neoplasm. Due to the supposed higher aggressivity, HAMN must be treated as an appendiceal adenocarcinoma. The treatment of this rare entity is not yet well standardized, because of the rarity of this disease.
CONCLUSION: HAMN is a very rare tumor. In the emergency setting, it is mandatory to avoid rupture of the appendix, to minimize the risk of developing pseudomyxoma peritonei. Pathology is essential for further decisions in these patients and plays a very important role in treatment and prognosis.
PMID:38688155 | PMC:PMC11067490 | DOI:10.1016/j.ijscr.2024.109716
Anticancer Res. 2024 May;44(5):2239-2241. doi: 10.21873/anticanres.17031.
ABSTRACT
BACKGROUND/AIM: Pseudomyxoma peritonei (PMP) is a rare condition characterized by diffuse spread of mucinous tumors within the peritoneal cavity. Traditional treatment modalities, such as cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are challenging in cases of recurrent disease, owing to anatomical complexities and increasing morbidity and mortality risk. BromAc® has emerged as a novel, targeted therapy for PMP with evidence for intra-tumoral administration to break down mucin deposits.
CASE REPORT: We present a 70-year-old female with confirmed diagnosis of symptomatic appendiceal PMP situated behind the stomach, refractory to prior CRS and HIPEC. Transhepatic intra-tumor injection of BromAc® was performed, guided by imaging, with catheter placement into the posterior gastric mucinous tumor. The procedure was well-tolerated, and post-treatment imaging revealed a significant 40% reduction in tumor burden. The patient had fever on cycle days two and three, which self-resolved and septic screen performed was negative. Following BromAc® administration, the patient demonstrated improvement in symptoms and quality of life.
CONCLUSION: This case highlights the potential efficacy and safety of transhepatic administration of BromAc® for the treatment of recurrent PMP behind the stomach. The targeted delivery of BromAc® directly into a mucinous tumor via the transhepatic route offers a minimally invasive alternative for cases where traditional surgical interventions pose challenges. However, further research and clinical trials are warranted to validate the broader applicability of this novel approach, assess long-term outcomes, and optimize procedural parameters for enhanced therapeutic outcomes in PMP treatment.
PMID:38677761 | DOI:10.21873/anticanres.17031
Int J Surg Case Rep. 2024 Jun;119:109656. doi: 10.1016/j.ijscr.2024.109656. Epub 2024 Apr 20.
ABSTRACT
INTRODUCTION AND IMPORTANCE: Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most commonly arising in the appendix.
CASE PRESENTATION: A 63-year-old male patient presented to the hospital with complaints of abdominal pain, significant weight loss, a palpable mass in the right iliac fossa, and evidence of ascites. A diagnosis of PMP was proposed based on findings from a computed tomography (CT) scan, which was subsequently confirmed through histopathological examination of a biopsy. The patient underwent successful treatment with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS-HIPEC).
CLINICAL DISCUSSION: Diagnosing PMP presents challenges due to its rarity and the potential for extensive spread throughout the peritoneal cavity, necessitating a multidisciplinary approach for successful treatment.
CONCLUSION: Pseudomyxoma peritonei is a rare yet medically significant condition. Documenting a case of this ailment in Palestine has the potential to advance medical understanding, raise awareness, and improve patient care standards within the local healthcare system. By documenting this uncommon condition, healthcare practitioners in Palestine can gain valuable insights into its manifestations, diagnostic processes, and treatment modalities. This contribution not only enriches the global medical literature but also promotes collaboration in addressing the challenges associated with rare diseases.
PMID:38677258 | PMC:PMC11067356 | DOI:10.1016/j.ijscr.2024.109656
Cancers (Basel). 2024 Apr 9;16(8):1446. doi: 10.3390/cancers16081446.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing, inadequately understood neoplasm with a 5-year progression-free survival rate of as low as 48%. It is characterized by varying degrees of malignancy and the production of mucinous and gelatinous structures. Typically, the development of pseudomyxoma peritonei is associated with the rupture of appendiceal mucinous tumors and other gastrointestinal or ovarian mucinous tumors. The goal of our literature review was to identify various aspects that characterize the ovarian causes of pseudomyxoma peritonei.
MATERIALS AND METHODS: The authors performed an extensive literature search between 1 February 2024 and 2 March 2024 on the following databases: Pubmed, Scopus, Oxford Journals, and Reaxys, and the findings were summarized into seven main clinical and paraclinical situations.
RESULTS: According to our research, the main instances in which pseudomyxoma peritonei can be triggered by an ovarian cause are the following: (1) mucinous cystadenoma; (2) mucinous ovarian cancer; (3) colon cancer with ovarian metastasis; (4) malignant transformation of an ovarian primary mature cystic teratoma; (5) appendiceal mucocele with peritoneal dissemination mimicking an ovarian tumor with peritoneal carcinomatosis; (6) mucinous borderline tumor developing inside an ovarian teratoma; and (7) the association between a mucinous bilateral ovarian cancer and a colonic tumor.
CONCLUSIONS: In our study, we aimed to provide a comprehensive overview of the ovarian causes of pseudomyxoma peritonei, including its epidemiology, imagery characteristics, symptoms, current treatment, and promising future therapies, in the hopes of finding feasible solutions, as a lack of understanding of this mucus-secreting malignant disease increases the risk of delayed diagnosis or uncontrolled deterioration.
PMID:38672528 | PMC:PMC11047873 | DOI:10.3390/cancers16081446
Am Surg. 2024 Sep;90(9):2323-2324. doi: 10.1177/00031348241248697. Epub 2024 Apr 17.
ABSTRACT
Gastropleural fistulas are rare complications with significant mortality and morbidity. There are limited reports on the successful management of gastropleural fistulas with advanced endoscopic procedures. The following case of a 75-year-old woman with a history of recurrent pseudomyxoma peritonei secondary to ruptured low-grade appendiceal mucinous neoplasm status post cytoreductive surgery highlights the successful treatment of a gastropleural fistula with endoscopic suturing.
PMID:38631338 | DOI:10.1177/00031348241248697
Cancers (Basel). 2024 Apr 3;16(7):1406. doi: 10.3390/cancers16071406.
ABSTRACT
Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the appendix or other organ ruptures. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is currently the preferred treatment, with excellent efficacy and safety, and is associated with breakthrough progress in long-term disease control and prolonged survival. However, the high recurrence rate of PMP is the key challenge in its treatment, which limits the clinical application of multiple rounds of CRS-HIPEC and does not benefit from conventional systemic chemotherapy. Therefore, the development of alternative therapies for patients with refractory or relapsing PMP is critical. The literature related to PMP research progress and treatment was searched in the Web of Science, PubMed, and Google Scholar databases, and a literature review was conducted. The overview of the biological research, treatment status, potential therapeutic strategies, current research limitations, and future directions associated with PMP are presented, focuses on CRS-HIPEC therapy and alternative or combination therapy strategies, and emphasizes the clinical transformation prospects of potential therapeutic strategies such as mucolytic agents and targeted therapy. It provides a theoretical reference for the treatment of PMP and the main directions for future research.
PMID:38611084 | PMC:PMC11010892 | DOI:10.3390/cancers16071406
Langenbecks Arch Surg. 2024 Apr 8;409(1):113. doi: 10.1007/s00423-024-03309-9.
ABSTRACT
PURPOSE: Peritoneal surface malignancies (PSM) are commonly known to have a dismal prognosis. Over the past decades, novel techniques such as cytoreductive surgery (CRS), hyperthermic intraperitoneal chemotherapy (HIPEC), and pressurized intraperitoneal aerosol chemotherapy (PIPAC) have been introduced for the treatment of PSM which could improve the overall survival and quality of life of patients with PSM. The decision to proceed with CRS and HIPEC is often challenging due the complexity of the disease, the extent of the procedure, associated side effects, and potential risks. Here, we present our experience with CRS and HIPEC to add to the ongoing discussion about eligibility criteria, technical approach, and expected outcomes and contribute to the evolution of this powerful and promising tool in the multidisciplinary treatment of patients with primary and secondary PSM.
METHODS: A single-center retrospective chart review was conducted and included a total of 40 patients treated with CRS and HIPEC from April 2020 to September 2022 at the University Hospital Münster Department of Surgery. All patients had histologically confirmed primary or secondary peritoneal malignancies of various primary origins.
RESULTS: Our study included 22 patients with peritoneal metastases from gastric cancer (55%), 8 with pseudomyxoma peritonei (20%), 4 with mesothelioma of the peritoneum (10%), and 6 patients with PSM originating from other primary tumor locations. Median PCI at time of cytoreduction was 4 (0-25). Completeness of cytoreduction score was 0 in 37 patients (92.5%), 1 in two patients (5%), and 2 in one patient (2.5%). Median overall survival across all patients was 3.69 years.
CONCLUSION: Complete cytoreduction during CRS and HIPEC can be achieved for patients with low PCI, for patients with high PCI in low-grade malignancies, and even for patients with initially high PCI in high-grade malignancies following a significant reduction of cancer burden due to extensive preoperative treatment with PIPAC and systemic chemotherapy.
PMID:38589714 | DOI:10.1007/s00423-024-03309-9
Transfus Med Hemother. 2023 Sep 14;51(2):122-125. doi: 10.1159/000533625. eCollection 2024 Apr.
ABSTRACT
INTRODUCTION: DEL is known to be one of the weakest D variants, which can be detected by the adsorption-elution technique or by molecular study. Currently, in Thailand, we do not routinely test for DEL variants serologically or genetically among serologic RhD-negative blood donors.
CASE PRESENTATION: We reported 2 cases of alloimmunization after transfused with Rh DEL, RHD*DEL1 allele, in the Thai population. The first case was a 73-year-old male with anemia who presented with post-cardiac arrest and septic shock. The patient was group B, RhD-negative, and was transfused with RhD-negative red blood cells (RBCs). Antibody screening and identification found that the patient developed anti-D and anti-Mia during the admission course. The second case was a 38-year-old woman with pseudomyxoma peritonei who developed anti-D after receiving four units of RhD-negative RBCs during cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Both patients did not receive anti-D immunoglobulin and had no previous history of anti-D detection. We retrospectively investigated and found two units of RHD*DEL1 among the RBCs transfused to these patients.
DISCUSSION: Previous reports of several cases of anti-D alloimmunization in RhD-negative recipients transfused by RHD*DEL1, an Asian-type DEL, are limited only to East Asia. We first identified 2 patients with anti-D alloimmunization after receiving the RHD*DEL1 RBCs in the Thai population. This raises concern about Rh DEL screening among D-negative Thai blood donors and whether to remove DEL units from the D-negative inventory to improve patient safety.
PMID:38584693 | PMC:PMC10996056 | DOI:10.1159/000533625
World J Gastrointest Surg. 2024 Mar 27;16(3):944-954. doi: 10.4240/wjgs.v16.i3.944.
ABSTRACT
BACKGROUND: Appendiceal mucinous neoplasms (AMNs), although not classified as rare, are relatively uncommon tumors most often discovered incidentally during colorectal surgery. Accurate identification of AMNs is difficult due to non-specific symptoms, overlapping tumor markers with other conditions, and the potential for misdiagnosis. This underscores the urgent need for precision in diagnosis to prevent severe complications.
CASE SUMMARY: This case report describes the unexpected discovery and treatment of a low-grade AMN (LAMN) in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma (AC). Preoperatively, non-specific gastrointestinal symptoms and elevated tumor markers masked the presence of AMN. The tumor, presumed to be an AMN peritoneal cyst intraoperatively, was confirmed as LAMN through histopathological examination. The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile: Positive for Homeobox protein CDX-2, Cytokeratin 20, special AT-rich sequence-binding protein 2, and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8. This profile aids in distinguishing appendiceal and ovarian mucinous tumors. Postoperative recovery was uncomplicated, and the patient initiated adjuvant chemotherapy for the colon AC.
CONCLUSION: This case highlights the diagnostic complexity of AMNs, emphasizing the need for vigilant identification to avert potential complications, such as pseudomyxoma peritonei.
PMID:38577069 | PMC:PMC10989352 | DOI:10.4240/wjgs.v16.i3.944
J Minim Access Surg. 2024 Mar 28. doi: 10.4103/jmas.jmas_299_23. Online ahead of print.
ABSTRACT
INTRODUCTION: Pseudomyxoma peritonei (PMP) is a condition characterised by the presence of gelatinous tumour-like growth within the peritoneal cavity. Combined cytoreductive surgery and intraperitoneal chemotherapy have shown to improve the survival rate in PMP patients. However, post-operative complications such as cognitive dysfunction, respiratory insufficiency and acute renal failure are still observed. This retrospective study aims to explore the risk factors associated with major post-operative complications and specifically investigate the correlation with intraoperative hypotension.
PATIENTS AND METHODS: This retrospective cohort study included PMP patients treated at Beijing Aerospace Center Hospital from 1 June, 2014 to 30 December, 2020. The primary outcome measures were major post-operative complications, including neurological, pulmonary, cardiovascular, surgical complications, acute hepatic injury and acute kidney injuries. The secondary outcome measures included infection, fever and deep venous thrombosis. Statistical analysis was conducted using EmpowerStats and R software.
RESULTS: A total of 782 patients were screened, and 668 patients were included in the statistical analysis. Amongst them, 234 (35.03%) individuals experienced major post-operative complications. Factors such as pre-operative American Society of Anaesthesiologists grading, age, haemoglobin and albumin levels, intraoperative mean arterial pressure, blood loss, fluid replacement volume, method of intraperitoneal hyperthermic perfusion and post-operative requirement for intensive care unit mechanical ventilation were found to be correlated with major complications.
CONCLUSION: The identified risk factors provide valuable insights for improving clinical pathways in the management of PMP. Further prospective studies are warranted to establish the association between these factors and patient outcomes.
PMID:38557560 | DOI:10.4103/jmas.jmas_299_23
JPGN Rep. 2023 Dec 27;5(1):66-69. doi: 10.1002/jpr3.12017. eCollection 2024 Feb.
ABSTRACT
This case report describes a 17-year-old patient with a low-grade appendiceal mucinous neoplasm. The patient presented with non-bloody diarrhea, abdominal pain, and weight loss. A colonoscopy revealed a cecal polypoid mass that required laparoscopic surgery. The residual appendix was dilated with myxoglobulosis and histopathology confirmed the diagnosis of a low-grade appendiceal mucinous neoplasm staged pT3Nx. The potential risk of pseudomyxoma peritonei is a serious complication of these tumors. Surveillance plans include computed tomography abdomen and pelvis, and tumor markers every 6 months for the next 2 years. This case highlights the importance of considering appendiceal malignancy in patients with abdominal pain and weight loss, despite the rarity of the disease. It also emphasizes the need for careful monitoring due to the possible complications associated with these tumors. Treatment and prognosis for appendiceal neoplasms depend on the histopathologic characteristics, tumor-nodes-metastasis stage, tumor grade, and presence of peritoneal disease.
PMID:38545266 | PMC:PMC10964329 | DOI:10.1002/jpr3.12017
Cancer Med. 2024 Mar;13(6):e7101. doi: 10.1002/cam4.7101.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare clinical malignant syndrome, and its rarity causes a lack of pathology research. This study aims to quantitatively analyze HE-stained pathological images (PIs), and develop a new predictive model integrating digital pathological parameters with clinical information.
METHODS: Ninety-two PMP patients with complete clinic-pathological information, were included. QuPath was used for PIs quantitative feature analysis at tissue-, cell-, and nucleus-level. The correlations between overall survival (OS) and general clinicopathological characteristics, and PIs features were analyzed. A nomogram was established based on independent prognostic factors and evaluated.
RESULTS: Among the 92 PMP patients, there were 34 (37.0%) females and 58 (63.0%) males, with a median age of 57 (range: 31-76). A total of 449 HE stained images were obtained for QuPath analysis, which extracted 40 pathological parameters at three levels. Kaplan-Meier survival analysis revealed eight clinicopathological characteristics and 20 PIs features significantly associated with OS (p < 0.05). Partial least squares regression was used to screen the multicollinearity features and synthesize four new features. Multivariate survival analysis identified the following five independent prognostic factors: preoperative CA199, completeness of cytoreduction, histopathological type, component one at tissue-level, and tumor nuclei circularity variance. A nomogram was established with internal validation C-index 0.795 and calibration plots indicating improved prediction performance.
CONCLUSIONS: The quantitative analysis of HE-stained PIs could extract the new prognostic information on PMP. A nomogram established by five independent prognosticators is the first model integrating digital pathological information with clinical data for improved clinical outcome prediction.
PMID:38506243 | PMC:PMC10952024 | DOI:10.1002/cam4.7101
Br J Surg. 2024 Mar 2;111(3):znae045. doi: 10.1093/bjs/znae045.
NO ABSTRACT
PMID:38477594 | DOI:10.1093/bjs/znae045
Cancers (Basel). 2024 Feb 21;16(5):866. doi: 10.3390/cancers16050866.
ABSTRACT
The 5th edition of the World Health Organization (WHO) classification of tumors of the digestive system distinguishes four categories of appendiceal tumors (ATs): serrated lesions and polyps, mucinous neoplasms, adenocarcinomas, and neuroendocrine neoplasms (NENs). The differential diagnosis of ATs can be challenging in medical practice, due to their rarity and lack of data from randomized controlled trials on a large, diverse group of patients. ATs are usually noted in specimens obtained during appendectomies due to clinical acute appendicitis. In the European population, most ATs (65%) occur over the age of 50 and among women (56.8%). According to histological type, 54.6% are neuroendocrine tumors (NETs); 26.8% cystic, mucinous, and serous neoplasms; and 18.6% adenocarcinoma not otherwise specified (NOS). On pathologic analysis, most AT findings are benign lesions or small NENs that do not require further therapeutic measures. The presence of appendiceal mucinous neoplasm (AMN) can lead to pseudomyxoma peritonei (PMP). While the multimodal treatment for abdominal malignancies has evolved over the past several decades, the clinical workup and treatment of ATs remain a challenge. Therefore, this review aims to describe the diagnostic possibilities, molecular-based diagnosis, staging, differences in the treatment process, and prognostic factors associated with ATs.
PMID:38473228 | PMC:PMC10931490 | DOI:10.3390/cancers16050866
Cancer Diagn Progn. 2024 Mar 3;4(2):198-203. doi: 10.21873/cdp.10308. eCollection 2024 Mar-Apr.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is a clinical entity of subtle onset abdominal pain, ascites, and distention associated with characteristic imaging. In most cases, laparoscopic exploration will give the definitive diagnosis and histopathologic verification. However, usually there are difficulties in the diagnosis of this disease.
CASE REPORT: Herein, we present a case of a 51-year-old female who developed ascites over 5 months. An investigational laparotomy established the diagnosis of PMP, after the discovery of a mucinous, grey-brown tumor that was CK20 positive and CK7 negative. Subsequently, chemotherapy with oxaliplatin combined with 5-FU (FOLFOX4 regimen), was initiated and the patient survived for 30 months. We also present a comprehensive review of the English literature concerning the different symptoms and radiological findings of this rare entity. According to the literature review, 35 cases of PMP with different clinical and radiological findings have been described. In the majority of the cases, ultrasound, computed tomography or magnetic resonance imaging was orientating towards a proper diagnosis before a diagnostic laparotomy.
CONCLUSION: The combination of a clinical picture with the characteristic imaging findings enables a prompt diagnosis of PMP, making prognosis more favorable.
PMID:38434922 | PMC:PMC10905292 | DOI:10.21873/cdp.10308
Int J Surg Protoc. 2024 Jan 20;28(1):6-11. doi: 10.1097/SP9.0000000000000017. eCollection 2024 Mar.
ABSTRACT
BACKGROUND: Peritoneal metastases from gastrointestinal or gynecologic malignancy are a prominent part of the natural history of these diseases. Peritoneal metastases, if not effectively treated, will result in a decreased survival and cause an impaired quality of life. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment specifically designed to combat peritoneal metastases. A group of patients who, from a theoretical perspective, may benefit from HIPEC are those patients with a positive peritoneal cytology. In order to identify these patients at the time of a surgical intervention, a same day cytology is to be performed.
MATERIALS AND METHODS: The result of this test is to be available at or before the completion of the cancer resection. If the cytology is positive, the patient immediately becomes a candidate for HIPEC. The HIPEC will be of maximal value if a complete cytoreduction, as judged by the surgeon, has been possible. This phase 1 trial is to demonstrate that the Surgical Oncology Service, the Department of Pathology, the Pharmacy and the Operating Room personnel can co-ordinate a phase 1 protocol to successfully complete the same day cytology with an efficient delivery of HIPEC. A standardized plan for consent, cytology collection, preparation of the specimen, reading of the specimen, reporting the results in a timely manner facilitates the administration of HIPEC in peritoneal cytology positive patients.
DISSEMINATION: Successful completion of these requirements is a positive result for this study and allows for future protocols to be generated. Successful completion of the same day cytology phase 1 protocol will allow the efficacy, safety, and efficiency of this plan of patient management to be evaluated.
PMID:38433868 | PMC:PMC10905492 | DOI:10.1097/SP9.0000000000000017
JAMA Netw Open. 2024 Feb 5;7(2):e240260. doi: 10.1001/jamanetworkopen.2024.0260.
ABSTRACT
IMPORTANCE: Serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125) have been useful in the management of gastrointestinal and gynecological cancers; however, there is limited information regarding their utility in patients with appendiceal adenocarcinoma.
OBJECTIVE: To assess the association of serum tumor markers (CEA, CA19-9, and CA125) with clinical outcomes and pathologic and molecular features in patients with appendiceal adenocarcinoma.
DESIGN, SETTING, AND PARTICIPANTS: This is a retrospective cohort study at a single tertiary care comprehensive cancer center. The median (IQR) follow-up time was 52 (21-101) months. Software was used to query the MD Anderson internal patient database to identify patients with a diagnosis of appendiceal adenocarcinoma and at least 1 tumor marker measured at MD Anderson between March 2016 and May 2023. Data were analyzed from January to December 2023.
MAIN OUTCOMES AND MEASURES: Association of serum tumor markers with survival in patients with appendiceal adenocarcinoma. Cox proportional hazards regression analyses were also performed to assess associations between clinical factors (serum tumor marker levels, demographics, and patient and disease characteristics) and patient outcomes (overall survival).
RESULTS: A total of 1338 patients with appendiceal adenocarcinoma were included, with a median (range) age at diagnosis of 56.5 (22.3-89.6) years. The majority of the patients had metastatic disease (1080 patients [80.7%]). CEA was elevated in 742 of the patients tested (56%), while CA19-9 and CA125 were elevated in 381 patients (34%) and 312 patients (27%), respectively. Individually, elevation of CEA, CA19-9, or CA125 were associated with worse 5-year survival; elevated vs normal was 81% vs 95% for CEA (hazard ratio [HR], 4.0; 95% CI, 2.9-5.6), 84% vs 92% for CA19-9 (HR, 2.2; 95% CI, 1.4-3.4), and 69% vs 93% for CA125 (HR, 4.6; 95% CI, 2.7-7.8) (P < .001 for all). Quantitative evaluation of tumor markers was associated with outcomes. Patients with highly elevated (top 10th percentile) CEA, CA19-9, or CA125 had markedly worse survival, with 5-year survival rates of 59% for CEA (HR, 9.8; 95% CI, 5.3-18.0), 64% for CA19-9 (HR, 6.0; 95% CI, 3.0-11.7), and 57% for CA125 (HR, 7.6; 95% CI, 3.5-16.5) (P < .001 for all). Although metastatic tumors had higher levels of all tumor markers, when restricting survival analysis to 1080 patients with metastatic disease, elevated CEA, CA19-9, or CA125 were all still associated worse survival (HR for CEA, 3.4; 95% CI, 2.5-4.8; P < .001; HR for CA19-9, 1.8; 95% CI, 1.2-2.7; P = .002; and HR for CA125, 3.9; 95% CI, 2.4-6.4; P < .001). Interestingly, tumor grade was not associated with CEA or CA19-9 level, while CA-125 was slightly higher in high-grade tumors relative to low-grade tumors (mean value, 18.3 vs 15.0; difference, 3.3; 95% CI, 0.9-3.7; P < .001). Multivariable analysis identified an incremental increase in the risk of death with an increase in the number of elevated tumor markers, with an 11-fold increased risk of death in patients with all 3 tumor markers elevated relative to those with none elevated. Somatic mutations in KRAS and GNAS were associated with significantly higher levels of CEA and CA19-9.
CONCLUSIONS AND RELEVANCE: In this retrospective study of serum tumor markers in patients with appendiceal adenocarcinoma, CEA, CA19-9, and CA125 were associated with overall survival in appendiceal adenocarcinoma. Given their value, all 3 biomarkers should be included in the initial workup of patients with a diagnosis of appendiceal adenocarcinoma.
PMID:38416491 | PMC:PMC10902735 | DOI:10.1001/jamanetworkopen.2024.0260
Surg Laparosc Endosc Percutan Tech. 2024 Apr 1;34(2):196-200. doi: 10.1097/SLE.0000000000001267.
ABSTRACT
INTRODUCTION: Our study's objective was to provide the method for, and preliminary findings from, robot-assisted cytoreductive surgery (r-CRS) combined with upper-abdominal peritonectomy in pseudomyxoma peritonei (PMP) with limited peritoneal surface malignancy (PSM).
MATERIALS AND METHODS: We conducted a retrospective pilot study on consecutive patients with PSM secondary to pseudomyxoma peritonei with a peritoneal cancer index (PCI) of under 10 who were indicated for r-CRS combined with upper-abdominal peritectomy. Perioperative and 30-day major morbidity and mortality characteristics were analyzed and compared with cases in which laparoscopic CRS (l-CRS) was performed under the same conditions.
RESULTS: Six patients underwent r-CRS combined with an upper-abdominal partial peritonectomy. Their mean PCI was 4.83. Complete cytoreduction was achieved in all patients. The mean duration of the operation was 156.8 minutes. There was no major complication and no mortality. The mean length of hospital stay after surgery was 6.33 days. r-CRS resulted in less blood loss, a lower C-reactive protein level, and a shorter length of hospital stay that were significantly different compared with those of l-CRS.
CONCLUSIONS: This is the initial technical report of a robotic approach for CRS combined with upper-abdominal peritonectomy. r-CRS with a combined upper-abdominal peritonectomy was shown to be safe and feasible for PMP with limited PSM.
PMID:38417107 | DOI:10.1097/SLE.0000000000001267
Cureus. 2024 Jan 25;16(1):e52908. doi: 10.7759/cureus.52908. eCollection 2024 Jan.
ABSTRACT
Appendicular mucinous neoplasms, constituting less than 1% of gastrointestinal tract neoplasms, are heterogeneous entities. They may be asymptomatic, discovered incidentally, or present as large tumors due to mucin accumulation. The lack of standardized treatment complicates management. Imaging studies, particularly CT scans, are crucial for diagnosis and follow-up. This case report presents two clinical cases of women in their sixth and seventh decades of life with a history of lower gastrointestinal bleeding, mild anemia in laboratory studies, and incomplete colonoscopies. The diagnosis, confirmed through CT scans, led to the decision for surgical intervention in both cases, involving laparoscopic right hemicolectomy with ileotransverse anastomosis. Subsequently, histopathological reports confirmed the diagnosis of high-grade appendicular mucinous neoplasms, and a follow-up plan was established with imaging studies every six months with no recurrence at two years. Over 50% of appendicular tumors are mucinous neoplasms originating from low-grade mucinous neoplasms. Given the low lymph node invasion (2%), appendectomy may suffice if the entire tumor is excised. Extensive resections or right hemicolectomy are reserved for larger tumors or high-grade neoplasms to minimize local recurrence risk. Mucinous neoplasms with acellular mucin and peritoneal invasion may require cytoreduction or right hemicolectomy, while those with mucinous epithelium may need hyperthermic intraperitoneal chemotherapy (HIPEC) due to the risk of local recurrence, worsened by the presence of extra appendiceal epithelial cells. Disease-free and overall survival depend on treatment and initial lesion characterization. A five-year survival rate of 86% is reported for low-grade mucinous neoplasms. Follow-up approaches lack an ideal standard, generally involving physical examinations and imaging studies every six months to one year during the first six years.
PMID:38406052 | PMC:PMC10893774 | DOI:10.7759/cureus.52908
Indian J Pathol Microbiol. 2024 Feb 14. doi: 10.4103/ijpm.ijpm_1255_21. Online ahead of print.
ABSTRACT
Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal mucinous neoplasm. They exhibit CK7-/CK20+ immunophenotype similar to carcinomas of the lower gastrointestinal tract. In this report, we have described a case of a well-differentiated mucinous carcinoma arising within a mature cystic teratoma. The mucinous carcinoma showed a spectrum of morphological patterns, including cystadenoma-like areas, proliferative/borderline areas, villous adenoma-like areas, and areas of invasive carcinoma. In addition, our case showed some unusual findings, namely, diffuse CK7 positivity, associated pseudomyxoma peritonei, and metastasis to lungs. These features were not demonstrated in any of the previously reported cases. Our case shows that the teratomatous mucinous neoplasm of the ovary may show CK7+/CK20+/CDX2 + immunoprofile making it immunohistochemically indistinguishable from a primary ovarian mucinous neoplasm or a metastatic mucinous carcinoma of the lower gastrointestinal tract.
PMID:38391380 | DOI:10.4103/ijpm.ijpm_1255_21
Front Oncol. 2024 Feb 8;14:1323796. doi: 10.3389/fonc.2024.1323796. eCollection 2024.
ABSTRACT
OBJECTIVE: Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This study aimed to explore the current situation and frontier trend of PMP through bibliometric and visualization analysis, and to indicate new directions for future research.
METHODS: The original research articles and reviews related to the PMP research were downloaded from Web of Science Core Collection on September 11, 2023. CiteSpace (6.2.R4) and VOSviewer(1.6.18) were used to perform bibliometric analysis of the publications, and establish the knowledge map. The data collected was analyzed using the Online Analysis Platform of Bibliometric to evaluate the cooperation of countries in this field.
RESULTS: We identified 1449 original articles and reviews on PMP published between 1998 and 2023. The number of publications on PMP increased continuously. The United States, the United Kingdom and China were the top contributors. The most productive organization was the MedStar Washington Hospital Center. Sugarbaker, Paul H. was the most prolific author and the most cited. Keyword analysis showed that "Pseudomyxoma peritonei", "cancer", "cytoreductive surgery", and "hyperthermic intraperitoneal chemotherapy" were the most common keywords. The earliest and latest used keywords were "mucinous tumors" and "impact", respectively. "classification", "cytoreductive surgery", "appendiceal" were the top 3 strongest citation bursts. The reference "Carr NJ, 2016, AM J SURG PATHOL" had the highest co-citations.
CONCLUSION: This bibliometric analysis showed an increasing trend in literature related to PMP. The research trends and hotspots identified in this study could guide the future research directions in this field, in order to promote the development of PMP.
PMID:38390264 | PMC:PMC10883648 | DOI:10.3389/fonc.2024.1323796
Am J Surg. 2024 Apr;230:78-81. doi: 10.1016/j.amjsurg.2024.01.031. Epub 2024 Feb 6.
ABSTRACT
INTRODUCTION: The treatment of peritoneal malignancies has evolved and select patients can undergo effective surgical therapies. Access to innovative oncology procedures can be improved if programs are developed within and outside of academic cancer centers. We report the creation of a high volume, comprehensive peritoneal malignancy program developed in a community center.
METHODS: A retrospective single-site study was conducted using registry data comprising all patients who underwent Cytoreductive Surgery (CRS) and Heated Intraperitoneal Chemotherapy (HIPEC) between October 2011-December 2021.
RESULTS: 353 patients underwent CRS and HIPEC. 208 patients experienced in-hospital morbidity (58.9 ​%). Group comparison by disease site, PCI, and the completeness of cytoreduction demonstrated survival differences in mean overall survival and disease-free survival in 1-, 3- and 5-year ranges.
CONCLUSIONS: This study demonstrates that with a standardized protocol and a surgeon-led multidisciplinary team it is possible to offer safe outcome driven, complex oncologic surgery in a community-based cancer program.
PMID:38369417 | DOI:10.1016/j.amjsurg.2024.01.031
Ann Surg Oncol. 2024 May;31(5):3325-3338. doi: 10.1245/s10434-023-14850-0. Epub 2024 Feb 10.
ABSTRACT
BACKGROUND: Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP.
METHODS: Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed.
RESULTS: Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively).
CONCLUSION: These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.
PMID:38341381 | PMC:PMC10997733 | DOI:10.1245/s10434-023-14850-0
Ann Surg Oncol. 2024 Feb 9. doi: 10.1245/s10434-024-14925-6. Online ahead of print.
NO ABSTRACT
PMID:38336936 | DOI:10.1245/s10434-024-14925-6
World J Clin Cases. 2024 Jan 16;12(2):361-366. doi: 10.12998/wjcc.v12.i2.361.
ABSTRACT
BACKGROUND: Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic appendicitis before surgery. The primary diagnostic tool for LAMN is abdominal computed tomography (CT) imaging. Surgical resection remains the cornerstone of LAMN management, necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture. Laparoscopy, known for its minimal invasiveness, reduced postoperative discomfort, and expedited recovery, is a safe and reliable approach for LAMN treatment. Despite the possibility of pseudomyxoma peritonei development, appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes, which can be attributed to the disease's slow growth and lower malignancy.
CASE SUMMARY: A 71-year-old male patient was admitted to our hospital with a pelvic space-occupying lesion detected 1 mo prior. Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses. No shifting dullness was noted, and digital rectal examination revealed no palpable mass. Enteroscopy revealed a raised, smooth-surfaced mass measuring 3.0 cm in the cecum. Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows. Laparoscopic surgery was performed and revealed a significantly dilated appendix, leading to laparoscopic resection of the appendix and part of the cecum. Post-surgical pathologic analysis confirmed LAMN. The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding, intestinal obstruction, or incision infection. No tumor recurrence was observed during a 7-mo follow-up period.
CONCLUSION: LAMN is a rare disease that lacks specific clinical manifestations. Abdominal CT plays a crucial role in diagnosing LAMN, and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
PMID:38313642 | PMC:PMC10835689 | DOI:10.12998/wjcc.v12.i2.361
Gan To Kagaku Ryoho. 2023 Dec;50(13):1516-1518.
ABSTRACT
Pseudomyxoma peritonei(PMP)is a rare condition caused by intraductal papillary mucinous neoplasm(IPMN). At our institution, 3 cases, along with literature review, of PMP caused by IPMN were treated with cytoreductive surgery. In case 1, a 70-year-old man was pathologically diagnosed with low-grade PMP with intraductal papillary mucinous carcinoma. Recurrence was discovered 68 months after surgery, and the patient died after 78 months. In case 2, a 69-year-old man was pathologically diagnosed with high-grade PMP with signet-ring cell carcinoma caused by intraductal papillary mucinous carcinoma. The patient died 8 months post-surgery. In case 3, a 77-year-old woman was pathologically diagnosed with low- grade(partially high-grade)PMP with intraductal papillary mucinous adenoma. There was a recurrence 14 months post- surgery. The patient is still alive because of systemic chemotherapy 32 months after surgery.
PMID:38303326
World J Surg Oncol. 2024 Jan 31;22(1):39. doi: 10.1186/s12957-024-03318-4.
ABSTRACT
BACKGROUND: The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff point for PCI to predict surgical resectability of PMP.
METHODS: A total of 366 PMP patients were included. The patients were divided into low-grade and high-grade groups. Based on the completeness of the cytoreduction (CC) score, both low-grade and high-grade PMP patients were further divided into complete cytoreductive surgery (CRS) and maximal tumor debulking (MTD) subgroups. The ability to predict surgical resectability of total and selected PCI (regions 2 + 9 to 12) was analyzed through receiver operating characteristic (ROC) curves.
RESULTS: Both total and selected PCI demonstrated excellent discriminative ability in predicting surgical resectability for low-grade PMP patients (n = 266), with the ROC-AUC of 0.940 (95% CI: 0.904-0.965) and 0.927 (95% CI: 0.889-0.955). The corresponding optimal cutoff point was 21 and 5, respectively. For high-grade PMP patients (n = 100), both total and selected PCI exhibited good performance in predicting surgical resectability, with the ROC-AUC of 0.894 (95% CI: 0.816-0.946) and 0.888 (95% CI: 0.810-0.943); correspondingly, the optimal cutoff point was 25 and 8, respectively. The discriminative ability between total and selected PCI in predicting surgical resectability did not show a statistical difference.
CONCLUSIONS: Both total and selected PCI exhibited good performance and similarity in predicting complete surgical resection for both low-grade and high-grade PMP patients. However, the selected PCI was simpler and time-saving in clinical practice. In the future, new imaging techniques or predictive models may be developed to better predict PCI preoperatively, which might assist in confirming whether complete surgical resection can be achieved.
PMID:38297355 | PMC:PMC10829395 | DOI:10.1186/s12957-024-03318-4
Oncology. 2024 Jan 23. doi: 10.1159/000536219. Online ahead of print.
ABSTRACT
INTRODUCTION: Pseudomyxoma peritonei (PMP) is a disease characterized by progressive accumulation of intraperitoneal mucinous ascites produced by neoplasms in the abdominal cavity. Since the prognosis of patients with PMP remain unsatisfactory, the development of effective therapeutic drug(s) is a matter of pressing concern. Genetic analyses of PMP have clarified the frequent activation of GNAS and/or KRAS. However, the involvement of global epigenetic alterations in PMPs has not been reported.
METHODS: To clarify the genetic background of the 15 PMP tumors, we performed genetic analysis using AmpliSeq Cancer HotSpot Panel v2. We further investigated global DNA methylation in the 15 tumors and eight non-cancerous colonic epithelial cells using Methylation EPIC array BeadChip (Infinium 850k) containing a total of 865,918 probes.
RESULTS: This is the first report of comprehensive DNA methylation profiles of PMPs in the world. We clarified that the 15 PMPs could be classified into at least two epigenotypes, unique methylation epigenotype (UME) and normal-like methylation epigenotype (NLME), and that genes associated with neuronal development and synaptic signaling may be involved in the development of PMPs. In addition, we identified a set of hypermethylation marker genes such as HOXD1 and TSPYL5 in the 15 PMPs.
CONCLUSIONS: These findings may help the understanding of the molecular mechanism(s) of PMP and contribute to the development of therapeutic strategies for this life-threatening disease.
PMID:38262376 | DOI:10.1159/000536219
J Cancer Res Ther. 2024 Jan 22. doi: 10.4103/jcrt.jcrt_191_23. Online ahead of print.
ABSTRACT
BACKGROUND: Pseudomyxoma peritonei (PMP) is an unusual clinical condition typically presenting with widespread mucinous neoplastic lesions within the peritoneum resulting in gelatin material-rich ascites. It was first described by Werth in 1884. Ever since, its clinical presentation, definition, site of origin, and prognosis have been a subject of debate. However, many histopathologic, immunohistochemical, and genetic studies have attempted to locate the primary lesion in the appendix in both genders.
OBJECTIVES: To analyze the histological origin and survival outcomes of pseudomyxoma peritonei in patients treated at a regional cancer center.
MATERIALS AND METHODS: Fifteen cases of PMP were diagnosed during the five-year study period. The demographic and clinicopathological details were retrieved; the slides were reviewed and histological parameters reassessed. Descriptive statistics were used to express proportions. Continuous variables were recorded as mean (SD) or median (IQR). Kaplan-Meier (KM) curve was used to estimate overall survival.
RESULTS: Mean age for PMP was found to be 47.5 years for low grade Mucinous Carcinoma Peritonei (MCP), 54.2 years for high grade MCP, and 58 years for high grade MCP with signet ring cells. Most common overall presentation was abdominal distension in 53.3% (8/15) of cases, followed by acute appendicitis in 20% (3/15) cases. PMP was detected synchronous with the primary tumor in 9/15 cases (60%). Primary lesion in the appendix was grossly identified in 7/15 cases, while it was not explored in the remaining eight cases. Yet, by combined clinical, radiological, histopathological, and immunohistochemical analysis, we identified that most of the cases (14/15) had an appendiceal origin (93.3%). The overall survival for 12 months was 50% and for 18 months was 37%.
CONCLUSION: The surgeon and radiologist may well bear in mind the most common possibility of an appendiceal origin for PMP and resect the appendix, irrespective of the presence of a grossly or radiologically detectable lesions. We emphasize that immunohistochemistry helped to detect the site of origin even when the primary was occult.
PMID:38261429 | DOI:10.4103/jcrt.jcrt_191_23
ANZ J Surg. 2024 Apr;94(4):614-620. doi: 10.1111/ans.18833. Epub 2024 Jan 19.
ABSTRACT
BACKGROUND: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard of care for selected cases of peritoneal surface malignancy. However, due to its morbidity and learning curve, it is only delivered in six centres in Australia and Aotearoa New Zealand (AoNZ). In this study, we report peri-operative morbidity and mortality following CRS/HIPEC at Waikato and Braemar Hospitals, which have treated patients from all regions of AoNZ since 2008.
METHODS: We retrospectively reviewed a database of all patients undergoing CRS and HIPEC from 01/01/2008 to 01/11/2020 at Waikato and Braemar Hospitals.
RESULTS: Two-hundred and forty procedures were performed for 221 patients with a mean age of 55, including 22 (9.2%) re-do procedures. One hundred and eighty-six cases were European, 32 were MÄori, and 16 were Pasifika. There were 152 pseudomyxoma peritonei, 39 colorectal adenocarcinomas, 29 appendiceal cancers, 8 ovarian cancers, 6 peritoneal mesothelioma, and 6 other tumour types. The median PCI was 16. HIPEC was administered to 196 out of 196 CC0/1 cases (100%) and 3 out of 44 CC2/3 cases (6.8%). Fifty-six cases (23.3%) had at least one major complication. There were two mortalities (0.8%) within 30 days. The median length of stay was 11 days. Operative duration was identified as an independent risk factor for major complications. There was considerable variation in the number of referrals from different regions of AoNZ. Over time, a decline in major complication rate is seen with increased case volume.
CONCLUSION: The Waikato region has achieved favourable short-term outcomes following CRS/HIPEC.
PMID:38240147 | DOI:10.1111/ans.18833