Mucocoele of the appendix: A case report

Fetched: June 15th, 2021, 5:02am GMT by Fareeha Farooqui

J Pak Med Assoc. 2021 Apr;71(4):1260-1263. doi: 10.47391/JPMA.01.

ABSTRACT

Mucinous cystadenoma is a rare tumour of the appendix. It accounts for only 0.4% of the gastrointestinal tract malignancies and is reported rarely in the literature. Therefore, surgical management is not yet established. Here we report the case of a 65-year-old female who presented with a dragging sensation and a feeling of a mass in the right iliac fossa. Her computed tomography (CT) suggested formation of an abscess in the parietal peritoneum. She was scheduled for laparotomy and upon exploration, a mass was found arising from the tip of the retroperitoneal appendix. The whole of the appendix was studded with mucoid material. Right hemicolectomy was performed and histopathology of the appendix showed mucinous cystadenoma with no evidence of malignant changes. The recovery was uneventful and the patient was discharged on the fourth post-operative day. The unusual presentation of retroperitoneal pseudomyxoma without any intraperitoneal pathology prompted us to report this case.

PMID:34125785 | DOI:10.47391/JPMA.01
Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in the management of peritoneal surface malignancies - An evidence-based review

Fetched: June 13th, 2021, 5:01am GMT by Mukur Dipi Ray

Curr Probl Cancer. 2021 Apr 15:100737. doi: 10.1016/j.currproblcancer.2021.100737. Online ahead of print.

ABSTRACT

BACKGROUND: Traditionally, peritoneal surface malignancies (PSM) were considered terminal diseases because of their advanced nature, therefore, systemic chemotherapy was given with palliative intent only. As a result, very poor survival outcomes were observed. But with the introduction of complete Cytoreductive surgery (CRS) and Hyperthermic intraperitoneal chemotherapy (HIPEC), the scenario has changed dramatically.

METHODOLOGY: An objective electronic database search was performed in Pubmed, NLM Catalog, Google scholar, Bookshelf, and Pubmed Central published in the time period from 2000 till 2020. All the randomized studies were included. In the absence of randomized studies, both prospective and retrospective studies were included. The outcomes of HIPEC were measured in terms of median survival, disease-free survival, overall survival, complications and drug toxicities.

RESULTS: CRS and HIPEC are considered the standard of care for PMP and MPM even in the absence of level 1 evidence due to lack of an effective alternative treatment. In colorectal and gastric cancer, several phase-three trials are showing overall survival benefit in selected cases while there is a prophylactic and palliative role of HIPEC in gastric cancer. Three reported phase 3 trials showed positive results in ovarian cancer. In peritoneal sarcomatosis, the role of HIPEC is yet to be proven.

CONCLUSION: The patient selection is the key to the successful outcomes after HIPEC. HIPEC should be performed by the experienced surgeons in specialized centres with a strong critical care and intensive care support to reduce the morbidity and mortality. Ongoing trials and future directions will prove to be an indispensable arm in the oncological armamentarium.

PMID:34116836 | DOI:10.1016/j.currproblcancer.2021.100737
Development of stress-induced cardiomyopathy after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Fetched: June 11th, 2021, 5:01am GMT by Saad Ahmed Naved

J Pak Med Assoc. 2021 Jun;71(6):1686-1688. doi: 10.5455/JPMA.05-749.

ABSTRACT

Pseudomyxoma Peritonei, a massive mucinous peritoneal collection due to a rare epithelial neoplasm, can be effectively treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS-HIPEC). A 43-year-old female, previously treated for mucinous ovarian carcinoma with CRS-HIPEC, and total abdominal hysterectomy and bilateral salpingo-oophorectomy, presented with new-onset abdominal distension and early satiety. She was diagnosed with Pseudomyxoma Peritonei. After 48 hours of treatment with CRS-HIPEC, she presented haemodynamically unstable with acute chest pain. Electrocardiogram showed broad complex tachycardia with ST depression in leads V3-6. Severe systolic dysfunction with Ejection Fraction (EF) of 20% along with severe pulmonary hypertension, visualized on Echocardiography. A diagnosis of Stress-induced Cardiomyopathy was established using InterTAK Diagnostic Score. Patients with CRS-HIPEC have presented with Stress-induced Cardiomyopathy. However, no specific relation between the two has been established. This case report discusses Stress-induced Cardiomyopathy as a complication of CRS-HIPEC.

PMID:34111099 | DOI:10.5455/JPMA.05-749
Pseudomyxoma retroperitonei masquerading as a psoas abscess

Fetched: June 8th, 2021, 5:02am GMT by Padma Vikram Badhe

BMJ Case Rep. 2021 Jun 2;14(6):e240299. doi: 10.1136/bcr-2020-240299.

ABSTRACT

A 52-year-old woman was referred to our department with multiple discharging sinuses and swelling in the right flank and iliac region for the past year. Ultrasound revealed a large collection in the right psoas muscle with the sinus tract reaching up to the skin in the right iliac region. Despite repeated attempts at drainage, the collection continued to increase in size. CT was requested and revealed a large heterogeneous irregular collection in the right psoas with fistulous communication with the cecum and skin with the erosion of the overlying ilium. Because of lack of vertebral involvement, enhancing internal septations, non-visualisation of the appendix and feculent material admixed with mucinous discharge from the sinus, pseudomyxoma retroperitonei secondary to ruptured mucinous neoplasm of the appendix was suspected. Mucoid material at the local site was sent for histopathology, which confirmed our suspicion. Our treatment plan after ileostomy was cytoreductive surgery along with adjuvant radiotherapy (40 Gy in 20 fractions) with chemotherapy (5-fluorouracil and folinic acid given for 30 weeks, once a week). However, after ileostomy, the patient refused further treatment, citing financial reasons.

PMID:34083186 | PMC:PMC8174495 | DOI:10.1136/bcr-2020-240299
Feasibility and outcomes of cytoreductive surgery and HIPEC for peritoneal surface malignancies in low- and middle-income countries: a single-center experience of 232 cases

Fetched: June 7th, 2021, 5:01am GMT by Suryanarayana Deo

World J Surg Oncol. 2021 Jun 5;19(1):164. doi: 10.1186/s12957-021-02276-5.

ABSTRACT

BACKGROUND: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has recently emerged as a viable management option for peritoneal surface malignancy (PSM). CRS and HIPEC is a complex, multidisciplinary and resource-intensive surgical procedure. It has a steep learning curve and is associated with significant morbidity and mortality. The expertise is mostly limited to few dedicated high-volume centers located in developed countries. We present a single institutional experience of 232 cases of CRS and HIPEC performed at a tertiary care cancer center in a low- and middle-income country (LMIC).

METHODS: A multidisciplinary PSM program was initiated in 2015 at a high-volume public-sector tertiary care cancer center in North India catering largely to patients belonging to low- and middle-income groups. Perioperative protocols were developed, and a prospective structured database was created to capture data. All patients undergoing CRS and HIPEC between January 2015 and December 2020 were identified, and the data was retrospectively analyzed for clinical spectrum, surgical details, and perioperative morbidity and mortality.

RESULTS: Two hundred and thirty-two patients underwent CRS and HIPEC during the study period. Epithelial ovarian carcinoma (56.5%) was the most common malignancy treated, followed by pseudomyxoma peritonei (18.5%), colorectal carcinoma (13.4%), and malignant mesothelioma (5.6%). Optimal CRS could be achieved in 94.4% of patients. Cisplatin and mitomycin were the most common drugs used for HIPEC. A total of 28.0% of patients had morbidity including deep vein thrombosis, subacute intestinal obstruction, sepsis, burst abdomen, lymphocele, urinoma, acute renal failure, and enterocutaneous fistula. The overall treatment-related mortality was 3.5%.

CONCLUSIONS: Results of the current study indicate that it is feasible to establish a successful CRS and HIPEC program for PSM in government-funded hospitals in LMIC facing resource constraints. The most common indication for CRS and HIPEC were carcinoma of the ovary followed by pseudomyxoma peritonei and colorectal carcinoma. Overall morbidity and mortality in the current series are comparable to global standards, reported from high-income countries. A protocol-based multidisciplinary team approach, optimal patient selection, and surgical expertise can help achieve optimal outcomes in government-funded hospitals in LMIC.

PMID:34090452 | PMC:PMC8180169 | DOI:10.1186/s12957-021-02276-5
Incidental diagnosis of a low-grade mucinous appendicular neoplasm: A case report

Fetched: May 31st, 2021, 5:01am GMT by Gabriel A Guzmán

Int J Surg Case Rep. 2021 May 24;83:105998. doi: 10.1016/j.ijscr.2021.105998. Online ahead of print.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Low-Grade Appendiceal Mucinous Neoplasms (LAMN) are noninvasive epithelial tumors of the appendix. Symptomatology is unspecific and can simulate appendicitis. The multidisciplinary approach for the correct diagnosis and management of this kind of tumor is important, as the prognosis depends on it.

PRESENTATION OF THE CASE: A 70-years-old man with an appendiceal picture is presented. McBurney and Blumberg's signs were found and the Echography has reported an acute appendicitis. He underwent laparoscopic surgery, founding a Tumor in the Cecum of 10 × 5 cm. We decided to perform an appendectomy with a total cecectomy. The pathological diagnosis was a LAMN. Ten months later, a Computed Tomography (CT) Scan control was performed reporting no evidence of Tumoral activity or Distance Metastasis, neither pseudomyxoma peritonei (PMP) evidence.

DISCUSSION: LAMN has been a very controversial tumor. The definitive management is appendectomy. There are controversies about the laparoscopic and open approach, and when there are positive margins. The stage was a pTis (LAMN) pNx according to the 8th edition of the American Joint Committee on Cancer (AJCC). The prognosis depends on the tumoral stage and the presence of PMP. In our case, we have performed a CT scan follow up and there was no Tumoral activity or Distance Metastasis, neither PMP evidence.

CONCLUSION: LAMN is a very interesting and rare tumor. The diagnosis of this case was a real challenge. Our management was simple due to the tumoral stage. Pathology played a very important role in treatment and prognosis.

PMID:34052715 | PMC:PMC8176355 | DOI:10.1016/j.ijscr.2021.105998
ASO Author Reflections: Managing Recurrent Pseudomyxoma Peritonei in 430 Patients After Complete Cytoreduction and HIPEC

Fetched: May 28th, 2021, 5:01am GMT by Nima Ahmadi

Ann Surg Oncol. 2021 May 26. doi: 10.1245/s10434-021-10179-8. Online ahead of print.

NO ABSTRACT

PMID:34041627 | DOI:10.1245/s10434-021-10179-8
Managing Recurrent Pseudomyxoma Peritonei in 430 Patients After Complete Cytoreduction and HIPEC: A Dilemma for Patients and Surgeons

Fetched: May 28th, 2021, 5:01am GMT by Nima Ahmadi

Ann Surg Oncol. 2021 May 26. doi: 10.1245/s10434-021-10093-z. Online ahead of print.

ABSTRACT

BACKGROUND: Epithelial appendiceal neoplasms are uncommon peritoneal malignancies causing a spectrum of disease including pseudomyxoma peritonei (PMP). The optimal management is cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Despite complete CRS (CCRS), recurrence develops in almost 45% of patients. No consensus exists for the optimal treatment of recurrent disease, with treatment strategies including repeat CRS, watch-and-wait, and palliative chemotherapy. This report aims to describe evolving management strategies for a large cohort with recurrence after CCRS.

METHODS: This retrospective study analyzed a prospective database of patients with recurrence after CCRS for appendiceal neoplasms from 1994 to 2017 who had long-term follow-up evaluation with tumor markers and computed tomography (CT).

RESULTS: Overall, 430 (37.6%) of 1145 PMP patients experienced recurrence at a median of 19 months. Of these 430 patients 145 (33.7%) underwent repeat CRS, 119 (27.7%) had a watch-and-wait approach, and 119 (27.7%) had palliative chemotherapy. The patients with recurrence had a median overall survival (OS) of 39 months, a 3-year survival of 74.6%, a 5-year survival of 57.4%, and a 10-year survival of 36.5%. In the multivariate analysis, the patients who had recurrence within 1 year after primary CRS (hazard ratio [HR], 3.55), symptoms at recurrence (HR, 3.08), a high grade of disease or adenocarcinoma pathology (HR, 2.94), signet ring cells (HR, 1.91), extraperitoneal metastatic disease (HR, 1.71), or male gender (HR, 1.61) had worse OS. The OS was longer for the patients who had repeat CRS (HR, 0.41). The patients who underwent repeat CCRS had a 3-year OS of 87.5%, a 5-year OS of 78.1%, and a 10-year OS of 67.9%.

CONCLUSIONS: Dilemmas persist around the optimal management of patients with recurrence after CRS and HIPEC for appendiceal tumors. Selected patients benefit from repeat CRS, particularly those with favorable tumor biology and focal disease.

PMID:34041626 | DOI:10.1245/s10434-021-10093-z
Complete Cytoreductive Surgery vs. Debulking Surgery for pseudomyxoma peritonei of appendiceal origin: A propensity score-matched study based on a single-center experience

Fetched: May 27th, 2021, 5:02am GMT by Ruiqing Ma

Eur J Surg Oncol. 2021 May 19:S0748-7983(21)00491-1. doi: 10.1016/j.ejso.2021.05.015. Online ahead of print.

ABSTRACT

OBJECTIVE: This study aimed to report the prognostic predictors and compare the long-term outcomes of complete cytoreductive surgery (CCRS) vs. debulking surgery (DS) in patients with pseudomyxoma peritonei (PMP) of appendiceal origin.

METHODS: A retrospective analysis of 1008 consecutive patients with PMP undergoing primary surgery from January 2008 to December 2019 was performed. A propensity score-matched (PSM) analysis (1:1) was performed, and oncologic outcomes were compared between the CCRS and DS groups.

RESULTS: Out of 1008 patients, 258 patients were excluded. Baseline characteristics differed significantly between the CCRS and DS groups (total n = 750). After PSM, 106 patients were selected from each group and the baseline characteristics were matched between groups. There were significant differences between groups in operative time, the incidence of major complications (P = 0.017), and the numbers of organs removed. The median follow-up was 28 (1-131) months. Median overall survival (OS) for the 212 patients was 52.0 months (95% CI 40.2-63.8), and 10-year OS was 39.0%. Median OS could not be calculated for the CCRS group; in the DS group, this value was 41 months (P = 0.010). The 10-year OS rate was 54.2% in the CCRS group and 31.2% in the DS group. Multivariate analyses identified CCRS (P = 0.012) and histopathologic subtype (P < 0.001) as independent prognostic factors for OS.

CONCLUSIONS: In this matched-pair analysis of patients with appendiceal PMP, CCRS was safe and associated with better prognosis than DS. The completeness of cytoreduction and histopathologic subtype were two independent prognostic factors for OS.

PMID:34034942 | DOI:10.1016/j.ejso.2021.05.015
Novel Use of Bromelain and Acetylcysteine (BromAc) for Pleural Involvement in Pseudomyxoma Peritonei

Fetched: May 13th, 2021, 5:02am GMT by Anthony R Lam

Case Rep Oncol. 2021 Mar 31;14(1):628-633. doi: 10.1159/000514273. eCollection 2021 Jan-Apr.

ABSTRACT

Pseudomyxoma peritonei (PMP) is a rare mucinous disease most commonly arising from the appendix. Pleural involvement arising from established PMP is seen in a small number of cases. Combined cytoreductive surgery and hyperthermic intrathoracic chemotherapy is the treatment of choice when managing intra-thoracic PMP. In cases of recurrence, surgical intervention may be technically challenging and carry higher rates of complications, morbidity, and mortality. Bromelain and acetylcysteine (BromAc^®) is a novel treatment modality that has demonstrated mucolytic properties. When injected directly into mucinous disease, it facilitates tumour dissolution and allows it to be aspirated. It has recently been tested in the treatment of inoperable peritoneal mucinous disease, with an acceptable safety profile and positive objective response. Here we describe the first two cases of BromAc^® administered directly into pleural adenomucinosis, with striking differences in response between the two patients likely due to differences in tumour hardness.

PMID:33976645 | PMC:PMC8077436 | DOI:10.1159/000514273
Clinicopathological Characteristics and Prognostic Prediction in Pseudomyxoma Peritonei Originating From Mucinous Ovarian Cancer

Fetched: May 11th, 2021, 5:01am GMT by Fengxian Fu

Front Oncol. 2021 Apr 21;11:641053. doi: 10.3389/fonc.2021.641053. eCollection 2021.

ABSTRACT

OBJECTIVE: To describe the clinicopathological characteristics of mucinous ovarian cancer (MOC)-derived pseudomyxoma peritonei (PMP) and identify prognostic factors for survival.

METHODS: Medical records from patients with MOC-derived PMP who attended the Aerospace Center Hospital, Beijing, China between January 2009, and December 2019 were retrospectively reviewed. Survival analysis was performed with the Kaplan-Meier method, the log-rank test, and a Cox proportional hazards model.

RESULTS: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for PMP originating from MOC were performed on 22 patients, who had a median age of 52 years at the time of surgery. At the last follow-up in June 2020, 9 (41%) patients were still alive. Median OS was 12 months (range, 1 to 102 months), and the 2-, 3-, and 5-year survival rates were 23, 9, and 5%, respectively.

CONCLUSION: Histopathologic subtype and PCI may be applied as predictors of prognosis in patients with MOC-derived PMP. Patients with high-grade disease could benefit from completeness of cytoreduction (CCR) 0/1.

PMID:33968739 | PMC:PMC8097027 | DOI:10.3389/fonc.2021.641053
New insights in the pathology of peritoneal surface malignancy

Fetched: May 11th, 2021, 5:01am GMT by Norman John Carr

J Gastrointest Oncol. 2021 Apr;12(Suppl 1):S216-S229. doi: 10.21037/jgo-2020-01.

ABSTRACT

Pathology is central to the management of peritoneal surface malignancy. This article highlights some recent advances that have had an impact on patient management or could do so in the near future. Malignant peritoneal mesothelioma, particularly the epithelioid subtype, is amenable to radical therapy in selected cases, and factors such as ki67 proliferation index, expression of BAP1 and mutation in CDKN2A show promise as prognostic indicators. Our understanding of multicystic mesothelioma has improved in recent years; it is a true neoplasm for which surgery may be indicated. Serous carcinomas involving the peritoneum are now known to originate from tubal epithelium. They are of two distinct types, high grade and low grade, which are now recognized as different neoplasms with distinctive features, oncogenesis and behavior. Pseudomyxoma peritonei (PMP) is an unusual condition that usually arises from an appendiceal mucinous neoplasm. Recent consensus in the classification and nomenclature of these lesions is discussed, including the distinction between low grade and high grade appendiceal mucinous neoplasms (HAMN), and the diagnostic criteria for appendiceal adenocarcinoma. PMP is divided into four prognostic groups: acellular mucin, low grade mucinous carcinoma peritonei, high grade mucinous carcinoma peritonei, and high grade mucinous carcinoma peritonei with signet ring cells. The pseudomyxoma microbiome is a promising area for clinical intervention but has been the subject of little research activity. Goblet cell adenocarcinoma (previously known as 'goblet cell carcinoid') is a distinctive type of appendiceal adenocarcinoma. Its behavior correlates with histologic features, but no general consensus for classification has been reached.

PMID:33968439 | PMC:PMC8100698 | DOI:10.21037/jgo-2020-01
Omics analyses in peritoneal metastasis-utility in the management of peritoneal metastases from colorectal cancer and pseudomyxoma peritonei: a narrative review

Fetched: May 11th, 2021, 5:01am GMT by Christin Lund-Andersen

J Gastrointest Oncol. 2021 Apr;12(Suppl 1):S191-S203. doi: 10.21037/jgo-20-136.

ABSTRACT

High-throughput "-omics" analysis may provide a broader and deeper understanding of cancer biology to define prognostic and predictive biomarkers and identify novel therapy targets. In this review we provide an overview of studies where the peritoneal tumor component of peritoneal metastases from colorectal cancer (PM-CRC) and pseudomyxoma peritonei (PMP) were analyzed. Most of the available data was derived from DNA mutation analysis, but a brief review of findings from transcriptomic and protein expression analysis was also performed. Studies reporting genomic analysis of peritoneal tumor samples from 1,779 PM-CRC and 623 PMP cases were identified. The most frequently mutated genes in PM-CRC were KRAS, APC, SMAD4, BRAF, and PIK3CA, while in PMP KRAS, GNAS, FAT4, TGFBR1, TP53 and SMAD3/4 mutations were most commonly identified. Analyses were performed by single-gene analyses and to some extent targeted next-generation sequencing, and a very limited amount of broad explorative data exists. The investigated cohorts were typically small and heterogeneous with respect to the methods used and to the reporting of clinical data. This was even more apparent regarding transcriptomic and protein data, as the low number of cases examined and quality of clinical data would not support firm conclusions. Even for the most frequently mutated genes, the results varied greatly; for instance, KRAS mutations were reported at frequencies between 20-57% in PM-CRC and 38-100% in PMP. Such variation could be caused by random effects in small cohorts, heterogeneity in patient selection, or sensitivity of applied technology. Although a large number of samples have been subjected to analysis, cross-study comparisons are difficult to perform, and combined with small cohorts and varying quality and detail of clinical information, the observed variation precludes useful interpretation in a clinical context. Although omics data in theory could answer questions to aid management decisions in PM-CRC and PMP, the existing data does not presently support clinical implementation. With the necessary technologies being generally available, the main challenge will be to obtain sufficiently large, representative cohorts with adequate clinical data and standardized reporting of results. Importantly, studies where the focus is specifically on peritoneal disease are needed, where the study designs are aligned with clearly defined research questions to allow robust conclusions. Such studies are highly warranted if patients with PM-CRC and PMP are to derive benefit from recent advances in precision cancer medicine.

PMID:33968437 | PMC:PMC8100703 | DOI:10.21037/jgo-20-136
Indications and outcomes for repeat cytoreductive surgery and heated intra-peritoneal chemotherapy in peritoneal surface malignancy

Fetched: April 30th, 2021, 5:01am GMT by P A Sutton

Surg Oncol. 2021 Apr 20;38:101572. doi: 10.1016/j.suronc.2021.101572. Online ahead of print.

ABSTRACT

INTRODUCTION: Cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) is offered in specialist centres as a treatment for peritoneal surface tumours. Despite its demonstrated efficacy, intra-abdominal recurrence occurs in 31-57% of patients. The aim of this study is to review the early and long-term outcomes in patients who undergo repeat CRS/HIPEC.

MATERIALS AND METHODS: A retrospective review of a prospectively maintained database of patients who had undergone repeat CRS/HIPEC for appendiceal neoplasms and colorectal peritoneal metastases (CRPM) from 2003 to 2019 was performed at a single specialist centre. Data pertaining to both short term outcomes and survival were evaluated.

RESULTS: Of 1259 patients who had undergone CRS/HIPEC, 84(6.7%) underwent repeat surgery: 45(53.6%) had pseudomyxoma peritonei (PMP) secondary to low grade appendiceal mucinous neoplasms (LAMN), 21(25.0%) had appendix carcinoma and 18(21.4%) had CRPM. Demographics, intra-operative findings and short-term outcomes were comparable across tumour types and between procedures. Median (95% CI) interval between procedures was 22.7(18.9-26.6) months and was comparable between tumour types. Median (95%CI) overall survival was not reached for the cohort overall or for those with PMP, but was 61.0(32.6-89.4) months for those with appendix cancer and 76.9(47.4-106.4) months for CRPM (p=<0.001). Survival was favourable in the PMP group (HR [95%CI] 0.044 [0.008-0.262]; p = 0.000) and unfavourable in the CC2-3 at index CRS procedure group (HR [95%CI] 25.612 [2.703-242.703]; p = 0.005).

CONCLUSION: Our findings demonstrate that repeat cytoredutive surgery with HIPEC can result in favourable survival, especially for patients with PMP when complete cytoreduction is achieved at index operation. We recommend that detailed patient assessment is performed through an expert multidisciplinary team meeting (MDT).

PMID:33915487 | DOI:10.1016/j.suronc.2021.101572
Treatment of advanced pseudomyxoma peritonei using cytoreductive surgery including total gastrectomy and perioperative chemotherapy

Fetched: April 30th, 2021, 5:01am GMT by Paul H Sugarbaker

J Surg Oncol. 2021 Apr 29. doi: 10.1002/jso.26506. Online ahead of print.

ABSTRACT

BACKGROUND: Pseudomyxoma peritonei is a disease caused by the widespread distribution of mucinous tumor into the peritoneal space from a perforated appendiceal neoplasm.

METHODS: All patients in this study had cytoreductive surgery with total gastrectomy plus perioperative intraperitoneal chemotherapy. A high diverting jejunostomy was used on all patients. Patient characteristics, adverse events, and survival were accumulated prospectively.

RESULTS: Fifty-eight patients were available for long-term follow-up. In the univariate analysis, features associated with a less favorable outcome included female gender (p = 0.0127), intestinal obstruction before treatment (p = 0.00791), and prior surgical score (PSS) (p = 0.0054). In the multivariate analysis, the two significant variables were grade (p = 0.0458) and PSS (p = 0.0041). Median survival was 12 years with a 5-, 10-, and 20-year survival of 76%, 58%, and 37%, respectively. There were two postoperative deaths (3.4%) and Grades 3 and 4 adverse events in 20 (34.5%) patients.

CONCLUSIONS: A 10-year survival after cytoreductive surgery, total gastrectomy with temporary high diverting jejunostomy, and perioperative chemotherapy occurred in 58% of these patients with advanced pseudomyxoma peritonei. High-grade disease and extensive prior surgery with a high PSS were associated with reduced benefit.

PMID:33914912 | DOI:10.1002/jso.26506
Incidentally found mucinous epithelial tumors of the appendix with or without pseudomyxoma peritonei: diagnostic and therapeutic algorithms based on current evidence

Fetched: April 28th, 2021, 5:01am GMT by Wim Ceelen

Acta Chir Belg. 2021 May 31:1-10. doi: 10.1080/00015458.2021.1894734. Online ahead of print.

ABSTRACT

Mucinous appendiceal tumors with or without the pseudomyxoma peritonei (PMP) syndrome are rare, but often present as an incidental finding. The confusing histology and lack of large prospective trials result in a considerable diagnostic and therapeutic challenge in these patients. We propose treatment algorithms in patients with incidentally found mucinous epithelial appendiceal tumors, with or without PMP, based on the currently available evidence. The therapeutic approach should take into account the histology and grade of the primary appendix tumor, as well as those of the associated peritoneal disease.

PMID:33904809 | DOI:10.1080/00015458.2021.1894734
Clinicopathologic characteristics of appendiceal mucinous tumor misdiagnosed as gynecological neoplasms

Fetched: April 28th, 2021, 5:01am GMT by C Chen

Zhonghua Zhong Liu Za Zhi. 2021 Apr 23;43(4):472-476. doi: 10.3760/cma.j.cn112152-20200320-00230.

ABSTRACT

Objective: To explore the clinicopathologic features and prognosis of female appendiceal mucinous tumor misdiagnosed as gynecological neoplasm. Methods: The clinical data of 12 cases of suspected gynecological neoplasm but confirmed as appendiceal mucinous tumor by operation and pathology in Beijing Obstetrics and Gynecology Hospital hospital from 2010 to 2019 were collected. The clinicopathologic features, treatment and prognosis of these patients were analyzed. Results: The median age of 12 patients was 58 years old and the clinical manifestations were atypical, including 5 cases with gastrointestinal symptoms, 6 cases with touched lesion in the right adnexal area, 3 cases with pseudomyxoma of peritonei, and 4 cases with increased serological tumor markers and ascites. Before operation, ultrasonic diagnosis showed cystic or cystic-solid mass in the right adnexal region in all cases. Pelvic and abdominal enhanced CT and/or enhanced MRI were performed in 5 cases. Two cases indicated possible origin of appendix and 1 case indicated lymphatic cystic tumor. Laparoscopic or laparotomy exploration was performed in all patients, during the operation 7 cases were performed appendectomy, 4 cases were performed tumor cell reduction and 1 case was treated in another hospital after laparotomy exploration. Eleven cases were diagnosed as low-grade appendiceal mucinous tumor and 1 case as appendiceal mucinous adenocarcinoma by postoperative pathology. Among the 11 patients with low-grade appendiceal mucinous tumor, 9 cases had no recurrence, 1 case lost follow-up, 1 case died of breast cancer and 1 case died 18 months after the operation. Conclusions: Gynecologists should improve their ability to differentiate appendiceal mucinous tumor from gynecological neoplasm. They can include ultrasonic diagnosis and CT scanning into their preoperative physical examination to improve their examination ability, and strive for specialist or surgical treatment to achieve the best result.

PMID:33902210 | DOI:10.3760/cma.j.cn112152-20200320-00230
Grincamycins P-T: Rearranged Angucyclines from the Marine Sediment-Derived Streptomyces sp. CNZ-748 Inhibit Cell Lines of the Rare Cancer Pseudomyxoma Peritonei

Fetched: April 27th, 2021, 5:02am GMT by Zhuo Shang

J Nat Prod. 2021 May 28;84(5):1638-1648. doi: 10.1021/acs.jnatprod.1c00179. Epub 2021 Apr 26.

ABSTRACT

While marine natural products have been investigated for anticancer drug discovery, they are barely screened against rare cancers. Thus, in our effort to discover potential drug leads against the rare cancer pseudomyxoma peritonei (PMP), which currently lacks effective drug treatments, we screened extracts of marine actinomycete bacteria against the PMP cell line ABX023-1. This effort led to the isolation of nine rearranged angucyclines from Streptomyces sp. CNZ-748, including five new analogues, namely, grincamycins P-T (1-5). The chemical structures of these compounds were unambiguously established based on spectroscopic and chemical analyses. Particularly, grincamycin R (3) possesses an S-containing α-l-methylthio-aculose residue, which was discovered in nature for the first time. All of the isolated compounds were evaluated against four PMP cell lines and some exhibited low micromolar inhibitory activities. To identify a candidate biosynthetic gene cluster (BGC) encoding the grincamycins, we sequenced the genome of the producing strain, Streptomyces sp. CNZ-748, and compared the BGCs detected with those linked to the production of angucyclines with different aglycon structures.

PMID:33899471 | DOI:10.1021/acs.jnatprod.1c00179
Epidemiology, histopathology, clinical outcomes and survival of 50 cases of appendiceal mucinous neoplasms: Retrospective cross-sectional single academic tertiary care hospital experience

Fetched: April 6th, 2021, 5:02am GMT by Ammar Aleter

Ann Med Surg (Lond). 2021 Mar 6;64:102199. doi: 10.1016/j.amsu.2021.102199. eCollection 2021 Apr.

ABSTRACT

BACKGROUND: Appendicular neoplasms are rare, most commonly as carcinoids followed by appendicular mucinous neoplasms (AMN). To date, there remains controversy regarding the best treatment of AMN and factors affecting its prognosis.

METHOD: Retrospective chart review of patients operated for appendicular pathology (January 2011-December 2018, follow up to December 2020) at our institution. For all AMN patients, data included pre-operative clinical presentation, and operative/post-operative findings.

RESULTS: 12454 patients underwent appendectomy, of whom 50 (0.4%) had AMN histopathologically (mean age = 47.2). Most patients had laparoscopic appendectomy as primary surgery. Low grade AMN was the most common subtype (n = 41, 82%), and pseudomyxoma peritonei (PMP) was found in 8 (16%) patients. Based on histopathology and margin involvement, the 50 patients were categorized into 3 prognostic categories of recurrence risk (no risk, 24 patients; low risk, 8; high recurrence risk, 18 patients). Disease-free survival (DFS) was lowest for high recurrence risk group (P < 0.001). Eleven (22%) patients had AMN involving resection margin, of whom 3 had no completion surgery and had no recurrence. Higher tumor markers were associated with lower DFS, however it was not statistically significant.

CONCLUSION: AMNs are rare but serious due to the risk of PMP. Laparoscopic approach for AMN may be feasible. Prognostic categories were significantly inversely correlated with recurrence risk; hence useful in predicting prognosis. Contrary to previous proposals, AMNs with acellular mucin at margin or local acellular mucin spillage may not require secondary surgery, especially if the patient is in low recurrence risk group. Tumor markers may predict risk of recurrence.

PMID:33815784 | PMC:PMC8010208 | DOI:10.1016/j.amsu.2021.102199
Mid-Term Audit of a National Peritoneal Surface Malignancy Program Implementation in a Low Middle Income Country: The Moroccan Experience

Fetched: April 4th, 2021, 5:01am GMT by Amine Souadka

Cancers (Basel). 2021 Mar 3;13(5):1088. doi: 10.3390/cancers13051088.

ABSTRACT

Implementing a multimodal management of peritoneal surface malignancies is a steep and complex process, especially as complete cytoreductive surgery (CRS) is the backbone and the major prognostic factor for hyperthermic intraperitoneal chemotherapy (HIPEC) procedures. The implementation of such a program is a challenging process, particularly in low-middle income (LMIC) countries where ressource restrictions may represent a major hurdle to HIPEC appliances acquisition. Herein is the first audit of the implementation of a national peritoneal malignancy program in a north African country. The audit process was performed according to the three implementation steps, namely initiation ("1":2005-2008), transition ("2":2009-2013) and consolidation ("3":2014-2017). We included all consecutive CRS without HIPEC performed with curative intent for ovarian, gastric, colorectal and pseudomyxoma peritonei type of malignancies with an Eastern Cooperative Oncology Group (ECOG) performance Status ≤ 2. Target outcomes for incomplete cytoreduction (ICRS), serious complications ≥ 3b according to the Clavien-Dindo scoring, and early oncologic failure (EOF; disease progression within 2 years of treatment) were compared between the three phases. Independent risk factors correlated to these three outcomes were calculated using a logistic regression model.198 CRS procedures were completed with 49, 60 and 89 cases performed in the three phases, respectively. Overall, patients were comparable except for ECOG and ASA scores which were more severe in the third phase. The comparison of ICRS, serious complications and EOF rates showed a significant reduction between the three phases with (34%, 18% and 4% p = <0.001), (30.6%, 20% and 11.2%, p = 0.019) and (38.8%, 23.3% and 12.4% p = 0.002) respectively. Undergoing CRS in phase 3 on the other hand was a predictive factor of better short term surgical and oncological outcomes and completeness of cytoreduction, while ECOG performance status and spleno-pancreatectomy were also predictive factors of serious complications.

PMID:33802609 | PMC:PMC7962020 | DOI:10.3390/cancers13051088
Parenteral nutrition complications in palliative medicine

Fetched: March 28th, 2021, 5:01am GMT by Zoe Smith

BMJ Support Palliat Care. 2021 Mar 26:bmjspcare-2021-002991. doi: 10.1136/bmjspcare-2021-002991. Online ahead of print.

ABSTRACT

Parenteral nutrition in palliative care is contentious, and decisions on starting or continuing its treatment in palliative patients centre on an individual's preference, balanced with quality of life. This case report describes the unusual onset of pain and agitation secondary to fluid retention, in a patient with metastatic pseudomyxoma peritonei, established on 2.5 L/day of parenteral nutrition. Immediate volume reduction of the parenteral nutrition to 1 L/day successfully reversed the patient's symptoms. To our knowledge, this is the first case of parenteral nutrition inducing pain and agitation in a palliative care patient. There is no specific internationally acclaimed guidance concerning parenteral nutrition content and volume in palliative patients, due to a lack of high quality studies. This case study highlights the need for further research into parenteral nutrition content and volume in palliative care, to prevent harmful effects from fluid retention, impacting on quality of life.

PMID:33771817 | DOI:10.1136/bmjspcare-2021-002991
A case of pseudomyxoma peritonei successfully treated with trifluridine/tipiracil (TAS-102) and bevacizumab after palliative debulking surgery

Fetched: March 24th, 2021, 5:01am GMT by Satoshi Hirano

Chin Clin Oncol. 2021 Mar 18:cco-21-2. doi: 10.21037/cco-21-2. Online ahead of print.

ABSTRACT

Pseudomyxoma peritonei is a rare disease with a reported prevalence of about 1-3 per million people annually. Cytoreductive surgery and perioperative hyperthermic intraperitoneal chemotherapy are considered as treatment options improving disease control or long-term survival. However, for patients with incomplete cytoreduction or debulking surgery, outcomes are significantly poorer compared with patients who have obtained complete or optimal cytoreduction. In cases of high-grade pseudomyxoma peritonei that are considered inoperable and/or unresectable, combination chemotherapy regimen with a neo-angiogenesis inhibitor such as bevacizumab is recommended. In this report, a 62-year-old Japanese man presented with abdominal distention. Examination of ascites demonstrated a jelly-like consistency and peritoneal pseudomyxoma was suspected. To relieve progressive symptoms, palliative debulking surgery with total colectomy was performed. Postoperative pathology confirmed high-grade appendiceal mucinous neoplasm with high-grade pseudomyxoma peritonei. In our case, combination chemotherapy with trifluridine/tipiracil (TAS-102) and bevacizumab was initiated after palliative debulking surgery. As a result, carcinoembryonic antigen level was kept stable and the volume of ascites remained almost the same as at the beginning of treatment for more than 2 years. In conclusion, combination chemotherapy comprising TAS-102 and bevacizumab in patients with palliative debulking could be a useful option for patients with high-grade mucinous appendiceal neoplasm and high-grade pseudomyxoma peritonei.

PMID:33752412 | DOI:10.21037/cco-21-2
Molecular profiling of appendiceal serrated lesions, polyps and mucinous neoplasms: a single-centre experience

Fetched: March 15th, 2021, 5:02am GMT by Giada Munari

J Cancer Res Clin Oncol. 2021 Jul;147(7):1897-1904. doi: 10.1007/s00432-021-03589-4. Epub 2021 Mar 12.

ABSTRACT

PURPOSE: Non-neuroendocrine neoplasms of the appendix are a phenotypically heterogeneous group of lesions; a comprehensive molecular characterization of these tumors is still lacking.

METHODS: A total of 52 samples taken from 49 patients was evaluated: 18 sessile serrated lesions (SSL; 3 with dysplasia), 2 high-grade tubular adenomas, 1 tubulo-villous adenoma,1 hyperplastic polyp, 18 low-grade appendiceal mucinous neoplasms (LAMN), 3 high-grade appendiceal mucinous neoplasms (HAMN) and 9 mucinous adenocarcinomas. Hotspot mutational profiling of the RNF43, SMAD4, KRAS, NRAS, BRAF and PIK3CA genes was performed. Expression of p53, MLH1, PMS2, MSH2, and MSH6 was evaluated by immunohistochemistry.

RESULTS: KRAS was the most frequently mutated gene (53.9% of cases), followed by RNF43 (15.4%), and BRAF (13.5%). In particular: KRAS was mutated in 44.4% of adenocarcinomas, 66.7% of HAMNs, 61.1% of LAMNs, 53.3% of SSL without dysplasia and in 66.7% of SSL with dysplasia; RNF43 was mutated in 33.3% of adenocarcinomas, 66.7% of HAMNs, 11.1% of LAMNs and in 6.7% of SSL without dysplasia; BRAF was mutated in 11.1% of adenocarcinomas, 26.7% of SSL without dysplasia and in 5.6% of LAMNs. Only a case of high-grade tubular adenoma showed mismatch repair deficiency, while immunohistochemical expression of p53 was altered in 21.1% of cases.

CONCLUSIONS: The histological phenotypic similarities between appendicular mucinous lesions and serrated colon lesions do not reflect a similar genetic landscape. Mismatch repair deficiency is a rare event during appendiceal mucinous carcinogenesis.

PMID:33712927 | PMC:PMC8164605 | DOI:10.1007/s00432-021-03589-4
Primary mucinous ovarian tumors vs. ovarian metastases from gastrointestinal tract, pancreas and biliary tree: a review of current problematics

Fetched: March 13th, 2021, 5:01am GMT by Pavel Dundr

Diagn Pathol. 2021 Mar 11;16(1):20. doi: 10.1186/s13000-021-01079-2.

ABSTRACT

BACKGROUND: Making the distinction between primary mucinous and metastatic ovarian tumors is often difficult, especially in tumors with a primary source from the gastrointestinal tract, pancreas and biliary tree. The aim of the following paper is to provide an overview of the problematics, with a focus on the possibilities of the differential diagnosis at the macroscopic, microscopic and immunohistochemical level.

MAIN BODY: The three main aspects of mucinous ovarian tumors are described in detail, including the comparison of the available diagnostic algorithms based on the evaluation of mostly macroscopic features, characterization of the spectrum of microscopic features, and a detailed analysis of the immunophenotype comparing 20 antibodies with the assessment of their statistical significance for differential diagnosis purposes. Specific features, including Krukenberg tumor and pseudomyxoma peritonei, are also discussed.

CONCLUSION: Despite the growing knowledge of the macroscopic and microscopic features of ovarian mucinous tumors and the availability of a wide range of immunohistochemical antibodies useful in this setting, there still remains a group of tumors which cannot be precisely classified without close clinical-pathological cooperation.

PMID:33706757 | PMC:PMC7953678 | DOI:10.1186/s13000-021-01079-2
A general overview of mucocele of appendix

Fetched: March 9th, 2021, 5:01am GMT by Mahendra Pratap Singh

J Family Med Prim Care. 2020 Dec 31;9(12):5867-5871. doi: 10.4103/jfmpc.jfmpc_1547_20. eCollection 2020 Dec.

ABSTRACT

Mucocele of the appendix is a very rare disease entity that often discovered incidentally during surgery. It can result from both non-neoplastic and neoplastic lesions and histopathological examination is needed for confirmation. Failure to make an early preoperative diagnosis may results in its rapture and spillage of mucin contents into the peritoneal cavity leading to a disastrous complication of pseudomyxoma peritonei (PMP) that has a very bad prognosis. A clear pathological terminology and management strategies of appendiceal mucocele (AM) is lacking. This literature review aims to derive detailed information related to clinical significance of AM to avoid complication of PMP and plan appropriately during surgery according to the current evidence. The relevant articles from scientific databases such as Medline, PubMed, Google Scholar were searched and extracted using the keywords "mucocele appendix" "cystadenoma%". Data based on epidemiology, clinical manifestations, complications, pathology, diagnostic work up and management were analyzed and summarized. A meticulous surgical excision is the mainstay of treatment and open surgical approach is still preferred over laparoscopy. Preoperative diagnosis of AM is very imperative as it may harbour neoplasm and can be made utilising the imaging tools like computed tomography and ultrasonography. Primary care physicians can have a crucial role in making early detection and timely referral for appropriate management in order to avoid complications. After appendectomy, 5-year survival rate for the simple AM is 91%-100% but it reduces to 25% for the malignant AM.

PMID:33681010 | PMC:PMC7928084 | DOI:10.4103/jfmpc.jfmpc_1547_20
High-grade appendiceal mucinous neoplasm presenting as a giant appendiceal mucocele

Fetched: March 9th, 2021, 5:01am GMT by Alan Lu

Radiol Case Rep. 2021 Feb 24;16(5):1051-1056. doi: 10.1016/j.radcr.2021.02.014. eCollection 2021 May.

ABSTRACT

Appendiceal mucinous neoplasms are rare findings defined by an accumulation of mucus within the vermiform appendix, and can be caused by a variety of conditions. Appendiceal mucinous neoplasms are important to consider because they can develop into pseudomyxoma peritonei as a consequence of perforation. We report a case of a 55-year-old man who initially presented with increasing abdominal girth, constipation, anorexia, and unintentional weight loss. Computed tomography examination of the abdomen and pelvis demonstrated a huge thin-walled cystic mass causing significant displacement of the surrounding abdominal and pelvic structures. The mass was amenable to resection and removed without perforation. Gross pathologic examination demonstrated a 44.0 × 40.0 × 23.0 cm unilocular cystic mass with a section of attached bowel. Microscopic examination revealed high-grade appendiceal mucinous neoplasm arising in a background of low-grade appendiceal mucinous neoplasm. This case report provides an evidence to include appendiceal mucinous neoplasms in the differential diagnosis of large abdominal cystic masses.

PMID:33680275 | PMC:PMC7917446 | DOI:10.1016/j.radcr.2021.02.014
Huge and Unique Pseudomyxoma Peritonei

Fetched: March 9th, 2021, 5:01am GMT by Brahmana Askandar Tjokroprawiro

J Obstet Gynaecol India. 2021 Mar 3:1-2. doi: 10.1007/s13224-021-01460-7. Online ahead of print.

NO ABSTRACT

PMID:33679016 | PMC:PMC7925255 | DOI:10.1007/s13224-021-01460-7
ASO Visual Abstract: From the Ronnett to the PSOGI Classification System for Pseudomyxoma Peritonei: A Validation Study

Fetched: March 3rd, 2021, 5:03am GMT by Blanca Rufián-Andujar

Ann Surg Oncol. 2021 May;28(5):2829-2830. doi: 10.1245/s10434-021-09708-2.

NO ABSTRACT

PMID:33650062 | DOI:10.1245/s10434-021-09708-2
Correction to: Intraperitoneal mitomycin C improves survival compared to cytoreductive surgery alone in an experimental model of highgrade pseudomyxoma peritonei

Fetched: March 3rd, 2021, 5:03am GMT by Olaf Sørensen

Clin Exp Metastasis. 2021 Apr;38(2):253. doi: 10.1007/s10585-021-10084-0.

NO ABSTRACT

PMID:33649933 | DOI:10.1007/s10585-021-10084-0
From Diagnosis to Management; Mucocele of Stump Appendicitis, Extremely Rare Finding in an Uncommon Surgical Disease: Literature Review

Fetched: February 26th, 2021, 5:02am GMT by Syed Muhammad Ali

Int J Surg Oncol. 2021 Feb 11;2021:8816643. doi: 10.1155/2021/8816643. eCollection 2021.

ABSTRACT

Mucocele of the appendix is the accumulation of mucoid material in the appendiceal lumen. Although the terminology is imprecise, as it does not differentiate between the benign and malignant nature of the condition, preoperative recognition is imperative as spillage of the mucus during surgical handling can result in grave complications like pseudomyxoma peritonei. Mucocele developing in a stump of the appendix, i.e., a remnant of appendiceal tissue after surgical removal of an inflamed organ, is an extremely uncommon phenomenon, as not many cases are reported in the literature. In this review, all cases reported in English literature are discussed.

PMID:33628502 | PMC:PMC7892232 | DOI:10.1155/2021/8816643
Evaluation of the significance of pseudomyxoma peritonei patients based on the Peritoneal Surface Oncology Group International (PSOGI) classification

Fetched: February 25th, 2021, 5:02am GMT by Jong Lyul Lee

Asian J Surg. 2021 Jun;44(6):848-853. doi: 10.1016/j.asjsur.2021.01.012. Epub 2021 Feb 20.

ABSTRACT

BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare disease characterized by mucinous ascites and deposits on the peritoneal surfaces. The study aimed to assess PMP patients according to the Peritoneal Surface Oncology Group International (PSOGI) classification, as a part of standardization of this rare disease.

METHODS: This retrospective study analyzed PMP patients who underwent surgery between January 2007 and December 2017. All histologic slides were re-evaluated and the clinical data were collected. According to the PSOGI, PMP was retrospectively classified into three categories: low-grade (LG-PMP), high-grade (HG-PMP), and signet-ring cells (SRC-PMP). The extent of peritoneal involvement was quantified by the peritoneal cancer index (PCI). The completeness of cytoreduction (CCR) was dichotomized as complete or incomplete.

RESULTS: Fifty-seven patients were included in this study, consisted of 39 patients with LG-PMP (74.0%), 14 with HG-PMP (20.8%), and 4 with SRC-PMP (5.2%). There was no operative mortality and major complications occurred in 24 patients (31.2%). The 5-year overall survival was 56.2% ± 8.1% for LG-PMP, 37.5% ± 12.1% for HG-PMP, and 25.0% ± 21.7% for SRC-PMP. Concerning CCR, the 5-year overall (complete: 59.5% ± 8.4% vs. incomplete: 12.7% ± 8.1%, p = 0.001) and disease-free survival (complete: 38.6% ± 8.9% vs. incomplete: 7.7% ± 6.8%, p = 0.001) were significantly different. In a multivariable analysis, PSOGI classification and CCR independently correlated with survival (p = 0.011 and 0.018, respectively).

CONCLUSIONS: The PSOGI classification provides prognostic stratification, hopefully requiring further validation including every single case of PMP established as a standard criteria.

PMID:33622598 | DOI:10.1016/j.asjsur.2021.01.012
Two-step cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei with high peritoneal carcinomatosis index

Fetched: February 25th, 2021, 5:02am GMT by Bertrand Trilling

World J Surg Oncol. 2021 Feb 23;19(1):60. doi: 10.1186/s12957-021-02171-z.

ABSTRACT

BACKGROUND: Complete cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the only curative treatment for pseudomyxoma peritonei (PMP) arising from the appendix. High peritoneal carcinomatosis index (PCI) is associated with an increased risk of surgical complications. The objective of this study was to present the results of a planned two-step surgical strategy to decrease postoperative morbidity and improve resectability of patients with very high PCI.

METHODS: All consecutive patients who underwent a planned two-step surgical approach for PMP between January 2012 and March 2020 were retrospectively included. This approach was offered for patients with low-grade PMP with PCI > 28 for which feasibility of a complete CRS in one operation was uncertain. The first surgery included a complete CRS of the inframesocolic compartment and omentectomy. HIPEC was delivered at the second surgery, after complete CRS of the supramesocolic compartment. Postoperative morbidity was assessed using the Clavien-Dindo classification and survival results were also collected.

RESULTS: Eight patients underwent the two-step approach. The median PCI was 33 (29-39) and the median time between the two procedures was 111 days (90-212 days). One patient was deemed unresectable at the second surgery. The rate of major morbidity was 0% for the first step and 25% for the second step, with no mortality. Median follow-up was 53.8 months (3-73 months).

CONCLUSION: A two-step surgical management for low-grade PMP patients with very high PCI is safe and feasible, with acceptable postoperative morbidity and no compromise on oncological outcomes.

PMID:33622339 | PMC:PMC7903627 | DOI:10.1186/s12957-021-02171-z
Jelly-like Pleural Effusions: An Uncommon Manifestation of Pseudomyxoma Peritonei

Fetched: February 24th, 2021, 5:02am GMT by Yimin Ma

Arch Bronconeumol (Engl Ed). 2021 Jan 27:S0300-2896(21)00019-3. doi: 10.1016/j.arbres.2021.01.002. Online ahead of print.

NO ABSTRACT

PMID:33618915 | DOI:10.1016/j.arbres.2021.01.002
Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines

Fetched: February 24th, 2021, 5:02am GMT by Yu-Lin Lin

Orphanet J Rare Dis. 2021 Feb 13;16(1):85. doi: 10.1186/s13023-021-01723-6.

ABSTRACT

BACKGROUND: Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.

MAIN BODY: As a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS) + hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS + HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS + HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS + HIPEC on PMP.

CONCLUSION: Currently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.

PMID:33581733 | PMC:PMC7881689 | DOI:10.1186/s13023-021-01723-6
The Prognostic Impact of Pathology on Patients With Pseudomyxoma Peritonei Undergoing Debulking Surgery: A Systematic Review and Meta-Analysis of Retrospective Studies

Fetched: February 24th, 2021, 5:02am GMT by Shengnan Zhou

Front Surg. 2020 Nov 16;7:554910. doi: 10.3389/fsurg.2020.554910. eCollection 2020.

ABSTRACT

Background: Pseudomyxoma peritonei (PMP) is a rare clinical condition with fatal outcomes, which is characterized by the progressive accumulation of mucinous ascites and peritoneal implants. Some studies have reported the effect of PMP biology on patient outcome. The objective of this study was to analyze published articles focusing on the impact of pathology on the prognosis of PMP patients undergoing debulking. Methods: Data from all studies regarding the prognosis of patients, with different pathologies, who underwent debulking surgery were analyzed. We searched PubMed, the Wiley Online Library, Ovid, and the Cochrane Library (through January 2020). Studies were confined to those articles written in English. Five studies were identified, and the differences in 5-year survival rates were analyzed according to the Kaplan-Meier survival curves. The hazard ratios (HRs) of the 5-year survival rates were calculated. Results: The mean and median 5-year survival rates of all patients were 39 and 40%, respectively. The median overall survival was 49.3 months. The mean 5-year survival rates of low-grade PMP was 45.2%. The five studies had sufficient data to calculate HRs from the 5-year survival rates data, and three had HRs lower than 1. The total HRs was 0.54, with a 95% CI between 0.33 and 0.89 (P = 0.01). Conclusions: Among PMP patients receiving debulking surgery who are not able to undergo complete cytoreductive surgery, low-grade biological PMP had a better prognosis than high-grade PMP.

PMID:33304920 | PMC:PMC7701122 | DOI:10.3389/fsurg.2020.554910
Laparoscopic cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for limited peritoneal metastasis. The PSOGI international collaborative registry

Fetched: February 24th, 2021, 5:02am GMT by A Arjona-Sanchez

Eur J Surg Oncol. 2021 Jun;47(6):1420-1426. doi: 10.1016/j.ejso.2020.11.140. Epub 2020 Dec 2.

ABSTRACT

INTRODUCTION: A laparoscopic approach for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (L-CRS+HIPEC) in highly selected patients has been reported in small cohorts with a demonstrable reduction in length of stay and post-operative morbidity. This study aims to analyse individual patient data from these international centres collected through the Peritoneal Surface Oncology Group International (PSOGI) L-CRS+HIPEC registry.

METHODS: An international registry was designed through a networking database (REDCAP®). All centres performing L-CRS+HIPEC were invited through PSOGI to submit data on their cases. Patient's characteristics, postoperative outcomes and survival were analysed.

RESULTS: Ten international centres contributed a total of 143 L-CRS+HIPEC patients during the study period. The most frequent indication was low grade pseudomyxoma peritonei in 79/143 (55%). Other indications were benign multicyst mesothelioma in 21/143(14%) and peritoneal metastasis from colon carcinoma in 18/143 (12,5%) and ovarian carcinoma in 13/143 (9%). The median PCI was 3 (2-5). The median length of stay was 6 (5-10) days, with 30-day major morbidity rate of 8.3% and 30-day mortality rate of 0.7%. At a median follow-up of 37 (16-64) months 126/143 patients (88.2%) were free of disease.

CONCLUSIONS: Analysis of these data demonstrates that L-CRS+HIPEC is a safe and feasible procedure in highly selected patients with limited peritoneal disease when performed at experienced centres. While short to midterm outcomes are encouraging in patients with less invasive histology, longer follow up is required before recommending it for patients with more aggressive cancers with peritoneal dissemination.

PMID:33298341 | DOI:10.1016/j.ejso.2020.11.140
Pseudomyxoma pleurii: unusual location of a rare disease

Fetched: February 24th, 2021, 5:02am GMT by Kaoutar Imrani

Radiol Case Rep. 2020 Nov 20;16(1):215-218. doi: 10.1016/j.radcr.2020.11.016. eCollection 2021 Jan.

ABSTRACT

Pseudomyxoma peritonei with pleural extension is very rare. Dissemination in the pleural cavity can be trans diaphragmatic or hematogenous. Appendicular and ovarian mucinous tumors are the most often involved. Imaging plays an important role in the diagnosis which is confirmed histologically. We report the case of a pseudomyxoma peritonei and pleurii in a 55-year-old female patient who underwent surgery for mucinous adenocarcinoma of the ovary. This diagnosis must be suggested when there is subpleural nodular thickening associated with pseudomyxoma peritonei.

PMID:33294092 | PMC:PMC7691680 | DOI:10.1016/j.radcr.2020.11.016
Predictors of Progression of Appendiceal Mucinous Neoplasm to Pseudomyxoma Peritonei

Fetched: February 24th, 2021, 5:02am GMT by Stefanie Sueda

Am Surg. 2020 Oct;86(10):1379-1384. doi: 10.1177/0003134820964464.

ABSTRACT

Appendiceal mucinous neoplasm (AMN) can present with a spectrum of disease. Predicting factors in development of pseudomyxoma peritonei (PMP) from AMN could aid in management and treatment. The aim of this study was to determine factors predictive of PMP from AMN. This was a retrospective multicenter study of all patients diagnosed with AMN from 2006-2017. Diagnosis of PMP was compared by (1) patient demographics, (2) tumor characteristics, and (3) surgery. Secondary end points were disease-specific survival (DSS) and overall survival (OS).One-hundred thirty-eight patients with AMN were identified. Thirty-six patients (26.1%) had a ruptured appendix at presentation, and 12 patients (8.7%) were diagnosed with PMP during the study period. Eight patients presented with PMP at the time of surgery. No demographic factors were predictive of PMP. Operative approach and extent of initial resection did not correlate with PMP. Tumor rupture at presentation was the only factor associated with PMP, though only 14% of patients who presented with simple rupture eventually progressed to PMP.OS was not different between those who were diagnosed with PMP and those who were not. DSS was significantly lower for the group diagnosed with PMP (P = .007). Tumor rupture at presentation did not influence OS or DSS. The only factor found to be significantly associated with PMP was tumor rupture at presentation. Diagnosis of PMP did not affect OS but did lead to decreased DSS.In conclusion, though a majority of patients who presented with rupture did not go on to develop PMP, tumor rupture at presentation was the only factor significantly associated with PMP. Diagnosis of PMP did not affect OS at 5 years. In patients with AMN who present with a ruptured appendix on final pathology, we recommended continued surveillance, though overall risk of PMP is relatively low.

PMID:33284666 | DOI:10.1177/0003134820964464
Comparative study of mucinous and non-mucinous appendiceal neoplasms with peritoneal dissemination treated by cyoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC)

Fetched: February 24th, 2021, 5:02am GMT by Niharika Rajan Garach

Eur J Surg Oncol. 2021 May;47(5):1132-1139. doi: 10.1016/j.ejso.2020.08.017. Epub 2020 Aug 25.

ABSTRACT

BACKGROUND: Appendiceal non-mucinous neoplasms (AnMN) are rare and poorly understood malignancies with no standard treatment. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is largely used to treat peritoneal disseminations from appendiceal mucinous neoplasms (AMN), but its role with AnMN is unclear.

METHODS: A prospective database of 315 patients with advanced appendiceal primaries undergoing CRS/HIPEC during 1996-2020 was reviewed. Baseline characteristics, operative and long-term outcomes of AnMN were compared with those of AMN. AMN were categorized according to PSOGI classification into high-grade, low-grade, and acellular mucin (AC), based on peritoneal disease histology.

RESULTS: Twenty-three patients (7.3%) with goblet cell carcinoma (GCC; n = 9), intestinal-type adenocarcinoma (ITAC; n = 12), and mixed adeno-neuroendocrine carcinoma (MANEC; n = 2) were identified. AnMN patients were more likely to be males (P = 0.006), have preoperative systemic chemotherapy (P = 0.001), grossly incomplete CRS (P = 0.001), and nodal metastases (P = 0.001), but not systemic relapse after CRS/HIPEC (P = 0.133). Median follow-up was 25.1 months (range 0.8-77.3) for AnMN, and 80.9 months (range 0.1-279.2) for AMN. Median overall survival was 24.0 months for AnMN, 66.2 months for high-grade AMN (P = 0.015), 160.0 months for low-grade ANM (P = 0.001), and not reached for AC (P = 0.001). Among AnMN patients, median survival was 23.4 months for GCC, 38.7 months for ITAC, 20.3 months for MANEC (P = 0.855). In the overall series, histological subtype (P = 0.001), incomplete cytoreduction (P = 0.001), and positive lymph-nodes (P = 0.003) correlated with poorer survival at multivariate analysis.

CONCLUSIONS: AnMN share with AMN a predominant local-regional dissemination pattern, but prognosis after CRS/HIPEC is worse. This strategy needs to be carefully considered for AnMN.

PMID:33280949 | DOI:10.1016/j.ejso.2020.08.017
Role of Epithelial-Mesenchymal Plasticity in Pseudomyxoma Peritonei: Implications for Locoregional Treatments

Fetched: February 24th, 2021, 5:02am GMT by Maria Luisa Calabrò

Int J Mol Sci. 2020 Nov 30;21(23):9120. doi: 10.3390/ijms21239120.

ABSTRACT

The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood. Epithelial-mesenchymal transition (EMT) plays a role in cancer development and progression by converting static epithelial cells into the migratory and microenvironment-interacting mesenchymal cells, and by the modulation of chemoresistance and stemness of tumor cells. Several findings highlight that pathways involved in EMT and its reverse process (mesenchymal-epithelial transition, MET), now collectively called epithelial-mesenchymal plasticity (EMP), play a role in peritoneal metastases. So far, the relevance of factors linked to EMP in a unique peritoneal malignancy such as pseudomyxoma peritonei (PMP) has not been fully elucidated. In this review, we focus on the role of epithelial-mesenchymal dynamics in the metastatic process involving mucinous neoplastic dissemination in the peritoneum. In particular, we discuss the role of expression profiles and phenotypic transitions found in PMP in light of the recent concept of EMP. A better understanding of EMP-associated mechanisms driving peritoneal metastasis will help to provide a more targeted approach for PMP patients selected for locoregional interventions involving cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

PMID:33266161 | PMC:PMC7731245 | DOI:10.3390/ijms21239120
Pulmonary metastases in pseudomyxoma peritonei - case report and review of the literature

Fetched: February 24th, 2021, 5:02am GMT by P Horák

Rozhl Chir. 2020 Fall;99(10):462-466.

ABSTRACT

Pseudomyxoma peritonei is a rare disease caused by dissemination of cancer forming mucous and gelatinous masses in the peritoneal cavity. Local recurrence is frequent; however, dissemination outside the peritoneal cavity is rare. Case report: We present the case report of a female patient treated for bilateral pulmonary metastases from pseudomyxoma peritonei. We removed 1 metastasis from her left lung and 12 metastases form her right lung. The patient remained in a good clinical state; however, additional lung metastases developed, as well as a metastasis in L2 vertebra for which the patient underwent a surgical procedure. Conclusion: Cytoreductive surgeries for intrathoracic involvement in pseudomyxoma peritonei with additional hyperthermic cytostatic intrapleural lavage in case of pleural involvement can lead to a longer lifespan in indicated patients.

PMID:33242964
Outcomes of home parenteral nutrition in 34 patients with intestinal failure from recurrent or progressive peritoneal malignancy of gastro-intestinal tract origin

Fetched: February 24th, 2021, 5:02am GMT by David Swain

Eur J Clin Nutr. 2020 Nov 21. doi: 10.1038/s41430-020-00810-4. Online ahead of print.

ABSTRACT

OBJECTIVES: To investigate the outcomes of 34 patients with intestinal failure secondary to advanced peritoneal malignancy on home parenteral nutrition (HPN).

METHODS: A retrospective analysis of all known patients receiving HPN at any time between January 2012 and the 31st March 2020 registered in a high volume peritoneal malignancy surgical centre database.

RESULTS: The median duration of HPN for all patients was 309.5 days (range 31-2198). Overall 11/34 went on to have multivisceral transplants. Of these 5/11 resumed normal oral intake off HPN, 3 died and 3 required ongoing HPN. Average time on HPN for patients with pseudomyxoma peritonei of appendix origin was 338 days (71-2198) compared with 90 days (31-260) in the group with more aggressive tumours.

CONCLUSIONS: HPN is feasible and effective in selected patients with pseudomyxoma peritonei as either a bridge to transplant or definitive treatment. As expected, patients with more aggressive tumours fare worse.

PMID:33221816 | DOI:10.1038/s41430-020-00810-4
Mode of Presentation in 1070 Patients With Perforated Epithelial Appendiceal Tumors, Predominantly with Pseudomyxoma Peritonei

Fetched: February 24th, 2021, 5:02am GMT by Umar S Shariff

Dis Colon Rectum. 2020 Sep;63(9):1257-1264. doi: 10.1097/DCR.0000000000001682.

ABSTRACT

BACKGROUND: Perforated epithelial appendiceal tumors are uncommon and can give rise to pseudomyxoma peritonei. Pseudomyxoma peritonei is rare, almost always of appendiceal origin, and presents in various ways relevant to abdominal surgeons.

OBJECTIVE: The aim of this study was to report the mode of presentation of pseudomyxoma peritonei of appendiceal origin in patients treated in a high-volume UK peritoneal malignancy center.

DESIGN: A retrospective observational study was conducted.

SETTINGS: This was a single-center study.

PATIENTS: Retrospective analysis of a prospective database of consecutive patients undergoing surgery between March 1994 and December 2016 was performed.

MAIN OUTCOME MEASURES: Mode of presentation was classified into 7 categories: "histological diagnosis at abdominal surgery for presumed appendicitis," "probable pseudomyxoma peritonei" based on abnormality on cross-sectional imaging (± image-guided biopsy)," "abnormal imaging (other cause suspected) and operative finding of pseudomyxoma peritonei," "diagnostic laparoscopy," "ovarian mass," "new-onset hernia," and "miscellaneous."

RESULTS: Overall, 1070 patients underwent surgery (female 61%, male 39%); median age was 57. The mode of presentation was abnormality on cross-sectional imaging in 324 of 1070 patients (30.3%), histological diagnosis at emergency surgery for presumed appendicitis in 203 of 1070 patients (19%), and abnormal imaging with eventual pseudomyxoma peritonei diagnosis in 180 of 1070 patients (16.8%); 124 of 651 women (19.2%) presented with an ovarian mass. New-onset hernia was the presenting feature in 9.9%; 83 of 1070 patients (7.7%) were diagnosed at diagnostic laparoscopy, and 32 of 1070 patients (3%) were described as "miscellaneous." Overall, 775 of 1070 patients (72.4%) had complete cytoreductive surgery with 10-year survival of 63.5%.

LIMITATIONS: Results are limited by the retrospective nature of the study, radiological improvements over the study period, and the number of patients who had overlapping features at presentation.

CONCLUSION: Perforated appendiceal tumors present in various ways but predominantly at cross-sectional imaging or coincidentally at laparoscopy or laparotomy. All abdominal surgeons will occasionally encounter cases, often unexpectedly, and recognition and referral to a specialized unit results in excellent outcomes in most cases. See Video Abstract at [links.lww.com] MODO DE PRESENTACIÓN EN 1070 PACIENTES CON TUMORES EPITELIALES APENDICULARES PERFORADOS, PREDOMINANTEMENTE CON PSEUDOMIXOMA PERITONEAL: Los tumores epiteliales apendiculares perforados son poco frecuentes y pueden dar lugar a Pseudomyxoma peritonei. El pseudomixoma peritoneal es raro, casi siempre de origen apendicular, y se presenta de diversas formas relevantes para los cirujanos abdominales.El objetivo fue informar el modo de presentación del Pseudomixoma peritoneal de origen apendicular en pacientes tratados en un centro de malignidad peritoneal de alto volumen en el Reino Unido.Se realizó un estudio observacional retrospectivo.Este fue un estudio de centro único.Análisis retrospectivo de una base de datos prospectiva de pacientes consecutivos sometidos a cirugía entre marzo de 1994 y diciembre de 2016.El modo de presentación se clasificó en 7 categorías: "diagnóstico histológico en la cirugía abdominal por presunta apendicitis", "probable Pseudomixoma peritoneal" basado en la anormalidad en la imagen de corte transversal (biopsia guiada por imagen +/-), "imagen anormal (se sospecha otra causa) y hallazgo quirúrgico de Pseudomixoma peritoneal", "laparoscopia diagnostica", "masa ovarica", "hernia de reciente aparicion" y "varios".En total, 1070 pacientes fueron operados (mujeres 61%, hombres 39%); edad media 57. El modo de presentación fue anormalidad en la imágen transversal en 324/1070 (30.3%), el diagnóstico histológico en cirugía de emergencia por presunta apendicitis en 203/1070 (19%), la imágen anormal con eventual diagnóstico de Pseudomixoma peritoneal en 180 / 1070 (16.8%), 124/651 (19.2%) las mujeres presentaron una masa ovárica. La hernia de reciente aparición fue la característica de presentación en 9.9%, 83/1070 (7.7%) fueron diagnosticados por laparoscopia diagnóstica y 32/1070 (3%) "misceláneos". En general, 775/1070 (72,4%) se sometió a cirugía citorreductora completa con una supervivencia a 10 años del 63,5%.Una deficiencia es que los resultados están limitados por la naturaleza retrospectiva del estudio, las mejoras radiológicas a lo largo del período de estudio, y varios pacientes tenían características superpuestas en la presentación.Los tumores apendiculares perforados se presentan de varias maneras, pero predominantemente en imágenes transversales o casualmente en laparoscopia o laparotomía. Todos los cirujanos abdominales encontrarán ocasionalmente con casos, a menudo inesperados, y el reconocimiento y la derivación a una unidad especializada da lugar a excelentes resultados en la mayoría de los casos. Consulte Video Resumen en [links.lww.com]

PMID:33216496 | DOI:10.1097/DCR.0000000000001682
Rendezvous procedure to establish ureteral continuity from a delayed ureteral leak following pelvic surgery. A case report

Fetched: February 24th, 2021, 5:02am GMT by George Chang

Int J Surg Case Rep. 2020;76:505-509. doi: 10.1016/j.ijscr.2020.10.014. Epub 2020 Oct 10.

ABSTRACT

BACKGROUND: Iatrogenic damage to the ureter as a result of an abdominal or pelvic surgical procedure is unusual. However, it does occur and the surgeon must be prepared to deal knowledgeably with the injury. Leaks that are recognized within the operating theater are managed, for the most part, at the same surgical procedure oftentimes with urologic consultation. A delayed leak presents unique problems in that direct access to the site of the leak is not possible except by a reoperative procedure. Delayed leaks present a clinical situation involving the urologist, interventional radiologist, as well as the surgeon.

METHODS: A patient who developed delayed urine leakage following a partial sacrectomy to remove recurrent mucinous appendiceal malignancy was studied. The leakage was controlled using a nephroureteral stent. Placement of the nephroureteral stent was made possible by the rendezvous procedure.

RESULTS: The sequence of procedures to reestablish ureteral continuity following a delayed leak are important in the successful placement of a nephroureteral stent. After establishing the site of the injury a percutaneous nephrostomy must be placed. Then, through the nephrostomy, a guidewire is placed in the ureter to be recovered and advanced into the bladder using a ureteroscope and grasping forceps. A nephroureteral stent is placed over the guidewire to bridge the gap and stent the ureteral defect.

CONCLUSIONS: The rendezvous procedure can be successful a large percentage of the time with a delayed ureteral leakage. Successful recovery of a guidewire in the ureter by ureteroscopy requires a combined interventional radiology and urologic procedure.

PMID:33207420 | PMC:PMC7596013 | DOI:10.1016/j.ijscr.2020.10.014
The role of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis: a systematic review including evidence from Japan

Fetched: February 24th, 2021, 5:02am GMT by Toshiyuki Kitai

Surg Today. 2020 Nov 13. doi: 10.1007/s00595-020-02180-7. Online ahead of print.

ABSTRACT

The prognosis of peritoneal carcinomatosis is poor. However, the emergence of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS + HIPEC) as a treatment option has prolonged survival and it can even potentially cure patients with peritoneal carcinomatosis. Randomized controlled studies and other observational studies indicated that this combined therapy potentially improved the prognosis of patients with colon, gastric, and ovarian cancers with acceptable morbidity and mortality rates. Even in rarer diseases, such as pseudomyxoma peritonei and malignant peritoneal mesothelioma, CRS + HIPEC markedly improved the prognoses over those with conventional treatment. Based on the accumulated evidence, clinical guidelines recommend CRS + HIPEC for selected patients with peritoneal carcinomatosis. However, several issues still need to be overcome. A standard method for HIPEC has not yet been established. Furthermore, the criteria employed for patient selection need to be clarified to achieve real benefits. The peritoneal cancer index, chemo-sensitivity and several biological markers are considered to be key factors.

PMID:33185798 | DOI:10.1007/s00595-020-02180-7
Cytoreductive Surgery Combined with Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Metastasis from Colorectal Cancer

Fetched: February 24th, 2021, 5:02am GMT by Hideaki Yano

Clin Colon Rectal Surg. 2020 Nov;33(6):372-376. doi: 10.1055/s-0040-1714242. Epub 2020 Sep 22.

ABSTRACT

Peritoneal metastasis from colorectal cancer (PM-CRC) is used to be considered a systemic and fatal condition; however, it has been growingly accepted that PM-CRC can still be local disease rather than systemic disease as analogous to liver or lung metastasis. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is now considered an optimal treatment for PM-CRC with accumulating evidence. There is a good reason that CRS + HIPEC, widely accepted as a standard of care for pseudomyxoma peritonei (PMP), could be a viable option for PM-CRC given a similarity between PM-CRC and PMP. Recent years have also seen that modern systemic chemotherapy with or without molecular targeted agents can be effective for PM-CRC. It is possible that neoadjuvant or adjuvant chemotherapy combined with CRS + HIPEC could further improve outcomes. Patient selection, utilizing modern images and increasingly laparoscopy, is crucial. Particularly, diagnostic laparoscopy is likely to play a significant role in predicting the likelihood of achieving complete cytoreduction and assessing the peritoneal cancer index score.

PMID:33162842 | PMC:PMC7605907 | DOI:10.1055/s-0040-1714242
Long-term outcomes for patients with peritoneal acellular mucinosis secondary to low grade appendiceal mucinous neoplasms

Fetched: February 24th, 2021, 5:02am GMT by T Evans

Eur J Surg Oncol. 2021 Jan;47(1):188-193. doi: 10.1016/j.ejso.2020.10.020. Epub 2020 Oct 16.

ABSTRACT

INTRODUCTION: Low grade appendiceal mucinous neoplasms (LAMN) are known to metastasise to the peritoneum resulting in pseudomyxoma peritonei (PMP). Literature suggests that the long-term outcome is dependent on the cellular grade of the peritoneal histology, less is known about the risk to patients with acellular mucinosis (AM) alone. This study aims to review long-term outcomes in patients with PMP treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC), whose peritoneal histology was AM secondary to LAMN.

METHODS: Pathological and treatment outcomes were collected from a prospectively maintained database between 2005 and 2019. Data was collected on patients with LAMN and AM diagnosed following CRS/HIPEC. A single institution performed the surgery and pathology reporting, samples reported by three different pathologists.

RESULTS: Of the 2079 patients with any appendiceal neoplasm referred between 2005 and 2019, 809 underwent CRS/HIPEC, 67 (8%) of those had PMP with purely AM secondary to a LAMN. In the AM group the median age was 59, 37 (55%) were female, follow up was for a median 39 (2-145) months. Inpatient mortality occurred in 1 patient (1.5%), disease specific mortality in 2 (3%), recurrence in 2 (3%) and disease progression in 1 (1.5%).

CONCLUSION: This study has identified AM secondary to LAMN as a low risk group for recurrence following CRS/HIPEC compared with epithelial pathology. Given such a low rate of recurrence we would recommend low intensity surveillance post CRS/HIPEC. Agreed standardised pathological assessment is required to exclude cellular material in specimens and diagnose AM.

PMID:33092969 | DOI:10.1016/j.ejso.2020.10.020
Mucinous Appendiceal Adenocarcinoma Penetrated the Quadratus Lumborum Muscle Posteriorly and Presented as Gluteal Abscess Managed Aggressively with Drainage Followed by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Combined with Int

Fetched: February 24th, 2021, 5:02am GMT by Ayman Zaki Azzam

Indian J Surg Oncol. 2020 Sep;11(Suppl 1):128-130. doi: 10.1007/s13193-020-01090-x. Epub 2020 May 21.

ABSTRACT

Appendiceal mucinous adenocarcinoma is a term that was given for invasive appendiceal tumors. It is always present with pseudomyxoma peritonei which is a complex disease. These tumors have the tendency to surround the primary tumor then extend primarily and extensively all over the peritoneal cavity as the primary site of metastases. The invasion through the abdominal wall muscles is rare but the penetration and passing through these muscles to the subcutaneous tissue are extremely rare. A 62-year-old male patient known to have pseudomyxoma peritonei presented with gluteal abscess. After 2 weeks from the abscess drainage, the patient underwent cytoreductive surgery (CRS) combined with intraoperative radiotherapy (IORT) and hyperthermic intraperitoneal chemotherapy (HIPEC). He tolerated the operation well and discharged home in a good condition. Even with extensive appendiceal mucinous neoplasms presented with the penetration of the abdominal wall muscles, still aggressive management should be considered.

PMID:33088148 | PMC:PMC7534762 | DOI:10.1007/s13193-020-01090-x
Laparoscopic cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for a limited low-grade pseudomyxoma peritonei-a video vignette

Fetched: February 24th, 2021, 5:02am GMT by Álvaro Arjona-Sánchez

Colorectal Dis. 2021 Jan;23(1):331-332. doi: 10.1111/codi.15409. Epub 2020 Nov 10.

NO ABSTRACT

PMID:33070418 | DOI:10.1111/codi.15409
Local Protocol for Management of Low-Grade Appendiceal Mucinous Neoplasm (LAMN)

Fetched: February 24th, 2021, 5:02am GMT by Mohamed Boshnaq

Indian J Surg Oncol. 2020 Sep;11(3):355-359. doi: 10.1007/s13193-020-01147-x. Epub 2020 Jun 23.

ABSTRACT

The management and surveillance of low-grade appendiceal mucinous neoplasm (LAMN) is a confusing topic in the colorectal MDM. This study was aiming to provide a local protocol for our trust for such cases. From prospectively maintained database, patients who underwent appendicectomy between 2011 and 2017 were identified. Those with histological confirmation of LAMN were included. Retrospective analysis of operative notes, investigations and follow-up, return to theatres or development of pseudomyxoma peritonei (PMP) was performed. Four thousand six hundred twenty-eight patients had appendicectomy; 36 were diagnosed with LAMN. Age range was 30-88 (20 females). Fifteen had their operation as emergency and 13 elective, either for abnormal-looking appendix on CT scan or as part of elective right hemicolectomy. In 8 patients, LAMNs were diagnosed incidentally during other surgery either intraoperatively or on histology. Two patients had mucus in the peritoneal cavity (not sampled). The remaining 34 had either contained mucocele or inflamed appendix. Regarding follow-up, 28 patients had CT scans only, and 2 had ultrasound scan (USS). Two had both CT and USS. Three had CT and MRI scans. One patient did not have radiological investigations. Eighteen patients had colonoscopies (50%). Three patients had no follow-up, while 33 had 5-year follow-up. Ten patients are still currently under follow-up. None of the 36 patients required further surgeries related to LAMN, and none has developed PMP to date of the study. We have developed a local protocol based on our findings and literature review for management and surveillance of LAMN in line with national centres.

PMID:33013110 | PMC:PMC7501353 | DOI:10.1007/s13193-020-01147-x
Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy with or without early post-operative intraperitoneal chemotherapy for appendix neoplasms with peritoneal metastases: A propensity score analysis

Fetched: February 24th, 2021, 5:02am GMT by Mikael L Soucisse

Eur J Surg Oncol. 2021 Jan;47(1):157-163. doi: 10.1016/j.ejso.2020.08.026. Epub 2020 Sep 6.

ABSTRACT

INTRODUCTION: - Early post-operative intraperitoneal chemotherapy (EPIC) can be used after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with resectable peritoneal metastases (PM). Whether EPIC adds any benefit is debatable.

METHODS: - We performed a retrospective case-control analysis of patients with PM of appendiceal origin treated by CRS + HIPEC ± EPIC at Uppsala University Hospital between 2004 and 2012. The 206 patients were divided into two groups depending on if they received EPIC or not. The two groups were propensity-matched with a 1:1 ratio. The patients in the EPIC group were mostly operated in the first three years of the unit's experience.

RESULTS: - After matching, 76 patients were left in each group. The groups were similar, except for the proportion of histological subtypes (p = 0.021) and chemotherapy agents used for HIPEC (0.017). Survival outcomes were stratified by histology. The patients who received EPIC had a longer hospital and ICU length of stay (15.71 vs 14.28 days, p = 0.049), (1.45 vs 1.05 days, p = 0.002), respectively. Post-operative complications were similar in both groups. Overall Survival (OS) and recurrence-free survival (RFS) did not differ for the patients with low-grade histology. The patients with high-grade tumors who received EPIC had a significantly worse OS (p = 0.0088) while having the same RFS as the patients who did not receive EPIC.

CONCLUSION: Our results suggest there is no benefit of EPIC in patients with advanced appendiceal tumors while increasing hospital and ICU length of stays. A suboptimal group matching might influence our results.

PMID:32943277 | DOI:10.1016/j.ejso.2020.08.026
Visceral scalloping in pancreatic ascites: An uncommon manifestation of pancreatitis

Fetched: February 24th, 2021, 5:02am GMT by Antriksh Kumar

Pleura Peritoneum. 2020 Feb 26;5(1):20200101. doi: 10.1515/pp-2020-0101. eCollection 2020 Mar 1.

ABSTRACT

Scalloping of visceral organs like liver and spleen can cause certain peritoneal diseases. It has usually been described with pseudomyxoma peritonei and peritoneal carcinomatosis. Occasionally, it has also been described with certain benign conditions like peritoneal tuberculosis. We describe visceral scalloping in setting of pancreatic diseases. We believe that pancreatic fluid collections exert significant pressure on the visceral organs to result in scalloping of the visceral surfaces.

PMID:32934975 | PMC:PMC7469503 | DOI:10.1515/pp-2020-0101
Clinicopathological Characteristics of Pseudomyxoma Peritonei Originated from Ovaries

Fetched: February 24th, 2021, 5:02am GMT by Fengcai Yan

Cancer Manag Res. 2020 Aug 21;12:7569-7578. doi: 10.2147/CMAR.S264474. eCollection 2020.

ABSTRACT

OBJECTIVE: This study aims to demonstrate clinicopathological characteristics and immunohistopathological phenotypes of pseudomyxoma peritonei (PMP) originated from ovaries.

METHODS: The primary origin of PMP was explored by reviewing H&E sections retrospectively and performing a series of immunohistochemical staining on CK7, CK20, CDX2, CEA, Villin, SATB2, CA125, ER, PR, and MUC.

RESULTS: Among 310 PMP patients, a few originated from extra-appendix, whereas eight cases were of ovarian origin (2.6%), including three teratoma-associated ovarian mucinous tumors and five primary ovarian mucinous tumors with spontaneous or iatrogenic rupture, respectively. Most peritoneal metastases were acellular mucin or low-grade mucinous carcinoma peritonei (6/8, 75%), while the rest were high-grade mucinous carcinoma peritonei (2/8, 25%). Tumors were positive for CK20, CDX2, CEA, and Villin. SATB2 was specifically diffuse positive in teratoma-associated ovarian mucinous tumors, and negative in primary ovarian mucinous tumors. Differential expression of MUC was observed in these tumors.

CONCLUSION: PMP of ovarian origin is extremely rare. The precise diagnosis requires serial sections of the appendix or suspicious tissue to exclude appendiceal mucinous neoplasms, as well as comprehensive analysis of clinical features, surgical findings, histopathological characteristics, and immunohistochemistry on specific biomarkers.

PMID:32904568 | PMC:PMC7457389 | DOI:10.2147/CMAR.S264474
Macroscopic and microscopic characteristics of low grade appendiceal mucinous neoplasms (LAMN) on appendectomy specimens and correlations with pseudomyxoma peritonei development risk

Fetched: February 24th, 2021, 5:02am GMT by Kirsten S Hegg

Ann Diagn Pathol. 2020 Oct;48:151606. doi: 10.1016/j.anndiagpath.2020.151606. Epub 2020 Aug 21.

ABSTRACT

Low grade appendiceal mucinous neoplasm (LAMN) is the primary source of pseudomyxoma peritonei (PMP). PMP may develop after seemingly complete resection of primary tumor by appendectomy, which is unpredictable due to lack of reliable prognostic indicators. We retrospectively reviewed 154 surgically resected LAMNs to explore if any of the macroscopic and microscopic characteristics may be associated with increasing risk of PMP development. Our major findings include: (1) As compared to those without PMP, the cases that developed PMP were more frequent to have (a) smaller luminal diameter (<1 cm) and thicker wall, separate mucin aggregations, and microscopic perforation/rupture, all suggestive of luminal mucin leakage; (b) microscopic acellular mucin presenting on serosal surface and not being confined to mucosa; and (c) neoplastic epithelium dissecting outward beyond mucosa, however, with similar frequency of neoplastic cells being present in muscularis propria. (2) Involvement of neoplastic cells or/and acellular mucin at surgical margin did not necessarily lead to tumor recurrence or subsequent PMP, and clear margin did not absolutely prevent PMP development. (3) Coexisting diverticulum, resulted from neoplastic or non-neoplastic mucosa being herniated through muscle-lacking vascular hiatus of appendiceal wall, was seen in a quarter of LAMN cases, regardless of PMP. The diverticular portion of tumor involvement was often the weakest point where rupture occurred. In conclusion, proper evaluation of surgical specimens with search for mucin and neoplastic cells on serosa and for microscopic perforation, which are of prognostic significance, should be emphasized.

PMID:32889392 | DOI:10.1016/j.anndiagpath.2020.151606
Immune response activation following hyperthermic intraperitoneal chemotherapy for peritoneal metastases: A pilot study

Fetched: February 24th, 2021, 5:02am GMT by Giammaria Fiorentini

World J Clin Oncol. 2020 Jun 24;11(6):397-404. doi: 10.5306/wjco.v11.i6.397.

ABSTRACT

BACKGROUND: Hyperthermic intraperitoneal chemotherapy (HIPEC) for peritoneal metastases (PM) is considered to be feasible, safe and to improve survival.

AIM: To investigate whether an immune response is activated following HIPEC for PM.

METHODS: Six patients were enrolled in this study. Peripheral blood samples were obtained from each patient prior to (day 0) and post-procedure (day 30), and used to evaluate the number of CD3⁺ total, CD3⁺/CD4⁺ T-Helper, CD3⁺/CD8⁺ cytotoxic T, CD3⁺/CD56⁺ natural killer and CD19⁺ B lymphocyte numbers, and CD4⁺: CD8⁺ T lymphocyte ratios.

RESULTS: The total numbers of CD3⁺, CD3⁺/CD4⁺ T-Helper, CD3⁺/CD8⁺ cytotoxic T, CD3⁺/CD56⁺ natural killer and CD19⁺ B lymphocytes, and CD4⁺: CD8⁺ lymphocyte ratios were increased in all but one patient 30 d following the cytoreductive surgery-HIPEC procedure, and these increases were significant (P ≤ 0.05) for CD3⁺/CD4⁺ T Helper and CD3⁺/CD8⁺ cytotoxic T lymphocyte numbers.

CONCLUSION: This report provides the first evidence that HIPEC exhibits immunomodulating activity in PM patients, resulting in generalized activation of the adaptive immune response. Moreover, the majority of lymphocyte populations increased following HIPEC and continued to be elevated several weeks following the procedure, consistent with a potential authentic immunomodulating effect rather than a normal inflammatory response, to be fully characterised in future studies.

PMID:32874953 | PMC:PMC7450817 | DOI:10.5306/wjco.v11.i6.397
Borderline epithelial ovarian tumors: what the radiologist should know

Fetched: February 24th, 2021, 5:02am GMT by K T Flicek

Abdom Radiol (NY). 2020 Aug 29. doi: 10.1007/s00261-020-02688-z. Online ahead of print.

ABSTRACT

Ovarian borderline tumors are neoplasms of epithelial origin that are typically present in young patients and tend to have a less aggressive clinical course than malignant tumors. Accurate diagnosis and staging of borderline tumors has important prognostic and management implications (like fertility-sparing procedures) for women of child-bearing age. This article will review the sonographic, CT, and MRI features of borderline epithelial ovarian tumors with histopathologic correlation. Borderline tumors have less soft tissue and thinner walls/septations than malignant tumors. Serous borderline tumors more commonly have papillary projections, which can simulate the appearance of a sea anemone. Mucinous borderline tumors often are larger, multi-cystic, and more commonly unilateral. The borderline mucinous tumors may also present with pseudomyxoma peritonei, which can make it difficult to distinguish from malignant mucinous carcinoma. Ultrasound is usually the first-line modality for imaging these tumors with MRI reserved for further characterizing indeterminate cases. CT is best used to stage tumors for both locoregional and distant metastatic disease. Overall, however, the imaging features overlap with both benign and malignant ovarian tumors. Despite this, it is important for the radiologist to be familiar with the imaging appearances of borderline tumors because they can present in younger patients and may benefit from different clinical/surgical management.

PMID:32860524 | DOI:10.1007/s00261-020-02688-z
Multicystic peritoneal mesothelioma treated with complete cytoreductive surgery, peritonectomy and hyperthermic intra-peritoneal chemotherapy-A case report

Fetched: February 24th, 2021, 5:02am GMT by Naveen Padmanabhan

Int J Surg Case Rep. 2020;74:152-157. doi: 10.1016/j.ijscr.2020.07.074. Epub 2020 Aug 8.

ABSTRACT

BACKGROUND: Multicystic Peritoneal mesothelioma is a rare and distinct variety of peritoneal mesothelioma with borderline malignant potential. Conventional Tumor bulking has been associated with recurrence of 45-50 %. Hence a comprehensive treatment with Complete cytoreductive surgery with involved field peritonectomy (CRS) and Hyperthermic Intra-peritoneal chemotherapy (HIPEC) is being increasingly adopted for MCPM.

CASE PRESENTATION: A 47 year old lady evaluated for peri-menopausal disturbance was diagnosed to have a multicystic lesion in the pelvis. With a preoperative suspicion of diagnosis of pseudomyxoma peritonei, CRS with HIPEC was planned. On exploration a diffuse multicystic mass was found in omentum and pouch of douglas with typical morphological features of MCPM. Complete cytoreduction was achieved with anterolateral and sub-diaphragmatic peritonectomy, omentectomy and panhystrectomy. HIPEC was performed with cisplatin 50 mg/m² for 40 min. Pathological examination revealed MCPM of omentum and uterine surface with focal clusters of mesothelial proliferation. However there was low proliferative activity 1-2 %.

DISCUSSION: MCPM presents with wide spread peritoneal spread but with relative sparing of visceral invasion. Literature review suggests the disease spread is similar to PMP and treatment with CCRS and HIPEC has yielded long term survivals in MCPM.

CONCLUSIONS: This patient with voluminous disease burden in abdomen required surgical management and HIPEC for her condition. Whether CCRS alone without HIPEC can be an alternative for limited disease will be interesting research for future clinical reports.

PMID:32846277 | PMC:PMC7452569 | DOI:10.1016/j.ijscr.2020.07.074
Molecular and clinicopathological features of appendiceal mucinous neoplasms

Fetched: February 24th, 2021, 5:02am GMT by Yuka Yanai

Virchows Arch. 2020 Aug 21. doi: 10.1007/s00428-020-02906-5. Online ahead of print.

ABSTRACT

Appendiceal mucinous tumors (AMTs) include low-grade mucinous appendiceal neoplasms (LAMNs), high-grade mucinous appendiceal neoplasms (HAMNs), and mucinous adenocarcinomas (MACs). We collected 51 AMT samples (LAMN: 34, HAMN: 8, MAC: 9). Three of the eight HAMN cases contained LAMN components, and four out of nine MAC cases contained LAMN and/or HAMN components within the tumor. A next-generation sequencing (NGS) cancer hotspot panel was used to analyze 11 pure LAMN, 4 HAMN, and 3 MAC cases. The results revealed KRAS and GNAS as the most frequently mutated genes. Sanger sequencing was then performed to detect KRAS, GNAS, and TP53 mutations in the remaining 31 cases and RNF43 mutations in all cases. KRAS/GNAS and TP53 mutations occurred exclusively in pure LAMNs; however, five LAMN cases had mutations in both KRAS and GNAS. RNF43 mutations almost exclusively occurred with KRAS/GNAS mutations in pure LAMNs. In MAC and HAMN, KRAS/GNAS mutation status was nearly preserved between lower-grade areas. Most of the detected RNF43 mutations was missense type. RNF43 mutations were detected in both components of MAC with lower-grade area; however, RNF43 mutations detected in these two lesions were entirely different. RNF43 mutations were detected in only one of the eight HAMN patients, which was the sole case without pseudomyxoma peritonei (PMP). None of the four MAC patients with RNF43 mutation showed PMP. These findings suggest that RNF43 mutations occur at a later stage of MAC development and do not associate with PMP. Furthermore, a gradual transition from LAMN to MAC via HAMN could be considered.

PMID:32821969 | DOI:10.1007/s00428-020-02906-5
Clinicopathologic Features of Low-grade Appendiceal Mucinous Neoplasm: A Single-institution Experience of 117 Cases

Fetched: February 24th, 2021, 5:02am GMT by Phoenix D Bell

Am J Surg Pathol. 2020 Nov;44(11):1549-1555. doi: 10.1097/PAS.0000000000001551.

ABSTRACT

Low-grade appendiceal mucinous neoplasm (LAMN) is an enigmatic tumor that lacks the capacity for classic invasion but can dissect through the appendiceal wall, causing pseudomyxoma peritonei (PMP). Most large studies of the histologic spectrum of LAMN and its rate of associated PMP include cases submitted from outside institutions, potentially skewing their findings. We identified 117 cases of LAMN at our institution. Hematoxylin and eosin-stained slides from each were reviewed, and clinical and pathologic parameters were noted. The patients were 76 females and 41 males, with a mean age of 60 years. Presenting symptoms were available for 113 patients; the majority (56%) were symptomatic, typically with abdominal pain. Ninety-one tumors (78%) were grossly dilated, and the entire appendix was submitted in 88 (75%) cases. Median lesion size was 5.5 cm. Ninety-two cases (79%) demonstrated epithelial denudation; these were often markedly dilated and contained intraluminal or mural microcalcifications. Thirty-two (27%) had a mucosal Schwann cell proliferation. On the basis of the American Joint Committee on Staging eighth edition cancer staging manual, of 117 cases, 66% were staged as pTis, 9% as pT3, 24% as pT4a, and 2% as pT4b. Ten cases (9%) were associated with histopathologic evidence of disseminated PMP. Only 1 patient died of disease, while 3 were alive with disease at last follow-up. Previous LAMN studies have utilized both departmental and extradepartmental material; our single-institution review demonstrated lower rates of PMP than some prior studies. Some LAMNs may be markedly dilated with extensive denudation, making the diagnosis difficult to confirm microscopically and ultimately requiring submission of the entire appendix for histologic evaluation.

PMID:32796173 | DOI:10.1097/PAS.0000000000001551
Neoadjuvant Intraperitoneal Chemotherapy in Patients with Pseudomyxoma Peritonei-A Novel Treatment Approach

Fetched: February 24th, 2021, 5:02am GMT by Aruna Prabhu

Cancers (Basel). 2020 Aug 7;12(8):2212. doi: 10.3390/cancers12082212.

ABSTRACT

Neoadjuvant intravenous chemotherapy in patients with pseudomyxoma peritonei (PMP) has not shown convincing results. The effectiveness of neoadjuvant intraperitoneal (IP) chemotherapy has never been reported. This prospective, non-randomized phase II study included patients with PMP treated between May 2017 and December 2018, who were not considered suitable for primary cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). The majority of patients were treated with laparoscopic HIPEC (oxaliplatin 200 mg/m², 60 min, 43 °C). IP chemotherapy was started 2 weeks after docetaxel 40 mg/m² + cisplatin 40 mg/m², accompanied by oral S1 (tegafur, gimeracil, and oteracil) (50 mg/m²) for 14 days, followed by one week rest. Clinical parameters and complications were recorded. In total, 22/27 patients qualified for CRS and HIPEC after neoadjuvant treatment. A complete cytoreduction (Completeness of cytoreduction Score 0/1) could be achieved in 54.5%. The postoperative morbidity rate was 13.6% and mortality was rate 4.5%. In total, 20/22 patients had major pathological tumor responses. The mean drop in CEA was 28.2% and in the peritoneal carcinomatosis index (PCI) was 2.6. Positive or suspicious cytology turned negative in 69.2% of patients. Thus, for PMP patients who were not amenable for primary surgery, the majority received complete cytoreduction after treatment with neoadjuvant IP chemotherapy, with satisfying tumor regression and with low complication rates. The oncological benefit in terms of survival with this new treatment regimen needs to be proven.

PMID:32784670 | PMC:PMC7465601 | DOI:10.3390/cancers12082212
Updates in Appendix Pathology: The Precarious Cutting Edge

Fetched: February 24th, 2021, 5:02am GMT by Norman J Carr

Surg Pathol Clin. 2020 Sep;13(3):469-484. doi: 10.1016/j.path.2020.05.006. Epub 2020 Jul 11.

ABSTRACT

Mucinous appendiceal tumors include low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm, and mucinous adenocarcinoma. Nonmucinous adenocarcinomas are less frequent. Recent consensus guidelines and the latest edition of the World Health Organization classification will allow consistent use of agreed nomenclature. Accurate diagnosis is important not only for patient management but also to allow comparison of results between centers and tumor registries. Serrated polyps are the most common benign polyp in the appendix. They need to be distinguished from low-grade appendiceal mucinous neoplasm, which can also mimic other benign conditions. Goblet cell adenocarcinomas are a distinctive type of appendiceal neoplasm.

PMID:32773195 | DOI:10.1016/j.path.2020.05.006
Emergency laparoscopic ileocecal resection for a low-grade appendiceal mucinous neoplasm with impending rupture: A case report.

Fetched: February 22nd, 2021, 5:01am GMT

Int J Surg Case Rep. 2021 Mar;80:105636. doi: 10.1016/j.ijscr.2021.02.022. Epub 2021 Feb 9.

ABSTRACT

INTRODUCTION AND IMPORTANCE: We report the case of a patient with a low-grade appendiceal mucinous neoplasm (LAMN) who underwent emergency laparoscopic ileocecal resection to avoid the metastatic spread of tumor cells due to an impending rupture.

CASE PRESENTATION: A 55-year-old woman presented to our hospital with pain in the right lower quadrant of the abdomen. Computed tomography revealed a markedly tense appendiceal mucinous tumor with surrounding inflammation, and laboratory test results showed elevated serum C-reactive protein (7.47 mg/dL), indicating impending rupture of the appendix. Magnetic resonance imaging revealed nodules inside the appendix, suggesting the possibility of appendiceal cancer. We performed emergency laparoscopic ileocecal resection with regional lymph node dissection. The tumor was pathologically diagnosed as a LAMN without rupture.

CLINICAL DISCUSSION: LAMN is classified as a clinically malignant tumor because it can cause pseudomyxoma peritonei due to perforation or the presence of residual tissue. Although an appendectomy would be appropriate for LAMN if the tumor margin is secured, ileocecal resection with lymph node dissection is necessary when preoperative discrimination of appendiceal cancer is impossible.

CONCLUSION: Further studies of preoperative imaging for appropriate differential diagnosis were necessary.

PMID:33609942 | PMC:PMC7900346 | DOI:10.1016/j.ijscr.2021.02.022
A case of Endometrioid endometrial adenocarcinoma with synchronous low-grade Appendiceal mucinous neoplasm and Pseudomyxoma peritonei.

Fetched: February 21st, 2021, 5:01am GMT

J Minim Access Surg. 2021 Feb 3. doi: 10.4103/jmas.JMAS_177_20. Online ahead of print.

ABSTRACT

Pseudomyxoma peritonei (PMP) is a rare condition usually associated with ruptured low-grade mucinous neoplasm of the appendix. Rarely, PMP can originate from mucinous adenocarcinoma of the ovary. However, the coexistence of adenocarcinoma of the endometrium and appendiceal mucinous neoplasm has not been reported. We present the case of a post-menopausal woman with endometrioid endometrial adenocarcinoma with unexpected low-grade appendiceal mucinous neoplasm and PMP.

PMID:33605928 | DOI:10.4103/jmas.JMAS_177_20
Western Australian Peritonectomy Service: a review of the first 6 years of operation of a low-volume peritonectomy centre.

Fetched: February 18th, 2021, 5:01am GMT

ANZ J Surg. 2021 May;91(5):885-889. doi: 10.1111/ans.16627. Epub 2021 Feb 15.

ABSTRACT

BACKGROUND: Cytoreductive surgery (CRS) with hyperthermic intraperitoneal early chemotherapy has gained acceptance as the standard of care for peritoneal surface malignancy over the past decade. This study reports the results of the first 6 years of activity of the Western Australian Peritonectomy Service to compare outcomes of a low-volume centre with world standards.

METHODS: An audit of all patients who received CRS ± hyperthermic intraperitoneal early chemotherapy treatment at the Western Australian Peritonectomy Service in its first 6 years of operation was performed and results were compared to the recent world literature.

RESULTS: A total of 130 patients were treated with 150 CRS procedures, including 50 pseudomyxoma peritonei (PMP), 53 colorectal cancers (CRCs), 27 appendix adenocarcinomas (AAs) and 20 other cancers. The median operating time was 12 h with median length of hospital stay of 15 days. Perioperative mortality was 0.67% and Clavien-Dindo III/IV morbidity was 20%. The mean packed red cell transfusion requirement was 0.34 units/case. Post-discharge readmission rate was 25% and the return to theatre rate was 13%. The 4-year overall survival rates for PMP, CRC and AA were 97%, 49% and 81%, respectively. The 3-year disease-free survival rates for PMP, CRC and AA were 74%, 26% and 36%, respectively.

CONCLUSION: A CRS centre averaging 25 cases per year can achieve strong outcomes in line with high-volume world centres.

PMID:33590661 | DOI:10.1111/ans.16627

Trial - Pseudomyxoma Peritonei

Flat Dose vs. Weight-based IP Chemotherapy for CRS/HIPEC

Posted: March 3rd, 2021, 3:00pm GMT

Condition: Peritoneal Carcinomatosis
Interventions: Drug: Mitomycin C, flat dose 40 mg; Drug: Mitomycin C, weight-based dose 12.5 mg/m2
Sponsor: Prakash Pandalai
Recruiting
Impact of Cardiac Coherence on Anxiety in Patients Operated on for a Peritoneal Carcinosis

Posted: July 18th, 2019, 2:00pm GMT

Conditions: Peritoneal Carcinomatosis; Pseudomyxoma Peritonei; Mesothelioma Peritoneum
Interventions: Other: Cardiac coherence; Other: Standard care
Sponsor: Institut du Cancer de Montpellier - Val d'Aurelle
Recruiting

1 2

← Phyllodes Rhabdomyosarcoma →

Rare Cancer News & Clinical Trials » PMP and Mucinous Tumors

Feeds

PMP and Mucinous Tumors (166 unread)

Posted: June 15th, 2021, 4:05am GMT

Posted: June 10th, 2021, 5:35pm GMT

Posted: June 10th, 2021, 5:35pm GMT

Posted: June 7th, 2021, 8:12am GMT

Posted: June 6th, 2021, 3:00am GMT

Posted: May 28th, 2021, 3:00pm GMT

Posted: May 19th, 2021, 10:05am GMT

Posted: May 17th, 2021, 7:05pm GMT

Posted: May 16th, 2021, 5:00am GMT

Posted: May 11th, 2021, 5:09pm GMT

Posted: May 4th, 2021, 2:43pm GMT

Posted: May 4th, 2021, 2:43pm GMT

Posted: May 4th, 2021, 2:43pm GMT

Posted: May 2nd, 2021, 5:00am GMT

Posted: April 28th, 2021, 5:00am GMT

Posted: April 27th, 2021, 8:30am GMT

Posted: April 23rd, 2021, 5:00am GMT

Posted: April 21st, 2021, 12:39pm GMT

Posted: April 20th, 2021, 5:00am GMT

Posted: April 18th, 2021, 5:00am GMT

Posted: April 17th, 2021, 5:00am GMT

Posted: April 17th, 2021, 5:00am GMT

Posted: April 16th, 2021, 5:00am GMT

Posted: April 14th, 2021, 3:00am GMT

Posted: April 14th, 2021, 3:00am GMT

Posted: April 13th, 2021, 5:00am GMT

Posted: April 11th, 2021, 9:00am GMT

Posted: April 9th, 2021, 5:00am GMT

Posted: April 7th, 2021, 3:30am GMT

Posted: April 3rd, 2021, 5:00am GMT

Posted: March 29th, 2021, 5:00am GMT

Posted: March 28th, 2021, 5:00am GMT

Posted: March 25th, 2021, 5:00am GMT

Posted: March 25th, 2021, 5:00am GMT

Posted: March 25th, 2021, 12:30am GMT

Posted: March 23rd, 2021, 5:00am GMT

Posted: March 21st, 2021, 5:00am GMT

Posted: March 14th, 2021, 6:00am GMT

Posted: March 13th, 2021, 6:00am GMT

Posted: March 11th, 2021, 3:35am GMT

Posted: March 10th, 2021, 4:02am GMT

Posted: March 9th, 2021, 6:00am GMT

Posted: March 7th, 2021, 6:00am GMT

Posted: March 6th, 2021, 6:00am GMT

Posted: February 28th, 2021, 6:00am GMT

Posted: February 26th, 2021, 8:07pm GMT

Posted: February 26th, 2021, 8:07pm GMT

Posted: February 26th, 2021, 6:00am GMT

Posted: February 23rd, 2021, 6:00am GMT

Posted: February 22nd, 2021, 6:00am GMT

Posted: February 10th, 2021, 7:27am GMT

Posted: January 31st, 2021, 1:21am GMT

Posted: January 18th, 2021, 6:00am GMT

Posted: January 15th, 2021, 6:00am GMT

Posted: January 14th, 2021, 6:00am GMT

Posted: January 14th, 2021, 6:00am GMT

Posted: January 14th, 2021, 6:00am GMT

Posted: December 21st, 2020, 6:00am GMT

Posted: December 1st, 2020, 6:00am GMT

Posted: November 25th, 2020, 5:58am GMT

Posted: October 22nd, 2020, 5:00am GMT

Posted: October 12th, 2020, 5:00am GMT

Posted: October 7th, 2020, 5:00am GMT

Posted: September 30th, 2020, 5:00am GMT

Posted: September 17th, 2020, 5:00am GMT

Posted: September 8th, 2020, 5:00am GMT

Posted: September 8th, 2020, 5:00am GMT

Posted: September 6th, 2020, 5:00am GMT

Posted: August 27th, 2020, 5:00am GMT

Posted: August 25th, 2020, 5:00am GMT

Posted: July 14th, 2020, 5:00am GMT

Posted: June 24th, 2020, 5:00am GMT

Posted: June 23rd, 2020, 5:00am GMT