Rare Cancer News & Clinical Trials » Hemangioendothelioma

Hemangioendothelioma (100 unread)

Embolization assisted surgical excision of primary intra-osseous hemangioma of the zygomatic bone

Fetched: November 26th, 2021, 5:02am GMT by Manjusha Ingle

Oral Oncol. 2021 Nov 22;123:105632. doi: 10.1016/j.oraloncology.2021.105632. Online ahead of print.

NO ABSTRACT

PMID:34823158 | DOI:10.1016/j.oraloncology.2021.105632

Permalink for 'Intracranial mesenchymal tumors with FET-CREB fusion are composed of at least two epigenetic subgroups distinct from meningioma and extracranial sarcomas'

Intracranial mesenchymal tumors with FET-CREB fusion are composed of at least two epigenetic subgroups distinct from meningioma and extracranial sarcomas

Fetched: November 26th, 2021, 5:02am GMT by Emily A Sloan

Brain Pathol. 2021 Nov 25:e13037. doi: 10.1111/bpa.13037. Online ahead of print.

ABSTRACT

'Intracranial mesenchymal tumor, FET-CREB fusion-positive' occurs primarily in children and young adults and has previously been termed intracranial angiomatoid fibrous histiocytoma (AFH) or intracranial myxoid mesenchymal tumor (IMMT). Here we performed genome-wide DNA methylation array profiling of 20 primary intracranial mesenchymal tumors with FET-CREB fusion to further study their ontology. These tumors resolved into two distinct epigenetic subgroups that were both divergent from all other analyzed intracranial neoplasms and soft tissue sarcomas, including meningioma, clear cell sarcoma of soft tissue (CCS), and AFH of extracranial soft tissue. The first subgroup (Group A, 16 tumors) clustered nearest to but independent of solitary fibrous tumor and AFH of extracranial soft tissue, whereas the second epigenetic subgroup (Group B, 4 tumors) clustered nearest to but independent of CCS and also lacked expression of melanocytic markers (HMB45, Melan A, or MITF) characteristic of CCS. Group A tumors most often occurred in adolescence or early adulthood, arose throughout the neuroaxis, and contained mostly EWSR1-ATF1 and EWSR1-CREB1 fusions. Group B tumors arose most often in early childhood, were located along the cerebral convexities or spinal cord, and demonstrated an enrichment for tumors with CREM as the fusion partner (either EWSR1-CREM or FUS-CREM). Group A tumors more often demonstrated stellate/spindle cell morphology and hemangioma-like vasculature, whereas Group B tumors more often demonstrated round cell or epithelioid/rhabdoid morphology without hemangioma-like vasculature, although robust comparison of these clinical and histologic features requires future study. Patients with Group B tumors had inferior progression-free survival relative to Group A tumors (median 4.5 vs. 49 months, p = 0.001). Together, these findings confirm that intracranial AFH-like neoplasms and IMMT represent histologic variants of a single tumor type ('intracranial mesenchymal tumor, FET-CREB fusion-positive') that is distinct from meningioma and extracranial sarcomas. Additionally, epigenomic evaluation may provide important prognostic subtyping for this unique tumor entity.

PMID:34821426 | DOI:10.1111/bpa.13037

Capillary hemangioma of the sphenoid sinus with intrasellar and parasellar extensions

Fetched: November 26th, 2021, 5:02am GMT by Maciej Pas

Radiol Case Rep. 2021 Nov 10;17(1):156-160. doi: 10.1016/j.radcr.2021.10.011. eCollection 2022 Jan.

ABSTRACT

Capillary hemangiomas are benign vascular tumors usually diagnosed in infancy and involving skin and soft tissues. We report a rare case of an adult capillary hemangioma of the left sphenoid sinus extending into the intrasellar and parasellar regions. A 71-year-old woman presented with complaints of headaches and diplopia. Magnetic resonance imaging revealed a T2-hyperintense mass-like lesion involving the left posterior ethmoid and sphenoid sinuses and left cavernous sinus, that had extended into the intrasellar and parasellar regions. Dynamic contrast-enhanced computed tomography revealed enhancing vessel-like structures within the lesion during the arterial phase, which were observed to expand further in the late phase of the examination. This characteristic feature indicated increased vascularity, suggesting the presence of a capillary hemangioma. The patient underwent endoscopic resection via the endonasal transpterygoid approach, and the lesion was diagnosed histopathologically as a capillary hemangioma. To aid diagnosis and clinical management, radiologists should be aware of the imaging findings related to capillary hemangiomas.

PMID:34820041 | PMC:PMC8599932 | DOI:10.1016/j.radcr.2021.10.011

The effects of surgical treatment and sclerotherapy for intramuscular venous malformations: a comparative clinical study

Fetched: November 26th, 2021, 5:02am GMT by Yun Hyun Kim

Arch Plast Surg. 2021 Nov;48(6):622-629. doi: 10.5999/aps.2021.00913. Epub 2021 Nov 15.

ABSTRACT

BACKGROUND: Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision.

METHODS: Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement.

RESULTS: Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group.

CONCLUSIONS: Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.

PMID:34818708 | DOI:10.5999/aps.2021.00913

Liver pathology in pregnancy

Fetched: November 25th, 2021, 5:02am GMT by Jacqueline E Birkness-Gartman

Pathol Int. 2021 Nov 24. doi: 10.1111/pin.13186. Online ahead of print.

ABSTRACT

Liver dysfunction occurs in up to 3% of pregnancies and can be due to pregnancy-associated liver injury, exacerbation of pre-existing liver disease, or co-incident with pregnancy. The most common form of pregnancy-associated liver injury is intrahepatic cholestasis of pregnancy (ICP). This condition is typically benign and self-limited, but is associated with fetal morbidity and mortality with high levels of serum bile acids. Acute fatty liver of pregnancy (AFLP) and the hypertensive disorders of pregnancy (including pre-eclampsia, eclampsia, and hemolysis, elevated liver enzymes, and low platelets [HELLP] syndrome) are more commonly associated with maternal and fetal complications and may necessitate expedient delivery. Histologically, ICP shows nonspecific features of cholestasis, while AFLP and the hypertensive disorders have more characteristic histologic findings. While not a true liver disease, hyperemesis gravidarum can cause elevated liver enzymes. Pregnant patients are at increased risk of developing severe hepatitis E and herpesvirus infections, Budd-Chiari syndrome, and gallstones, and they may also experience worsening of known chronic liver disease. Mass lesions in pregnancy including hemangiomas, focal nodular hyperplasia, and hepatocellular adenomas and carcinomas can present unique challenges for diagnosis and management. This review will explore the pathophysiology, presentation, histologic features, and management of these conditions.

PMID:34818440 | DOI:10.1111/pin.13186

Unusual Mesenchymal Tumors of the Lower Gastrointestinal Tract: When You Hear Hoofbeats in the Night, Do Not Forget the Zebras

Fetched: November 25th, 2021, 5:02am GMT by Marco Barella

Pathobiology. 2021;88(6):434-442. doi: 10.1159/000517962. Epub 2021 Oct 4.

ABSTRACT

INTRODUCTION: Little information about clinical presentation of mesenchymal tumors of the lower gastrointestinal (GI) tract due to their extreme heterogeneity is available for clinical management. Usually, small solitary asymptomatic polyps are accidently found during a screening colonoscopy performed for hematochezia, abdominal pain, constipation, diarrhea, and bowel obstruction. In this case series, we illustrate our experience with mesenchymal tumors of the lower GI tract, which are a group of unusual and quite challenging lesions.

CASE PRESENTATION: We retrospectively collected mesenchymal tumors of the lower GI tract in our institution (Fondazione IRCSS Ca' Granda - Ospedale Maggiore Policlinico di Milano) during the last 10 years. We reviewed the histological slides, and, when necessary, we performed immunohistochemical analyses to better characterize the tumors. A total of 99 cases were identified: 45 GISTs, 42 lipomas, 4 leiomyomas, 3 Kaposi sarcomas, 1 schwannoma, 1 ganglioneuroma, 1 hemangioma, 1 inflammatory fibroid polyp, and 1 challenging case of spindle cell melanoma. We focused on the most rare entities excluding therefore all GISTs and lipomas from re-evaluation.

CONCLUSION: Mesenchymal tumors of the lower GI tract represent a highly heterogeneous group of lesions encompassing GISTs, lipomas, smooth muscle tumors (leiomyoma and leiomyosarcoma), GI schwannomas, inflammatory fibroid polyps, solitary fibrous tumors, and other unusual spindle cell tumors. Immunohistochemistry and, in selected cases, molecular biology remain a useful tool which, in addition to a meticulous study of the morphology, helps the pathologist in the tangled jungle of differential diagnosis.

PMID:34814148 | DOI:10.1159/000517962

Indocyanine Green Assisted Endoscopic Transorbital Excision of Lateral Orbital Apex Cavernous Hemangioma

Fetched: November 25th, 2021, 5:02am GMT by Ben Chat Fong Ng

World Neurosurg. 2021 Nov 19:S1878-8750(21)01762-9. doi: 10.1016/j.wneu.2021.11.060. Online ahead of print.

NO ABSTRACT

PMID:34808413 | DOI:10.1016/j.wneu.2021.11.060

Successful Outcome After Intralesional Curettage for Spindle Cell Hemangioma of Fibula in an Infant: A Case Report

Fetched: November 23rd, 2021, 5:01am GMT by Tao Han

Front Pediatr. 2021 Nov 4;9:767927. doi: 10.3389/fped.2021.767927. eCollection 2021.

ABSTRACT

Spindle cell hemangioma (SCH), a non-neoplastic reactive vascular lesion, rarely locates in bones. We herein report a successful case of intralesional curettage for an infant with SCH of fibula. An 11-month-old boy was admitted to our center with a painless mass in the right proximal calf. Preoperative digital radiograph demonstrated a massive vascular lesion with an irregular bone destruction of proximal fibula. The lesion was removed via the intralesional curettage approach and pathologically diagnosed as SCH. The patient gained bone structure recovery of right proximal fibula. Two years after the surgery, he experienced no local recurrence. For the management of SCH of fibula with partial bone destruction, we suggest early-stage intralesional curettage as its safety and effectiveness.

PMID:34805052 | PMC:PMC8600265 | DOI:10.3389/fped.2021.767927

Radiological features of renal pelvic hemangioma: a case series

Fetched: November 23rd, 2021, 5:01am GMT by Huajun Yu

Transl Androl Urol. 2021 Oct;10(10):3766-3772. doi: 10.21037/tau-21-489.

ABSTRACT

BACKGROUND: Renal pelvic hemangioma (RPH) is often misdiagnosed as renal pelvis cancer (RPC) due to its similarity in presentation, and there are few reports on the imaging findings of RPH. This study is aimed at improving the understanding of imaging findings specific for RPH by a retrospective analysis of the imaging findings of RPH.

METHODS: RPH cases confirmed by pathology and with high-quality images were collected in the analysis. Nine cases of RPH were enrolled, of which 6 cases underwent ultrasound (US); 7 cases underwent computed tomography (CT), including 6 cases with an enhanced scan; and 2 cases underwent magnetic resonance imaging (MRI), including 1 case with an enhanced scan. All images of cases were analyzed and sorted independently by two senior attending radiologist blinded to the pathological results, according to the imaging indicators, such as the density and intensity on CT and MRI respectively. When the opinions between radiologists were inconsistent, images were re-evaluated together until a consensus was reached.

RESULTS: Nine cases of RPH were collected from 5 males and 4 females, aged 16-70 years old, with a median age of 41 years. Five cases were located in the left kidney and 4 cases were located in the right kidney. The clinical symptoms mostly presented with hematuria. Nine cases demonstrated solitary masses, with 4 cases with blurred margins and 5 cases with well-defined margins. The size of the mass was about 1.5-8.0 cm, and the median size was 2.5 cm. The US showed mostly hypoechoic masses and color Doppler flow imaging (CDFI) showed minimal to no blood flow signal. Unenhanced CT scans showed mostly hypodensity and mostly mild continuous enhancement on an enhanced scan. The intensity of lesions was commonly heterogeneous on MRI due to hemorrhage and necrosis. One case showed mild continuous enhancement on an enhanced MRI scan.

CONCLUSIONS: The imaging findings of RPH commonly present as a focal lesion with blurred or well-defined margins, mild and continuous enhancement, and no cachexia of the clinical symptoms. RPH should be differentiated from malignant tumors of the renal pelvis for treatment.

PMID:34804820 | PMC:PMC8575578 | DOI:10.21037/tau-21-489

Permalink for 'Successful laparoscopic removal of ovarian borderline cystadenoma in patient with multiorgan hemangioma and ventriculoperitoneal shunt: a case report and literature review'

Successful laparoscopic removal of ovarian borderline cystadenoma in patient with multiorgan hemangioma and ventriculoperitoneal shunt: a case report and literature review

Fetched: November 23rd, 2021, 5:01am GMT by Chenyi Zhang

J Surg Case Rep. 2021 Nov 11;2021(11):rjab380. doi: 10.1093/jscr/rjab380. eCollection 2021 Nov.

ABSTRACT

As medical diagnosis and treatment level improved, patients with ventriculoperitoneal shunt (VPS) live longer and may develop conditions that need laparoscopic surgery. The safety of laparoscopy in patients with VPS continues to be challenged due to pneumoperitoneum. Here, we report a patient with medical history of VPS and hemangioma, diagnosed with ovarian borderline mucinous cystadenoma, received laparoscopic surgery in supine position and 10 mmHg pneumoperitoneum pressure, in which no clamping or externalizing catheter, no perioperative or postoperative complications. We also present a literature review and discuss the precautions needing considering during laparoscopy. For patients with VPS, laparoscopic surgery can be recognized as a potentially safe and feasible procedure.

PMID:34804476 | PMC:PMC8599040 | DOI:10.1093/jscr/rjab380

Angiolymphoid Hyperplasia with Eosinophilia: Many Syllables, Many Unanswered Questions

Fetched: November 23rd, 2021, 5:01am GMT by Allyson Brahs

J Clin Aesthet Dermatol. 2021 Jun;14(6):49-54. Epub 2021 Jun 1.

ABSTRACT

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, benign inflammatory vasoproliferation. The literature is divided regarding whether it embodies a vascular neoplasm or a reactive process secondary to various stimuli. ALHE presents as solitary or clustered papules or nodules primarily on the head and neck, especially on or around the auricle. Histologically, ALHE is characterized by a proliferation of blood vessels lined by plump epithelioid endothelial cells and a prominent perivascular infiltrate rich in lymphocytes and eosinophils. ALHE follows a benign clinical course, yet treatment is challenging because of its high recurrence rate. We present the case of a 37-year-old Filipino man with lesions located on the central face. Kimura disease was considered due to his age, sex, and ethnicity; however, his clinical features-specifically, the presence of discrete papules and lack of lymphadenopathy-and his histological findings were consistent with ALHE. He reported trauma prior to the onset of the lesions, suggesting a reactive etiology.

PMID:34804356 | PMC:PMC8594535

Giant hepatic hemangioma causing prolonged fever and indicated for resection

Fetched: November 23rd, 2021, 5:01am GMT by Chihiro Yoshimizu

Intern Med. 2021 Nov 20. doi: 10.2169/internalmedicine.8405-21. Online ahead of print.

ABSTRACT

Hepatic hemangiomas are benign liver tumors, and most of them progress asymptomatically. We report a case of hepatic hemangioma considered the cause of fever. A 53-year-old woman had a fever of 40°C for about 3 months without infection. Hepatic hemangiomas with internal bleeding of 10 cm in size on liver S8/7 and S3/2 were observed. These were resected laparoscopically for diagnostic treatment. She was afebrile after the operation. The pathological diagnosis was hematoma inside cavernous hemangioma. It should be noted that a bleeding hepatic hemangioma may cause fever of unknown origin and be indicated for resection.

PMID:34803101 | DOI:10.2169/internalmedicine.8405-21

MR Imaging of Vascular Malformations and Tumors of Head and Neck

Fetched: November 23rd, 2021, 5:01am GMT by Ahmed Abdel Khalek Abdel Razek

Magn Reson Imaging Clin N Am. 2022 Feb;30(1):199-213. doi: 10.1016/j.mric.2021.07.005.

ABSTRACT

Soft tissue vascular anomalies show a wide heterogeneity of clinical manifestations and imaging features. MR imaging has an important role in the diagnosis and management of vascular lesions of the head and neck. MR angiography is mandatory in cases of arteriovenous and combined malformations to assess the high-flow nature/component of the lesions and plan therapy. Infantile hemangiomas can be differentiated from congenital hemangiomas by clinical course. Reactive vascular tumors have nonspecific features similar to infantile hemangiomas. Locally malignant and malignant vascular tumors have irregular borders, infiltration of different tissue planes, and lower apparent diffusion coefficient values than benign vascular tumors.

PMID:34802579 | DOI:10.1016/j.mric.2021.07.005

Tips, Tricks, and Pearls to Expertly Treat Common Pediatric Dermatologic Conditions

Fetched: November 21st, 2021, 5:01am GMT by James Treat

Dermatol Clin. 2022 Jan;40(1):95-102. doi: 10.1016/j.det.2021.09.009.

ABSTRACT

Pediatric dermatology is an incredibly rewarding field. Children are resilient and funny and just want to know that you are advocating for their best interests. Teaming up with children and their parents can lead to fantastic therapeutic alliances and success. The author has divided this chapter based on a handful of common pediatric dermatologic diseases and what he sees as some of the main clinical and therapeutic tips and tricks that have helped him in his practice.

PMID:34799040 | DOI:10.1016/j.det.2021.09.009

Topical timolol in dermatology: infantile hemangiomas and beyond

Fetched: November 20th, 2021, 5:02am GMT by M Alzaid

Clin Exp Dermatol. 2021 Nov 19. doi: 10.1111/ced.15021. Online ahead of print.

ABSTRACT

Timolol, a non-selective β-adrenergic receptor blocker, is well-tolerated and is becoming increasingly popular in dermatology especially after its use in the management of infantile hemangiomas. Its effects are mainly due to vasoconstriction, inhibition of angiogenesis and keratinocyte migration promotion for re-epithelialization and wound healing. We review the evidence behind the use of timolol in several dermatological conditions including infantile hemangiomas, pyogenic granulomas, Kaposi's sarcoma, chronic wound healing, post-surgical wounds, acne vulgaris, rosacea, eczema and red scrotum syndrome.

PMID:34798680 | DOI:10.1111/ced.15021

Liver metastasis mimicking a liver cyst of a thymoma in a 38-year-old immunocompromised patient

Fetched: November 19th, 2021, 5:01am GMT by Golo Petzold

Z Gastroenterol. 2021 Nov 18. doi: 10.1055/a-1659-4419. Online ahead of print.

ABSTRACT

Cystic liver lesions (CLL) are common and, in the majority of cases, benign. However, the range of differential diagnoses of CLL is wide. A combination of medical history, blood test results, and imaging can help find the correct diagnosis. We report the case of a 38-year-old immunocompromised female patient with a history of thymectomy and postoperative radiation 3 years prior due to thymoma. Subsequently, the patient was referred to our department for clarification of a cystic liver lesion. During short-term follow-up, the lesion increased in size, and due to the contrast agent behavior in the ultrasound and MRI examination, the suspicion of a biliary cystadenocarcinoma was considered.Furthermore, imaging showed several subcentimetric liver lesions of unknown dignity. Finally, pericystectomy and atypical partial liver resection was performed. Histology revealed a cystic metastasis of the malignant B3 thymoma and a cavernous hemangioma. Liver metastases of a thymoma are rare, and this is the first case of a cystic liver metastasis of a thymoma. The presented case illustrates that in the management of CLLs beside imaging techniques, the medical history with previous conditions should be considered, especially in past malignancies.

PMID:34794195 | DOI:10.1055/a-1659-4419

Multicentric Castleman disease in a DOCK8-deficient patient with Orf virus infection

Fetched: November 19th, 2021, 5:01am GMT by Elif Tugce Aydin Goker

Pediatr Allergy Immunol. 2021 Nov 18. doi: 10.1111/pai.13666. Online ahead of print.

NO ABSTRACT

PMID:34792227 | DOI:10.1111/pai.13666

Permalink for 'Whole Exome Sequencing Identifies Somatic Variants in an Oral Composite Hemangioendothelioma Characterized by YAP1-MAML2 Fusion'

Whole Exome Sequencing Identifies Somatic Variants in an Oral Composite Hemangioendothelioma Characterized by YAP1-MAML2 Fusion

Fetched: November 19th, 2021, 5:01am GMT by Ioannis G Koutlas

Head Neck Pathol. 2021 Nov 17. doi: 10.1007/s12105-021-01393-7. Online ahead of print.

ABSTRACT

Composite hemangioendothelioma (CHE) is considered a borderline malignant vascular tumor defined by an admixture of distinct vascular neoplastic components. A 21-year-old female is presented herein with a 1 cm painless mandibular vestibular mass of less than a year duration. The infiltrating tumor was characterized by dilated vascular channels lined by endothelial cells with bland ovoid or round nuclei exhibiting, occasionally, hobnail/matchstick-like arrangement. Intravascular cell proliferations with hyaline globular deposits were also present. Additionally, lobular spindle and epithelioid cell aggregates, as well as slit-like spaces exhibiting a retiform or angiosarcomatous morphology were observed. Intracytoplasmic signet-ring or lipoblast-like vacuolization was also noted. Mitotic activity was exceptionally rare. Vascular spaces and the stroma featured lymphocytes and plasma cells. Neoplastic cells were positive for CD31, CD34, D2-40 and ERG, negative for CAMTA1 and synaptophysin, while type IV collagen highlighted the plasmalemma of most vessels and hyaline globules. Fluorescence in situ hybridization revealed gene rearrangements in both YAP1 and MAML2 genes, in keeping with a YAP1-MAML2 fusion. Whole exome sequencing (WES) identified three missense mutations FLT1 [p.R1016G], PIK3CA [p.H1047L], and C11orf42 [p.A304P] and a mitochondrial frameshift insertion MT-ND4 [c.1107_1108insC; p.P370fs]. These WES results suggest that FLT1 and/or PIK3CA variants may contribute to tumor growth/transformation while the MT-ND4 variant may relate to proliferation, angiogenesis and/or inhibition of apoptosis.

PMID:34791601 | DOI:10.1007/s12105-021-01393-7

Benign Pericardial Hemangioma-A Rare Cause of Cardiac Tamponade

Fetched: November 19th, 2021, 5:01am GMT by Dhanya Jacob

Indian J Radiol Imaging. 2021 Nov 13;31(3):754-757. doi: 10.1055/s-0041-1736405. eCollection 2021 Jul.

ABSTRACT

Pericardial tumors are very rare. It can be primary or secondary, of which secondary tumors are more common. Pericardial hemangiomas are extremely rare primary neoplasms and there are only very few cases published in the literature. These patients can be asymptomatic. When symptomatic, they present with dyspnea, palpitation, or atypical chest pain. Severity of symptoms depends on the size and location of the tumor. Pericardial effusion with features of cardiac tamponade can lead to a life-threatening situation. Here, we report a case of pericardial hemangioma in a patient who presented with breathlessness and tamponade which was diagnosed preoperatively with computed tomography.

PMID:34790331 | PMC:PMC8590538 | DOI:10.1055/s-0041-1736405

IDH1 R132C and ERC2 L309I Mutations Contribute to the Development of Maffucci's Syndrome

Fetched: November 19th, 2021, 5:01am GMT by Peng Cheng

Front Endocrinol (Lausanne). 2021 Nov 1;12:763349. doi: 10.3389/fendo.2021.763349. eCollection 2021.

ABSTRACT

BACKGROUND: Maffucci's syndrome is characterized by the coexistence of multiple enchondromas and soft-tissue hemangiomas. It has been clear that somatic mosaic isocitrate dehydrogenase type 1 (IDH1) or isocitrate dehydrogenase type 2 (IDH2) mutations are associated with Maffucci's syndrome and Ollier disease, but the mechanisms underlying hemangiomas of the Maffucci's syndrome is still obscure. This study aimed to determine the mechanism of hemangiomas in Maffucci's syndrome.

METHODS: We received a 26-year-old female patient with typical Maffucci's syndrome, and exome sequencing was conducted using DNA from her peripheral blood and enchondroma tissues. Somatic mutations were characterized by a comparative analysis of exome sequences and further confirmed by the sequencing of PCR products derived from original blood and tissue samples. The mutations of an additional 69 patients with Ollier disease were further tested. The functional impacts of these somatic mutations on Maffucci's syndrome, especially the development of hemangiomas, were evaluated.

RESULTS: We reported a typical case of Maffucci's syndrome, which was confirmed by both imaging findings and pathology. Through exome sequencing of this patient's DNA samples, we identified an R132C mutation in the isocitrate dehydrogenase type 1 (IDH1) gene and an L309I mutation in the ELKS/RAB6-interacting/CAST family member 2 (ERC2) gene in this patient. Approximately 33.3% of the clones were positive for the IDH1 R132C mutation, and 19.0% of the clones were positive for the ECR2 L309I mutation. The IDH1 R132C mutation was detected in most of the patients with Ollier disease (51/69 patients), and the mean frequency of this mutation was 63.3% in total sequence readouts, but the ECR2 L309I mutation was absent in all of the patients with Ollier disease. In vitro experiments confirmed that the IDH1 R132C mutation promotes chondrocyte proliferation, and the ERC2 L309I mutation enhances angiogenesis.

CONCLUSIONS: Our results suggest that while IDH1 is a known pathogenic gene in enchondromatosis, ERC2 is a novel gene identified in Maffucci's syndrome. The somatic L309I mutation of ERC2 contributes to the pathogenesis of hypervascularization to facilitate the development of hemangiomas in Maffucci's syndrome. The combination of the IDH1 R132C and ERC2 L309I mutations contributes to the development of Maffucci's syndrome, and these results may enable further research on the pathogenesis of Maffucci's syndrome.

PMID:34790172 | PMC:PMC8591216 | DOI:10.3389/fendo.2021.763349

Long-term follow-up of liver alveolar echinococcosis using echinococcosis multilocularis ultrasound classification

Fetched: November 19th, 2021, 5:01am GMT by Jasmin Schuhbaur

World J Gastroenterol. 2021 Oct 28;27(40):6939-6950. doi: 10.3748/wjg.v27.i40.6939.

ABSTRACT

BACKGROUND: When Echinococcus multilocularis infects humans as a false intermediate host, alveolar echinococcosis (AE) usually manifests primarily intrahepatically and is initially asymptomatic. If the disease remains undiagnosed and untreated, progressive growth occurs, reminiscent of malignant tumours. The only curative therapy is complete resection, which is limited to localised stages, and palliative drug therapy is used otherwise. Consequently, early diagnosis and reliable detection of AE lesions are important. For this reason, abdominal ultrasonography, as the most common primary imaging for AE, relies on classification systems.

AIM: To investigate how hepatic AE lesion sonomorphology changes over time in the Echinococcosis Multilocularis Ulm Classification (EMUC)-ultrasound (US) classification.

METHODS: Based on data from Germany's national echinococcosis database, we evaluated clinical and US imaging data for 59 patients according to the AE case definition in our preliminary retrospective longitudinal study. There had to be at least two liver sonographies ≥ 6 mo apart, ≥ 1 hepatic AE lesion, and complete documentation in all US examinations. The minimum interval between two separately evaluated US examinations was 4 wk. The AE reference lesion was the largest hepatic AE lesion at the time of the first US examination. To classify the sonomorphologic pattern, we used EMUC-US. In addition to classifying the findings of the original US examiner, all reference lesions at each examination time point were assigned EMUC-US patterns in a blinded fashion by two investigators experienced in US diagnosis. Statistical analysis was performed using SAS version 9.4 (SAS Institute Inc., Cary, NC, United Stated). P values < 0.05 were considered statistically significant.

RESULTS: The preliminary study included 59 patients, 38 (64.5%) women and 21 (35.6%) men. The mean age at initial diagnosis was 59.9 ± 16.9 years. At the time of initial ultrasonography, a hailstorm pattern was present in 42.4% (25/59) of cases, a hemangioma-like pattern in 16.9% (10/59), a pseudocystic pattern in 15.3% (9/59), and a metastasis-like pattern in 25.4% (15/59). For the hailstorm pattern, the average lesion size was 67.4 ± 26.3 mm. The average lesion size was 113.7 ± 40.8 mm with the pseudocystic pattern and 83.5 ± 27.3 mm with the hemangioma-like pattern. An average lesion size of 21.7 ± 11.0 mm was determined for the metastasis-like pattern. Although the sonomorphologic pattern remained unchanged in 84.7% (50/59) of AE reference lesions, 15.3% (9/59) showed a change over time. A change in pattern was seen exclusively for AE lesions initially classified as hemangioma-like or pseudocystic. A total of 70% (7/10) of AE lesions initially classified as hemangioma-like showed a relevant change in pattern over time, and 85.7% (6/7) of these were secondarily classified as having a hailstorm pattern, with the remainder (1/7; 14.3%) classified as having a pseudocystic pattern. A total of 22.2% (2/9) of AE lesions initially classified as pseudocystic showed a relevant change in pattern over time and were classified as having a hailstorm pattern. For AE lesions initially classified as having a hailstorm or metastatic pattern, no pattern change was evident. All patients with pattern change were on continuous drug therapy with albendazole.

CONCLUSION: The sonomorphology of hepatic AE lesions may change over time. The hemangioma-like and pseudocystic patterns are affected.

PMID:34790016 | PMC:PMC8567478 | DOI:10.3748/wjg.v27.i40.6939

Choriocarcinoma misdiagnosed as cerebral hemangioma: A case report

Fetched: November 18th, 2021, 5:01am GMT by Hui-Qiong Huang

World J Clin Cases. 2021 Oct 26;9(30):9174-9181. doi: 10.12998/wjcc.v9.i30.9174.

ABSTRACT

BACKGROUND: Choriocarcinoma is a subtype of gestational trophoblastic disease, gestational trophoblastic neoplasia. Patients with brain metastasis are rare and information on the optimal treatment and patient outcome is limited. In order to improve the prognosis of this disease, accurate and timely treatments are very important for the patient of brain metastasis by choriocarcinoma.

CASE SUMMARY: A 17-year-old unmarried girl was misdiagnosed with a cerebral hemangioma with intracranial hemorrhage in a local hospital after presentation with severe head pain. She underwent craniotomy three times for treatment. The pathological results of posterior intracranial hematoma showed choriocarcinoma, and the patient was diagnosed as choriocarcinoma (21 points in stage IV). After uterine artery embolization, etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine chemotherapy for 7 cycles, and whole brain radiotherapy, the patient achieved remission. She has been followed for 2 years with no signs of tumor recurrence.

CONCLUSION: For female patients of childbearing age with an intracranial hematoma, the possibility of brain metastasis by choriocarcinoma should be considered. It is necessary to obtain a detailed history, including menstruation, beginning age of first sex, contraception, etc. The level of β-human chorionic gonadotropin should be tested at the beginning, and a stratified treatment should be administered according to the International Federation of Gynecology and Obstetrics staging and World Health Organization prognostic scoring systems.

PMID:34786402 | PMC:PMC8567533 | DOI:10.12998/wjcc.v9.i30.9174

Pediatric vascular tumors of the liver: Review from the pathologist's point of view

Fetched: November 18th, 2021, 5:01am GMT by Fleur Cordier

World J Hepatol. 2021 Oct 27;13(10):1316-1327. doi: 10.4254/wjh.v13.i10.1316.

ABSTRACT

Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature, histologic overlap and the rarity of some entities. Here we give an up-to-date overview of the most important entities. We discuss the clinic, histology and pathophysiology of hepatic congenital and infantile heman gioma, hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma.

PMID:34786168 | PMC:PMC8568580 | DOI:10.4254/wjh.v13.i10.1316

Permalink for 'Efficacy Evaluation of 755-nm Long-Pulse Alexandrite Laser Combined with 0.5% Timolol Maleate Eye Drops in the Treatment of Thicker Infantile Hemangioma'

Efficacy Evaluation of 755-nm Long-Pulse Alexandrite Laser Combined with 0.5% Timolol Maleate Eye Drops in the Treatment of Thicker Infantile Hemangioma

Fetched: November 18th, 2021, 5:01am GMT by Jianming Wu

Clin Cosmet Investig Dermatol. 2021 Nov 9;14:1621-1628. doi: 10.2147/CCID.S330411. eCollection 2021.

ABSTRACT

PURPOSE: Assessment of the clinical effectiveness and safety of 755-nm long-pulse alexandrite laser combined with 0.5% timolol maleate eye drops in treating thicker infantile hemangioma (IH).

MATERIALS AND METHODS: Retrospective analysis of IH treated with 755-nm long-pulse alexandrite laser and topical timolol in the Second Affiliated Hospital of Wenzhou Medical University from October 2019 to October 2020. Seventy-eight cases were included, with a five-week laser treatment interval. Treatment status was documented during the 35 weeks before each treatment, the effect was assessed at the visual analog scale (VAS), and side effects were recorded. During the 6-month follow-up period, the recurrence and residual skin lesions were monitored. The relationship between IH thickness, treatment duration and VAS was analyzed.

RESULTS: Among the 78 children with hemangioma, 4 children were treated with a combination of propranolol, fractional laser and cinnamyl alcohol injection due to poor curative effect. Finally, the lesions were effectively alleviated. At the 5th, 15th, 25th, and 35th weeks of treatment, the average VAS of 74 children were 3.56 ± 1.20, 4.61 ± 1.43, 5.63 ± 1.60, and 6.63 ± 1.72, respectively. We analyzed VAS in different thickness groups with Repeated Measures Analysis of Variance(RMANOVA). The results show that the VAS of the thickness 2-3 mm and 3-5mm groups were higher than the 5-7mm and 7-8mm groups (F group = 440.54, P <0.05, F time = 448.31, P <0.05). During the 6-month follow-up period, none of the 74 children relapsed and the residual skin lesions gradually vanished.

CONCLUSION: Combined treatment of IHs with a 755-nm long-pulse alexandrite laser and 0.5% timolol maleate eye drops which has apparent clinical efficacy and safety reduce residual skin lesions and decrease the IH recurrence rate.

PMID:34785921 | PMC:PMC8590841 | DOI:10.2147/CCID.S330411

Ultrasound and alternative multimodality imaging of intra-abdominal and pelvic cystic masses in the newborn

Fetched: November 17th, 2021, 5:01am GMT by Weiyong Lee

Ultrasound. 2021 Nov;29(4):241-251. doi: 10.1177/1742271X20984814. Epub 2021 Feb 17.

ABSTRACT

Introduction: Ultrasound is used commonly to detect and diagnose intra-abdominal and pelvic cystic masses in the newborn as it is easily available, relatively low cost, and non-invasive. Discussion: The diagnosis can be made or narrowed down by determining the location, size, sonographic features, organ involvement, and internal vascularity. The differential diagnoses include hydronephrosis, multicystic dysplastic kidney, adrenal haemorrhage, cystic teratomas, ovarian cysts, enteric cysts, meconium cysts, and liver haemangiomas. With the appropriate radiological knowledge, the ultrasound practitioner can help obtain an accurate diagnosis. Conclusion: This pictorial essay will familiarise the reader with the different common causes of intra-abdominal and pelvic masses detected on ultrasound through a wide range of conditions. The role of ultrasound in the evaluation of these conditions will be discussed and alternative imaging correlates will be offered.

PMID:34777544 | PMC:PMC8579366 | DOI:10.1177/1742271X20984814

Pulmonary sclerosing pneumocytoma mimicking carcinoid on preoperative imaging in a 14-year-old female

Fetched: November 17th, 2021, 5:01am GMT by Amitabha Mandal

Indian J Thorac Cardiovasc Surg. 2021 Nov;37(6):676-679. doi: 10.1007/s12055-021-01153-w. Epub 2021 Apr 21.

ABSTRACT

Pulmonary sclerosing pneumocytoma is a rare benign neoplasm of the lung, commonly occurs in middle-aged persons with a marked female predominance. Earlier, it was known as sclerosing hemangioma. Here, we present a case of pulmonary sclerosing pneumocytoma which was diagnosed as carcinoid of the lung, based on imaging, in a 14-year-old female. Besides radiology, the uniqueness of this case lies in the young age (14 years) of the patient. She was presented with a 3.3 × 2.5 × 2.2 cm soft tissue density mass with a tiny speck of calcification in the anterior basal segment of the lower lobe of the right lung. Based on imaging findings on fluorodeoxyglucose positron emission tomography (FDG PET) scan and DOTANOC scan, a diagnosis of carcinoid was made. We performed a video-assisted thoracoscopic right lower lobectomy. Histopathological examination showed features of pulmonary sclerosing pneumocytoma. Surgery is an established method of treatment for pulmonary sclerosing pneumocytoma. Enucleation, lobectomy, sleeve resection are possible treatment options. To define the role of adjuvant therapy, further direct evidence is required. The metastatic potential of this disease is yet to be established.

PMID:34776665 | PMC:PMC8546018 | DOI:10.1007/s12055-021-01153-w

Management of Urethral Hemangioma

Fetched: November 17th, 2021, 5:01am GMT by Ridwan Alam

Urology. 2021 Nov 12:S0090-4295(21)01038-4. doi: 10.1016/j.urology.2021.10.027. Online ahead of print.

ABSTRACT

Hemangiomas are benign proliferations of blood vessels, and urethral hemangiomas are extremely rare presentations of this condition. As the condition is quite rare, there is very little literature about best management strategies. The best resources for determining management are case reports which focus on treatment via transurethral ablation or endoscopic resection. However, there is no guidance on management of urethral hemangiomas which are refractory to these minimally invasive methods. Herein, we summarize the literature for urethral hemangioma management, present the case of a male presenting with refractory hematuria due to a hemangioma in the penile urethra, and describe the operative technique for the open excision and reconstruction of this hemangioma.

PMID:34780843 | DOI:10.1016/j.urology.2021.10.027

Case Report: Simultaneous Resection of Bone Tumor and CAD/CAM Titanium Cranioplasty in Fronto-Orbital Region

Fetched: November 17th, 2021, 5:01am GMT by Konstantin S Yashin

Front Surg. 2021 Oct 27;8:718725. doi: 10.3389/fsurg.2021.718725. eCollection 2021.

ABSTRACT

Background: Simultaneous resection of bone tumors in the fronto-naso-orbital region is a great challenge due to the need for adequate reconstruction of the facial skeleton. Pre-operative virtual planning of resection margins and the simultaneous fabrication of the cranioplasty using computer-aided design/computer-aided manufacturing (CAD/CAM) technology could allow combining the tumor resection and cosmetic restoration steps into a single procedure. Methods: We present five consecutive cases of patients with bone tumors of the fronto-naso-orbital region. The indications for surgery included: (1) the presence of a major cosmetic defect; (2) progressive tumor growth. The histological examination revealed vascular malformation, hemangioma, and fibrous dysplasia in two cases. Tumor resection was performed with the help of a drilling template in form of a tumor. The computer-designed cranioplasty formed based on the non-involved side of the skull of the patient was manufactured. In one patient, the reconstruction was performed using two separate implants. Results: The position of the implant fits in with pre-operative planning in two cases; in those cases, the additional trimming of the implant or bone defect was required. Good cosmetic outcomes were noted in all patients, and no complications occurred. No repeat surgery was necessary. The template has proved to have high application potential. Conclusion: Simultaneous resection and CAD/CAM cranioplasty in the case of bone tumors in the fronto-orbital region is a promising technique with the aim of minimizing operation time and achieving a good esthetic outcome.

PMID:34778354 | PMC:PMC8578092 | DOI:10.3389/fsurg.2021.718725

Notch pathway inhibitor DAPT accelerates in vitro proliferation and adipogenesis in infantile hemangioma stem cells

Fetched: November 17th, 2021, 5:01am GMT by Xing Xu

Oncol Lett. 2021 Dec;22(6):854. doi: 10.3892/ol.2021.13115. Epub 2021 Oct 26.

ABSTRACT

The Notch signaling pathway is crucial in both adipogenesis and tumor development. It serves a vital role in the development and stability of blood vessels and may be involved in the proliferative phase of infantile hemangiomas, which express various related receptors. Therefore, it was hypothesized that the Notch signaling pathway inhibitor N-[N-(3,5-difluorophenacetyl)-L-alanyl]-S-phenylglycine t-butyl ester (DAPT), a γ-secretase inhibitor, might help accelerate the regression of infantile hemangiomas. The present in vitro study evaluated whether inhibition of the Notch signaling pathway using DAPT could alter adipogenesis in hemangioma stem cells (HemSCs) derived from infantile hemangioma (IH) specimens. A total of 20 infants (age, ≤6 months) with hemangiomas who had not yet received any treatment were selected, and their discarded hemangioma tissues were obtained. HemSCs were isolated from the fresh, sterile IH specimens and treated with DAPT. Reverse transcription-quantitative PCR and western blotting were used to demonstrate the inhibition of the Notch signaling pathway by DAPT. A proliferation assay (Cell Counting Kit-8), oil red O staining, flow cytometry and a transwell assay were used to detect proliferation, adipogenesis, apoptosis and migration of HemSCs. Treatment with DAPT upregulated the expression levels of CCAAT/enhancer-binding protein (C/EBP) α, C/EBPβ, peroxisome proliferator-activated receptor-γ, adiponectin and insulin-like growth factor 1, and promoted the proliferation, apoptosis, migration and lipid accumulation in HemSCs in vitro. Targeting the Notch signaling pathway using DAPT may potentially accelerate the regression of infantile hemangiomas.

PMID:34777588 | PMC:PMC8581475 | DOI:10.3892/ol.2021.13115

Sclerosing Angiomatoid Nodular Transformation (SANT): A Rare Splenic Tumor and Unusual Cause of Anemia

Fetched: November 14th, 2021, 5:04am GMT by Bee Shan Ong

Am J Case Rep. 2021 Nov 13;22:e933598. doi: 10.12659/AJCR.933598.

ABSTRACT

BACKGROUND Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign disease of the splenic red pulp of unknown etiology. Definite diagnosis is made on histopathology alone as it has no diagnostic radiologic characteristics. CASE REPORT We present a case of a large incidental splenic mass in a middle-aged man, whose refractory anemia resolved with splenectomy. Our initial imaging studies failed to differentiate this lesion from other splenic lesions like hamartoma and haemangioma. To the best of our knowledge, no SANT cases in the literature have been investigated with red cell scan, as performed in our patient, nor has any case had resolved anemia after treatment. Splenectomy was offered as malignancy could not be safely excluded. Histopathology confirmed the diagnosis of SANT. The patient made good recovery and had no signs of recurrence 2 years later. CONCLUSIONS SANT of the spleen is a rare condition that can mimic many other splenic tumors and poses diagnostic challenge when histopathology is unavailable. Our case adds to the number of SANT reported in literature in the hope of elucidating the pathophysiology of this rare condition.

PMID:34772906 | DOI:10.12659/AJCR.933598

Permalink for 'Efficacy and Safety of Interferon-Alpha 2b for Patients with Hepatic Epithelioid Hemangioendothelioma: Outcomes of a Case-Series Analysis'

Efficacy and Safety of Interferon-Alpha 2b for Patients with Hepatic Epithelioid Hemangioendothelioma: Outcomes of a Case-Series Analysis

Fetched: November 14th, 2021, 5:04am GMT by Xiaolei Liu

Cancer Manag Res. 2021 Nov 3;13:8273-8279. doi: 10.2147/CMAR.S334171. eCollection 2021.

ABSTRACT

BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor type. No effective medicine or standard treatment for HEH has been established.

PATIENTS AND METHODS: From March 2014 to April 2021, 62 patients with pathologically diagnosed HEH were observed regularly, and interferon-alpha 2b (IFN-a 2b) was administered to patients with progressive disease or reoccurrence. Adverse events (AEs) were assessed and recorded, and a tumor assessment scan was performed every 3 months.

RESULTS: A total of 42 patients with HEH received IFN-a 2b treatment in this study. No severe (grade ≥3) AEs were reported in the group overall. The most common treatment-related AEs in patients receiving IFN-a 2b were fever (50.0%) and fatigue (21.4%). Partial response and complete response were achieved in 20 patients (47.6%) and 2 patients (4.8%), respectively, and the objective response rate was 52.4%. Stable disease was observed in 12 patients (28.6%), and the disease control rate was 81.0%. Progressive disease was observed in 8 patients (19.0%). The 1-, 3-, and 5-year progression-free survival rates were 81.0%, 69.2%, and 62.3%, respectively. Only 1 patient died as a result of disease progression during the study. The 1-, 3-, and 5-year overall survival rates were 100%, 97.2%, and 97.2%, respectively.

CONCLUSION: IFN-a 2b is a safe and effective treatment for patients with HEH. The encouraging results with IFN-a 2b use make it a promising option for patients who have other types of epithelioid hemangioendothelioma; additional clinical trials are needed.

PMID:34764690 | PMC:PMC8572744 | DOI:10.2147/CMAR.S334171

Tetralogy of Fallot with haemangioma of the upper extremity in a child with biventricular failure: a diagnostic conundrum

Fetched: November 14th, 2021, 5:04am GMT by Mansi Verma

BMJ Case Rep. 2021 Nov 11;14(11):e247195. doi: 10.1136/bcr-2021-247195.

NO ABSTRACT

PMID:34764132 | PMC:PMC8587452 | DOI:10.1136/bcr-2021-247195

Permalink for 'Efficacy of Compound Danshen Dripping Pills combined with western medicine in the treatment of diabetic retinopathy: a systematic review and meta-analysis of randomized controlled trials'

Efficacy of Compound Danshen Dripping Pills combined with western medicine in the treatment of diabetic retinopathy: a systematic review and meta-analysis of randomized controlled trials

Fetched: November 14th, 2021, 5:04am GMT by Hui Huang

Ann Palliat Med. 2021 Oct;10(10):10954-10962. doi: 10.21037/apm-21-2563.

ABSTRACT

BACKGROUND: The Compound Danshen Dripping Pills have been widely used in the treatment of diabetic retinopathy (DR), but there is a lack of systematic review of reports on this topic. To explore the efficacy of Compound Danshen Dripping Pills combined with western medicine in the treatment of diabetic retinopathy, we conducted a meta-analysis.

METHODS: Randomized controlled trials published in the Chinese Medical Literature Database (CBM), Embase, PubMed, and Medline databases from January 2010 to August 2021 were searched. After screening the qualified literature, literature quality was evaluated by the Cochrane Handbook for Systematic Reviews of Interventions. Meta-analysis was performed on outcome measures including effective rate, visual field gray value, hemangioma volume, hemorrhagic plaque area, and visual acuity after diabetic retinopathy treatment with Compound Danshen Dripping Pills using Revman 5.3 analysis software to comprehensively evaluate the utility of Compound Danshen Dripping Pills.

RESULTS: A total of 167 documents were preliminarily searched, and 8 studies involving 524 patients were included for meta-analysis. Meta-analysis showed that the statistical value of the effective rate of diabetic retinopathy treatment in the intervention group and control group was OR =5.00, 95% CI: 2.84, 8.83, P<0.0001. The statistical value of visual field gray value comparison was MD =-0.93, 95% CI: -0.98, -0.89, P<0.00001. The statistical value of hemangioma volume was MD =-3.16, 95% CI: -3.48, -2.84, P<0.00001. The statistical value of hemorrhagic plaque area comparison was MD =-0.65, 95% CI: -0.97, -0.32, P<0.0001. The statistical value of visual acuity comparison was MD =0.15, 95% CI: 0.10, 0.19, P<0.00001.

DISCUSSION: The Compound Danshen Dripping Pills combined with western medicine are effective and safe in the treatment of diabetic retinopathy.

PMID:34763458 | DOI:10.21037/apm-21-2563

Hepatic epithelioid hemangioendothelioma: a diagnostic challenge of vascular tumors

Fetched: November 14th, 2021, 5:04am GMT by Carlos Pacheco-Molina

Cir Cir. 2021;89(S1):6-9. doi: 10.24875/CIRU.20000777.

ABSTRACT

Epitheloid hemangioendothelioma is a very rare tumor, with a variable presentation and unpredictable clinical behavior. The etiology and the triggering risk factors have not been specified. Unlike other primary liver tumors, it does not arise in the background of chronic liver disease. The approach is challenging due to the spectrum of possibilities and the need for immunohistochemistry to establish the definitive diagnosis. The information available so far is limited due to the few published cases, this favors that the therapeutic options are few or that there is insufficient evidence to standardize them when the lesion is not resectable.

PMID:34762620 | DOI:10.24875/CIRU.20000777

Clinical analysis of 16 cases of pulmonary epithelioid hemangioendothelioma

Fetched: November 11th, 2021, 5:01am GMT by J X Zhu

Zhonghua Jie He He Hu Xi Za Zhi. 2021 Nov 12;44(11):966-971. doi: 10.3760/cma.j.cn112147-20201218-01174.

ABSTRACT

Objective: To improve the diagnosis and treatment of pulmonary epithelioid hemangioendothelioma (P-EHE). Methods: Sixteen patients diagnosed with P-EHE in Fuzhou Pulmonary Hospital of Fujian Province from January 2009 to July 2020 were collected. Their gender, age, imaging findings, pathological characteristics, treatment protocols, survival and other clinical data were summarized and analyzed. Results: The ratio of male to female among the 16 patients was 1∶1; and the average age of onset was 47.75 years. Most cases of PEHE (9/16) were found by physical examination, while some cases developed respiratory symptoms such as cough, sputum, shortness of breath, hemoptysis, chest pain, etc. (7/16). In most patients the lesions were localized to the lungs (11/16), while bone metastasis (1/16), lymph node metastasis (1/16), and pleural metastasis (4/16) also occurred. The pathological tissues were obtained mainly through surgical thoracoscopy. Chest CT images showed multiple nodules in both lungs, with most of the nodules less than 2.0 cm in diameter, and calcifications were seen, while solitary nodules and masses were rare, and pleural metastases could be manifested as pleural thickening and pleural effusion. The pathological findings were well-defined eosinophilic nodules with irregularly arranged nest-like structures. Those eosinophilic nodules had few central cells and abundant peripheral cells, which extended into the alveolar cavity like papillae. The tumor cells were epithelioid with small atypia, and vacuoles and red blood cells could be seen in the cytoplasm of individual tumor cells. Immunohistochemically, the tumor cells were positive to CD34, CD31, Factor Ⅷ andvimentin (VIM). Follow-up of 0.5 to 11 years showed that four patients died, two lost to follow-up, and the rest of the patients were in good condition, with a median overall survival (OS) of 4.58 years. Conclusions: PEHE is a rare low-grade lung tumor with no specific clinical manifestations. It can be diagnosed with chest imaging and pathological immunohistochemistry. Moreover, there is currently no standard treatment for PEHE, and most patients have a good prognosis.

PMID:34758523 | DOI:10.3760/cma.j.cn112147-20201218-01174

Permalink for 'Early redox homeostasis disruption contributes to the differential cytotoxicity of imiquimod on transformed and normal endothelial cells'

Early redox homeostasis disruption contributes to the differential cytotoxicity of imiquimod on transformed and normal endothelial cells

Fetched: November 11th, 2021, 5:01am GMT by Rodrigo Rocco

Exp Dermatol. 2021 Nov 10. doi: 10.1111/exd.14499. Online ahead of print.

ABSTRACT

The off-label use of imiquimod (IQ) for hemangioma treatment has shown clinical benefits. We have previously reported a selective direct IQ-cytotoxic effect on transformed (H5V) vs. normal (1G11) endothelial cells (EC). In the present study, we investigated the mechanism underlying this selective cytotoxicity in terms of TLR7/8 receptor expression, NF-κB signalling and time-dependent modifications of oxidative stress parameters (ROS: reactive oxygen species, catalase and superoxide dismutase activities, GSH/GSSG and lipid peroxidation). TLR7/8 level was extremely low in both cell lines, and IQ did not upregulate TLR7/8 expression or activate NF-κB signalling. IQ significantly induced ROS in H5V after 2 h and strongly affected antioxidant defenses. After 12 h, enzyme activities were restored to baseline levels but a robust drop in GSH/GSSG persisted together with increased lipid peroxidation levels and a marked mitochondrial dysfunction. Although in normal IQ-treated EC some oxidative stress parameters were affected after 4 h, mitochondrial health and GSH/GSSG ratio remained notably unaffected after 12 h. Therefore, the early alterations (0-2 h) in transformed EC breached redox homeostasis as strongly as to enhance their susceptibility to IQ. This interesting facet of IQ as redox disruptor could broaden its therapeutic potential for other skin malignancies, alone or in adjuvant schemes.

PMID:34758172 | DOI:10.1111/exd.14499

Epithelioid Hemangioendothelioma in the Tongue: A Rare Case Report

Fetched: November 11th, 2021, 5:01am GMT by Deniz Surmeli Cirkin

Turk Patoloji Derg. 2021 Nov 10. doi: 10.5146/tjpath.2021.01560. Online ahead of print.

ABSTRACT

Epithelioid hemangioendothelioma is a rare malignant vascular neoplasm caused by the proliferation of neoplastic endothelial cells. Epithelioid hemangioendothelioma may develop in any organ, but it is commonly observed in the extremities. The tongue is a very unusual location for epithelioid hemangioendothelioma. A 55-year-old male patient presented to the outpatient head and neck clinic with lumps in the tongue, pain, and limitation of motion. The polypoid mass detected in the anterior midline of the tongue was excised. Microscopically, the tumor cells included slightly pleomorphic oval or round vesicular nuclei with an eosinophilic cytoplasm that variably contained vacuoles. There were 4 mitoses per 10 high power fields and there was no necrosis. In immunohistochemical study, the tumor cells were positively stained with CD31 and CD34 whereas they were negatively stained with TFE3, SMA, S-100, HHV-8 and EMA. The patient was diagnosed with "epitheloid hemangioendothelioma". Only ten cases have been reported in the tongue in the literature. Our case was the eleventh case, and we aimed to report this case as a rare entity with an unusual location.

PMID:34757619 | DOI:10.5146/tjpath.2021.01560

An incidental hepatic lesion in a cirrhotic liver

Fetched: November 11th, 2021, 5:01am GMT by Lan Zhang

Clin Res Hepatol Gastroenterol. 2021 Oct 28:101825. doi: 10.1016/j.clinre.2021.101825. Online ahead of print.

NO ABSTRACT

PMID:34757154 | DOI:10.1016/j.clinre.2021.101825

Epithelioid Hemangioma involving Large Arteries in the Skin

Fetched: November 11th, 2021, 5:01am GMT by Janina Markidan

J Cutan Pathol. 2021 Nov 9. doi: 10.1111/cup.14166. Online ahead of print.

ABSTRACT

Epithelioid hemangioma (EH) is a benign vascular lesion, typically consisting of small vascular channels lined by epithelioid endothelial cells and associated with a dense lymphocytic infiltrate with eosinophils. Here, we report a rare case of EH involving large arteries. The patient presented with a 9-month history of an asymptomatic nodule on the forehead, which was thought to be an epidermal inclusion cyst. Skin biopsy revealed large arteries with clusters of epithelioid cells in the vascular walls and lumen. Scattered eosinophils were noted in the walls. Adjacent areas showed groups of small-caliber vessels lined by prominent endothelial cells and associated with a dense lymphoid infiltrate with eosinophils. No significant cytologic atypia was noted. Given the presence of the classic small-vessel involvement, along with CD31 reactivity for the epithelioid cells in the large vessels, the findings are classified as EH involving large arteries, which is an uncommon subtype. There have only been a handful of such cases reported in the literature.

PMID:34755378 | DOI:10.1111/cup.14166

Itraconazole therapy for infant hemangioma: Two case reports

Fetched: November 11th, 2021, 5:01am GMT by Zhe Liu

World J Clin Cases. 2021 Oct 6;9(28):8579-8586. doi: 10.12998/wjcc.v9.i28.8579.

ABSTRACT

BACKGROUND: Infantile hemangiomas (IHs) are the most common childhood benign tumors, showing distinctive progression characteristics and outcomes. Due to the high demand for aesthetics among parents of IH babies, early intervention is critical in some cases. β-Adrenergic blockers and corticosteroids are first-line medications for IHs, while itraconazole, an antifungal medicine, has shown positive results in recent years.

CASE SUMMARY: In the present study, itraconazole was applied to treat two IH cases. The therapeutic course lasted 80-90 d, during which the visible lesion faded by more than 90%. Moreover, no obvious side effects were reported, and the compliance of the baby and parents was desirable.

CONCLUSION: Although these outcomes further support itraconazole as an effective therapeutic choice for IHs, large-scale clinical and basic studies are still warranted to improve further treatment.

PMID:34754871 | PMC:PMC8554448 | DOI:10.12998/wjcc.v9.i28.8579

Tracheal Capillary Hemangioma Successfully Treated With Combined Bronchoscopic Cryotherapy and Argon-Plasma Coagulation

Fetched: November 11th, 2021, 5:01am GMT by Parinya Ruenwilai

Cureus. 2021 Oct 6;13(10):e18547. doi: 10.7759/cureus.18547. eCollection 2021 Oct.

ABSTRACT

We report a 43-year-old female who presented with recurrent non-massive hemoptysis for four months. Chest radiograph was normal. Thoracic computed tomography (CT) scan revealed a 0.8-centimeter intraluminal polypoid mass abutting the middle part of trachea. A fiber-optic bronchoscopy demonstrated a lobulated reddish mass at dorsolateral aspect of the right side of tracheal wall. Cryotherapy to remove the mass and argon plasma coagulation to stop bleeding at the stump was performed. The histological evaluation conﬁrmed the diagnosis of a tracheal lobular capillary hemangioma. There was no recurrence hemoptysis nor the recurrence of tumor during a one-year follow-up.

PMID:34754691 | PMC:PMC8570915 | DOI:10.7759/cureus.18547

Pure epidural spinal cavernous haemangioma

Fetched: November 11th, 2021, 5:01am GMT by Ragavan Manoharan

Surg Neurol Int. 2021 Oct 19;12:523. doi: 10.25259/SNI_805_2021. eCollection 2021.

ABSTRACT

BACKGROUND: Pure epidural spinal cavernous hemangiomas (SCH) account for only 4% of all spinal epidural lesions. Our literature review identified 61 publications reporting on, a total of 175 cases in the magnetic resonance imaging era. Here, we reviewed those cases, and have added our case of what appeared to be a multifocal SCH.

CASE DESCRIPTION: A 72-year-old male presented with a progressive paraparesis attributed to a T5/T6 dorsolateral extradural mass extending into the right T5/6 foramen. Surgical excision documented the lesion, histologically, was a SCH. A second similar lesion was noted involving the left C7/T1 foramen; as the patient was asymptomatic from this lesion, and no additional biopsy was performed. The patient returned to normal neurological function within 2 months postoperatively.

CONCLUSIONS: Here, a 72-year-old male presented with a pathologically confirmed T5/T6 epidural SCH and a secondary C7/T1 foraminal lesion suspected to represent a secondary focus of an epidural SCH.

PMID:34754573 | PMC:PMC8571415 | DOI:10.25259/SNI_805_2021

Permalink for 'IFN-γ facilitates liver fibrogenesis by CD161+CD4+ T cells through a regenerative IL-23/IL-17 axis in chronic hepatitis B virus infection'

IFN-γ facilitates liver fibrogenesis by CD161+CD4+ T cells through a regenerative IL-23/IL-17 axis in chronic hepatitis B virus infection

Fetched: November 11th, 2021, 5:01am GMT by Jing Li

Clin Transl Immunology. 2021 Nov 2;10(11):e1353. doi: 10.1002/cti2.1353. eCollection 2021.

ABSTRACT

OBJECTIVES: This study aimed to determine the role of CD161⁺CD4⁺ T cells in chronic hepatitis B virus (HBV) infection.

METHODS: A total of 94 patients with chronic hepatitis B (CHB), 73 with liver cirrhosis (LC) and 28 healthy controls were enrolled to determine frequency, cytokine production and chemokine receptor expression of circulating CD161⁺CD4⁺ T cells. Among these, 50 CHB and 34 LC patients were followed up for a period of 52-week entecavir monotherapy to assess the association of CD161⁺CD4⁺ T cells with seroconversion of HBV e antigen (HBeAg). In addition, 15 patients with hepatocellular carcinoma (HCC) and 15 with hepatic haemangioma (HHA) were enrolled to compare the paired circulating and intrahepatic CD161⁺CD4⁺ T cells.

RESULTS: CD161⁺CD4⁺ T cells were found to accumulate in the circulation of HBV cohorts, which showed a significant correlation with the clinical parameters of disease progression. In addition, higher numbers of circulating CD161⁺CD4⁺ T cells were associated with an improved serological response of HBeAg to antiviral treatment. Moreover, CD161⁺CD4⁺ T cells as compared to homologous CD161^-CD4⁺ T cells produced more pro-inflammatory cytokines including interleukin (IL)-17 and interferon (IFN)-γ and expressed higher levels of liver-homing chemokine receptors including CCR6, CXCR6 and CX3CR1. Notably, a significant enrichment of CD161⁺CD4⁺ T cell subsets co-expressing IFN-γ and IL-17 was observed in HBV-associated cirrhotic livers. During in vitro co-cultures, circulating CD161⁺CD4⁺ T cells in the chronic HBV setting exhibited prominent pro-fibrogenic effects by regulating primary hepatic stellate cells through a regenerative IFN-γ/IL-23/IL-17 axis.

CONCLUSIONS: In chronic HBV infection, CD161⁺CD4⁺ T cells play antiviral, pro-inflammatory and pro-fibrogenic roles.

PMID:34754450 | PMC:PMC8563156 | DOI:10.1002/cti2.1353

Permalink for 'Timing and Efficacy of 595-nm Pulsed-Dye Laser Combined with 0.5% Timolol Maleate Solution in the Treatment of Superficial Infantile Hemangiomas'

Timing and Efficacy of 595-nm Pulsed-Dye Laser Combined with 0.5% Timolol Maleate Solution in the Treatment of Superficial Infantile Hemangiomas

Fetched: November 11th, 2021, 5:01am GMT by Weikang Shi

Clin Cosmet Investig Dermatol. 2021 Nov 2;14:1593-1599. doi: 10.2147/CCID.S337850. eCollection 2021.

ABSTRACT

PURPOSE: Infantile hemangioma (IH) is the most common benign tumor in infancy, and superficial IH is the most common type. IH can reportedly resolve spontaneously, but this is associated with complications, such as scars, atrophy, hypopigmentation, telangiectasia, and skin sagging, in 70% of cases. This study explores the safety and feasibility of therapeutic intervention with the 595-nm pulsed-dye laser (PDL) combined with 0.5% timolol maleate solution in superficial IH and compares the difference in efficacy between the early group and the late group.

PATIENTS AND METHODS: This retrospective study examined 167 patients with superficial IH who underwent combination therapy at the Dermatology Clinic of the Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University between July 2019 and July 2020. The early and late groups were composed of children aged ≤2 and >2 months, respectively. Treatment was administered for 6 months, and patients were followed up for another 6 months. Two independent, double-blinded physicians reviewed photographs of the skin lesions before and after treatment to evaluate efficacy.

RESULTS: The early group demonstrated higher treatment efficacy than the late group. The early (n = 45) and late (n = 122) groups had treatment efficacy rates of 95.5% and 86.1%, respectively; the difference was statistically significant (P< 0.05). The early and late groups underwent 3.51 ± 0.50 and 4.73 ± 0.68 months of treatment, respectively; the difference was statistically significant (P< 0.05). Seventeen (44.4%) patients in the early group had immediate adverse reactions but no permanent sequelae, whereas 25 (20.49%) and 13 (10.7%) patients in the late group had immediate and permanent sequelae, respectively. The difference was statistically significant (P<0.05).

CONCLUSION: This retrospective study demonstrated that 595-nm PDL combined with 0.5% timolol maleate solution was a safe and effective local treatment for superficial IH. Early treatment required fewer treatments, had better curative effects, and a lower probability of permanent sequelae.

PMID:34754209 | PMC:PMC8572025 | DOI:10.2147/CCID.S337850

En-bloc Resection of A Giant Solid Hemangioblastoma of The Vermis

Fetched: November 11th, 2021, 5:01am GMT by Revanth Goda

Neurol India. 2021 Sep-Oct;69(5):1200-1203. doi: 10.4103/0028-3886.329537.

ABSTRACT

INTRODUCTION: Hemangioblastomas (HMB) are extensively vascularized and benign neoplasms that are diagnosed predominantly in adults. The solid type of HMB is technically challenging to operate. The piecemeal resection of the tumor has been shown to have devastating intraoperative complications. Therefore, en-bloc tumor resection was shown to be the safest procedure to reduce the risk of intraoperative bleeding and facilitate the removal of large solid tumors. Unfortunately, most of these tumours are also not amenable for embolization, as they are fed by multiple pial vessels. However, the large arterial feeders may be embolized.

OBJECTIVE: This video abstract presents a case of en-bloc resection of a giant solid type of Vermian HMB.

SURGICAL TECHNIQUE: A 38-year-old male patient presented with headache and vomiting for 1 year, imbalance during walking for 6 months, and hoarseness of voice for 2 months. He underwent a CSF diversion procedure in another hospital and was referred to our center for definitive surgery. Contrast-enhanced MRI showed a large heterogeneously enhancing solid lesion of size 46 × 33 × 40 mm³ with central necrotic area in the posterior fossa with perilesional edema and several large flow voids on T2-MR sequence. Preoperative embolization was performed; however, there was no significant reduction in the vascularity of the lesion. The patient underwent a wide midline suboccipital craniotomy with C1 laminectomy and gross total en-bloc resection of HMB.

RESULTS: The patient had an uneventful recovery in the postoperative period.

CONCLUSIONS: The safest approach in the resection of giant solid HMB involves proper preoperative planning and understanding the vascular pattern of the lesion, wide exposure, circumferential dissection, and the en-bloc delivery of the tumor.

PMID:34747784 | DOI:10.4103/0028-3886.329537

Propranolol response in patients with segmental vs. focal facial hemangiomas. A retrospe ctive case-control study

Fetched: November 10th, 2021, 5:02am GMT by Florian Schmid

J Am Acad Dermatol. 2021 Nov 6:S0190-9622(21)02744-4. doi: 10.1016/j.jaad.2021.10.054. Online ahead of print.

NO ABSTRACT

PMID:34752824 | DOI:10.1016/j.jaad.2021.10.054

Complex treatment of children with vascular head and neck malformations

Fetched: November 10th, 2021, 5:02am GMT by E Y Gavelya

Stomatologiia (Mosk). 2021;100(5):30-37. doi: 10.17116/stomat202110005130.

ABSTRACT

AIM: The article analyzes the results of treatment of children affected with lesions of blood vessels of the head and neck. The research is aimed at developing and implementing minimally invasive techniques to treat such children.

MATERIAL AND METHODS: The study group comprised 4416 patients with hyperplasia of blood vessels (the so-called infantile and congenital hemangiomas) and 397 patients with blood vessel malformations, examined and treated from 1991 to 2020.

RESULTS: The paper summarizes developed indications and the results of implementation of effective minimally invasive methods for the treatment of children with vascular lesions: radiofrequency thermal ablation and pulsed dye laser treatment of children with severe forms of arterio-venous malformation in their maxillofacial area and. The treatment is currently the golden standard that provides stable aesthetic results.

CONCLUSION: The most optimal and preferred methods of vascular lesions treatment proved to be minimally invasive techniques that include: radiofrequency thermal ablation, interstitial laser coagulation, pulse phototherapy, sclerotherapy and systemic treatment of beta-blockers.

PMID:34752031 | DOI:10.17116/stomat202110005130

Permalink for 'The FcεRI signaling pathway is involved in the pathogenesis of lacrimal gland benign lymphoepithelial lesions as shown by transcriptomic analysis'

The FcεRI signaling pathway is involved in the pathogenesis of lacrimal gland benign lymphoepithelial lesions as shown by transcriptomic analysis

Fetched: November 10th, 2021, 5:02am GMT by Jing Li

Sci Rep. 2021 Nov 8;11(1):21853. doi: 10.1038/s41598-021-01395-z.

ABSTRACT

This study aimed to analyze the role of the FcepsilonRI (FcεRI) signaling pathway in the pathogenesis of benign lymphoepithelial lesion of lacrimal gland (LGBLEL). Transcriptomic analysis was performed on LGBLEL and orbital cavernous hemangioma (CH) patients diagnosed via histopathology in Beijing Tongren Hospital, Capital Medical University, between July 2010 and October 2013. Four LGBLEL and three orbital CH patients, diagnosed between October 2018 and August 2019, were randomly selected as experimental and control groups, respectively. RT-PCR, immunohistochemical staining, and western blotting were used to verify genes and proteins related to the FcεRI signaling pathway. Transcriptomic analysis showed that the FcεRI signaling pathway was upregulated in the LGBLEL compared with the CH group. The mRNA expression levels of important genes including SYK, p38, JNK, PI3K, and ERK were significantly increased in the LGBLEL group (P = 0.0066, P = 0.0002, P = 0.0003, P < 0.0001, P < 0.0001, respectively). Immunohistochemical staining results showed that SYK, p38, and ERK were positively expressed in LGBLEL, while JNK and PI3K were not. The protein contents of P-SYK, P-p38, P-JNK, P-PI3K, and P-ERK were significantly higher in the LGBLEL than in the CH group (P = 0.0169, P = 0.0074, P = 0.0046, P = 0.0157, P = 0.0156, respectively). The FcεRI signaling pathway participates in the pathogenesis of LGBLEL.

PMID:34750466 | PMC:PMC8576038 | DOI:10.1038/s41598-021-01395-z

Verrucous venous malformation with thrombocytopenia in a neonate

Fetched: November 10th, 2021, 5:02am GMT by Sophia C Reyes-Hadsall

Pediatr Dermatol. 2021 Nov 8. doi: 10.1111/pde.14857. Online ahead of print.

ABSTRACT

Verrucous venous malformations (VVM) are rare, congenital, slow-flow vascular anomalies that have been historically difficult to characterize due to clinical mimics and unclear histological evaluation. A life-threatening complication of VVMs is localized intravascular coagulation. Herein, we describe a male neonate who presented with a congenital VVM on the left lower extremity with associated severe thrombocytopenia. We discuss the multifaceted diagnostic approach used to identify this VVM, while highlighting the use of WT-1 as a negative predictive marker; we additionally outline novel treatment options and management beyond cutaneous involvement.

PMID:34749432 | DOI:10.1111/pde.14857

Giant Frontotemporal Cavernous Malformation in a 2-Month-Old Infant: A Case Report and Review of the Literature

Fetched: November 10th, 2021, 5:02am GMT by Olivia E Gilbert

Pediatr Neurosurg. 2021 Nov 8:1-7. doi: 10.1159/000519856. Online ahead of print.

ABSTRACT

INTRODUCTION: Cavernous malformations in the pediatric population are exceedingly rare, especially in infants. Giant cavernous malformations (GCM) are even more rare and have a diameter >4 cm. The onset of symptoms predominantly occurs in adulthood, but the rate of hemorrhage is significantly higher in the pediatric population. Similar to non-GCM, GCM can be misidentified as tumors on imaging due to their tumefactive pattern with edema. Here, we present a rare case of a right frontotemporal GCM in a 2-month-old girl, the youngest recorded case in the existing literature.

CASE PRESENTATION: A previously healthy 2-month-old girl presented to the emergency department following an increasing frequency of seizure-like activity that began 3 days prior to presentation. Magnetic resonance imaging of the brain with and without contrast characterized a large (5.8 × 4.3 × 4.2 cm) heterogeneous lesion of the right temporal lobe with diffuse scattered blood products of various ages seen throughout the lesion. She underwent a right-sided craniotomy where a gross total excision was achieved. Pathology confirmed the diagnoses of a GCM. The patient's seizures subsequently resolved, and she continues to do well postoperatively.

DISCUSSION/CONCLUSION: GCM can be mistaken for tumors due to their large size, cystic nature, and surrounding edema, but a vascular lesion should always remain in the differential diagnosis before operating, even in infants. Surgery is generally recommended in patients that present with a symptomatic hemorrhage, recurrent hemorrhages, persistent seizures despite medical management, or progressively worsening neurological deficits if the GCM is in a safe location. It has been shown that 70-99% of patients undergoing surgery with successful removal of the GCM can achieve seizure freedom 2 years postoperatively. Complete surgical excision of this infant's GCM was successful in treating her neurologic symptoms; therefore, pathological confirmation of this lesion is critical and should prompt a complete surgical excision.

PMID:34749375 | DOI:10.1159/000519856

Noninferiority and Safety of Nadolol vs Propranolol in Infants With Infantile Hemangioma: A Randomized Clinical Trial

Fetched: November 10th, 2021, 5:02am GMT by Elena Pope

JAMA Pediatr. 2021 Nov 8:e214565. doi: 10.1001/jamapediatrics.2021.4565. Online ahead of print.

ABSTRACT

IMPORTANCE: Propranolol for infantile hemangiomas (IH) has been shown to be effective and relatively safe. However, other less lipophilic β-blockers, such as nadolol, may be preferable in individuals who experience propranolol unresponsiveness or adverse events.

OBJECTIVE: To document the noninferiority and safety of oral nadolol compared with oral propranolol in infants with IH.

DESIGN, SETTING, AND PARTICIPANTS: This double-blind noninferiority prospective study with a noninferiority margin of 10% compared propranolol with nadolol in infants aged 1 to 6 months with problematic IH. The study was conducted in 2 academic pediatric dermatology centers in Canada between 2016 and 2020. Infants aged 1 to 6 months with a hemangioma greater than 1.5 cm on the face or 3 cm or greater on another body part causing or with potential to cause functional impairment or cosmetic disfigurement.

INTERVENTIONS: Oral propranolol and nadolol in escalating doses up to 2 mg/kg/d.

MAIN OUTCOMES AND MEASURE: Between-group differences comparing changes in the bulk (size and extent) and color of the IH at week 24 with baseline using a 100-mm visual analog scale.

RESULTS: The study included 71 patients. Of these, 36 were treated with propranolol. The mean (SD) age in this group was 3.1 (1.4) months, and 31 individuals (86%) were female. Thirty-five infants were treated with nadolol. The mean (SD) age in this group was 3.2 (1.6) months, and 26 individuals (74%) were female. The difference in IH between groups by t test was 8.8 (95% CI, 2.7-14.9) for size and 17.1 (95% CI, 7.2-30.0) for color in favor of the nadolol group, demonstrating that nadolol was noninferior to propranolol. Similar differences were noted at 52 weeks: 6.0 (95% CI, 1.9-10.1) and 10.1 (95% CI, 2.9-17.4) for size and color improvement, respectively. For each doubling of time unit (week), the coefficient of involution was 2.4 (95% CI, 0.5-4.4) higher with nadolol compared with propranolol. Safety data were similar between the 2 interventions.

CONCLUSIONS AND RELEVANCE: Oral nadolol was noninferior to oral propranolol, indicating it may be an efficacious and safe alternative in cases of propranolol unresponsiveness or adverse events, or when faster involution is required.

TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02505971.

PMID:34747977 | PMC:PMC8576629 | DOI:10.1001/jamapediatrics.2021.4565

Case Report: Cutaneous Pleomorphic Lymphangiosarcoma in a Dog Exhibiting Features of Human Composite Hemangioendothelioma

Fetched: November 10th, 2021, 5:02am GMT by Matthew R Cook

Front Vet Sci. 2021 Oct 22;8:666226. doi: 10.3389/fvets.2021.666226. eCollection 2021.

ABSTRACT

Background: Angiosarcomas are a broad category of vascular origin neoplasms that are poorly characterized in veterinary species. Lymphangiosarcoma (LAS) is an uncommon type of angiosarcoma reported in humans and canines arising from lymphatic endothelium. LAS can be differentiated from other angiosarcomas in dogs based on expression of Prospero-related homeobox gene-1 (PROX-1) or lymphatic vessel endothelial receptor-1 (LYVE-1). Composite hemangioendothelioma (CHE) is a rare angiosarcoma subtype described in people and characterized by a variable biologic behavior and infrequent metastasis. This variant of angiosarcoma histologically combines features of retiform hemangioendothelioma and epithelioid hemangioendothelioma. Information regarding the cytologic and histopathologic appearance and clinical course of dogs with vascular tumors that exhibit features of CHE are unknown. Here, we report a case of pleomorphic LAS with features of CHE arising in a dog and treated with surgery and adjuvant chemotherapy. Case presentation: A 10-year-old intact male Labrador retriever presented with an approximately 6-cm-diameter cutaneous mass caudal to the left elbow that was progressively growing over 1.5 years. On physical examination, palpable extensions were identified coursing proximally over the triceps with concurrent loco-regional peripheral lymphadenopathy. Fine needle aspirates (FNA) and cytologic assessment of the cutaneous mass, left prescapular, and accessory axillary lymph nodes reported that this appeared to be a metastatic epithelial neoplasm, although a mixed carcinoma or collision tumor could not be excluded. An incisional biopsy of the mass was submitted for histopathology and was consistent with a well-differentiated angiosarcoma with features of CHE. The neoplasm expressed vimentin, CD31, von Willebrand factor (vWf), and PROX-1, supporting the diagnosis of LAS. Complete staging was performed, and no additional metastatic lesions were identified. Left forelimb amputation and lymph node removal were performed. Based on the diagnosis of metastatic LAS, doxorubicin chemotherapy was administered. 7 months post-amputation, the tumor recurred at the amputation site without evidence of metastatic disease. Conclusion: This report describes a malignant, locally aggressive lymphatic origin vascular tumor in a dog, with features consistent with descriptions of CHE in humans. Cytologic features in this case were discordant with its true mesenchymal etiology, obfuscating diagnosis. The morphologic features of the mesenchymal neoplastic population and immunohistochemistry (IHC) labeling ultimately supported a diagnosis of pleomorphic LAS with features of CHE.

PMID:34746271 | PMC:PMC8569467 | DOI:10.3389/fvets.2021.666226

Navigation-guided nasal endoscopy for removal of the cavernous hemangioma of the orbital apex through the sphenoid approach

Fetched: November 10th, 2021, 5:02am GMT by Y H Wang

Zhonghua Yan Ke Za Zhi. 2021 Nov 11;57(11):837-843. doi: 10.3760/cma.j.cn112142-20210207-00081.

ABSTRACT

Objective: To explore the feasibility of navigation-guided nasal endoscopy for removal of the cavernous hemangioma of the orbital apex through the sphenoid approach. Methods: Retrospective case series study. From May 2012 to December 2019, 12 patients (12 eyes) with imaging findings of cavernous hemangioma in the orbital apex were collected at the Eye Hospital Affiliated to Nanchang University, including 3 males and 9 females aged 32 to 59 years. All patients underwent navigation-guided sinusoscopy through the sphenoid approach to remove the cavernous hemangioma of the orbital apex (video attached). Changes of visual function and complications after operation were analyzed. Results: In 3 patients without visual impairment, the postoperative visual function was still normal. Among the remaining 9 patients with preoperative visual impairment, visual function was fully recovered in 3 patients after operation, was improved in 2 patients, and had no change in 4 patients. There were no complications in 3 of the 12 patients, and 9 patients had transient, mildly limited intraocular rotation with diplopia after operation, which all returned to normal within 1 month. Conclusion: Navigation-guided sinus endoscopy through the sphenoid approach is effective and feasible in the removal of the cavernous hemangioma of the orbital apex. (Chin J Ophthalmol, 2021, 57: 837-843).

PMID:34743469 | DOI:10.3760/cma.j.cn112142-20210207-00081

Vascular Tumors of Bone: Updates and Diagnostic Pitfall

Fetched: November 10th, 2021, 5:02am GMT by Yin P Hung

Surg Pathol Clin. 2021 Dec;14(4):645-663. doi: 10.1016/j.path.2021.06.007. Epub 2021 Oct 7.

ABSTRACT

Vascular tumors of bone can be diagnostically challenging because of their rarity and histologic overlap with diverse mimics. Vascular tumors of bone can be categorized as benign (hemangioma), intermediate-locally aggressive (epithelioid hemangioma), intermediate-rarely metastasizing (pseudomyogenic hemangioendothelioma), and malignant (epithelioid hemangioendothelioma and angiosarcoma). Recurrent genetic alterations have been described, such as FOSB rearrangements in pseudomyogenic hemangioendothelioma and a subset of epithelioid hemangiomas; CAMTA1 or TFE3 rearrangements in epithelioid hemangioendothelioma. This review discusses the clinical, histologic, and molecular features of vascular tumors of bone, along with diagnostic pitfalls and strategies for avoidance.

PMID:34742485 | DOI:10.1016/j.path.2021.06.007

Giant cell epulis

Fetched: November 5th, 2021, 5:01am GMT by Georgi Tchernev

Wien Med Wochenschr. 2021 Nov 4. doi: 10.1007/s10354-021-00894-y. Online ahead of print.

ABSTRACT

Giant cell epulis (peripheral giant cell granuloma) typically appears as a reactive benign lesion in the oral cavity in areas following local irritation or chronic trauma. Here we describe the case of a 45-year-old male patient who presented with the chief complaint of a large gingival mass in the anterolateral maxilla. There had been progressive growth within the past few months, with increased painless discomfort during mastication. The patient also reported bleeding during interdental cleaning. A full physical work-up led to the suspicion of giant cell epulis alongside other differentials including mucosal hemangioma and squamous cell carcinoma, with unremarkable laboratory values. Imaging including computed tomography showed signs of previous insertion of metal implants on either side of the lesion alongside mucosal hyperplasia. A confirmatory biopsy was taken and showed multiple giant cells on a reactive bed of stroma, in line with the diagnosis of giant cell epulis. Oral inflammatory conditions such as giant cell epulis have greater chances of local recurrence and, therefore, careful investigation with timely and accurate diagnosis is imperative for appropriate early treatment. Complete surgical excision should then be employed to prevent relapses, as incomplete removal can lead to further recurrence. Identification and eradication of potential sources of irritation should also be considered when treating the patient, to avoid further recurrence.

PMID:34735668 | DOI:10.1007/s10354-021-00894-y

Primary epithelioid hemangioendothelioma of the penis: a case report and literature review

Fetched: November 5th, 2021, 5:01am GMT by Alberto Artiles Medina

Transl Androl Urol. 2021 Sep;10(9):3697-3703. doi: 10.21037/tau-21-277.

ABSTRACT

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour with an intermediate behaviour between benign hemangioma and malignant angiosarcoma. There is scarce data on the penile EHE's management and its natural history, as our knowledge is based on few reported cases with a short follow-up period. We present a case report and conducted a literature review, including 17 cases. The relevance of this case report derives from the need for better clinical characterization of patients with penile EHE and the importance of defining the outcomes. We report the case of a 53-year-old male with a 1-year history of sleep-related painful erections. Imaging techniques showed a well-defined hypoechoic and hypervascular solid nodule on the dorsal aspect of the penis. It was surgically removed, and the histopathological study revealed a low-risk EHE of the penis. Follow-up magnetic resonance imaging (MRI) and computed tomography did not demonstrate local recurrence nor metastases. According to the literature review, most of the patients were in their fifth and sixth decades of life at the time of diagnosis and lesions were usually located in the glans. The most common clinical presentation was as a painful mass. Follow-up period ranged from 2 months to 5 years. Three patients showed systemic metastases, two of which died due to cancer. The conclusions from the literature review are limited by the reduced number of cases and the short follow-up. This case report highlights the importance of understanding the diagnosis and treatment of this type of rare non-squamous malignant tumours of the penis. Penile EHE is a malignant vascular tumour that is very rare in this location. The best treatment is local excision, with re-excision or intraoperative margins assessment. Occasionally, systemic chemotherapy and radiation therapy can be useful. There is consensus on the importance of very strict follow-up of these patients.

PMID:34733664 | PMC:PMC8511538 | DOI:10.21037/tau-21-277

Management of adult laryngeal hemangioma with CO2 laser

Fetched: November 5th, 2021, 5:01am GMT by Hamoud Alshaya

Saudi Med J. 2021 Nov;42(11):1252-1253. doi: 10.15537/smj.2021.42.11.20210319.

NO ABSTRACT

PMID:34732560 | DOI:10.15537/smj.2021.42.11.20210319

Brain Metastasis in Soft Tissue Sarcoma at Initial Presentation

Fetched: November 5th, 2021, 5:01am GMT by Ryo Itoga

Anticancer Res. 2021 Nov;41(11):5611-5616. doi: 10.21873/anticanres.15376.

ABSTRACT

BACKGROUND/AIM: Brain metastasis is a rare condition among patients with soft tissue sarcoma (STS), and its precise incidence remains unclear. The aim of this study was to investigate which patients should be screened for brain metastasis.

PATIENTS AND METHODS: We identified all patients with STS diagnosed between 2010 and 2015 in the SEER database. Incidence of brain metastasis at initial presentation and higher incidence of brain metastasis by histological subtype were investigated. In addition, risk factors for brain metastasis were examined.

RESULTS: A total of 26,676 patients were included for analysis, of whom 162 patients (0.6%) had brain metastasis. Alveolar soft part sarcoma (6.3%), malignant hemangioendothelioma (3.1%) and malignant schwannoma (2.6%) showed higher incidence of brain metastasis. Deep-rooted tumor, trunk tumor, and histological high-grade tumor tended to show higher incidence of brain metastasis.

CONCLUSION: Risk factors for brain metastasis were deep location, trunk development and histologically high-grade tumor, or specific histological subtypes.

PMID:34732433 | DOI:10.21873/anticanres.15376

Corrigendum to "A hemangioma in the masseter muscle: a case report"

Fetched: November 5th, 2021, 5:01am GMT by Daehwan Park

Arch Craniofac Surg. 2021 Oct;22(5):285. doi: 10.7181/acfs.2021.00283.e1. Epub 2021 Oct 20.

NO ABSTRACT

PMID:34732042 | PMC:PMC8568492 | DOI:10.7181/acfs.2021.00283.e1

Cervical vertebral hemangioma with aggressive behavior

Fetched: November 4th, 2021, 5:01am GMT by J M Jiménez-Ávila

Acta Ortop Mex. 2021 Mar-Apr;35(2):206-210.

ABSTRACT

INTRODUCTION: Hemangiomas are the most common primary tumors of the spine. Mainly asymptomatic Incidental finding when performing an imaging study. The incidence reported at autopsies is 11% in the spine, multifocal lesions are present in 25 to 30% of cases.

CLINICAL CASE: Vertebral cervical hemangioma with benign and stable characteristics that underwent surgical treatment, observing aggressive post-surgical behavior two months later.

CONCLUSION: The approach to vertebral pathology is algorithmic and protocolized, it is necessary to determine the treatment based on the overall understanding of the disease and according to the clinical practice guidelines.

PMID:34731925

HNF1A-AS1 inhibits proliferation, migration and invasion of IL-6-induced hemangioma endothelial cells by targeting miR-363-3p

Fetched: November 4th, 2021, 5:01am GMT by Wenhao Xu

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2021 Nov 10;38(11):1091-1096. doi: 10.3760/cma.j.cn511374-20200830-00633.

ABSTRACT

OBJECTIVE: To explore the effect of HNF1A-AS1 on the proliferation, migration and invasion of IL-6-induced hemangioendothelial cells (HemEC) and possible mechanism.

METHODS: RT-qPCR was used to detect the expression level of HNF1A-AS1 and miR-363-3p in the tumor tissue and adjacent normal skin tissue from 35 patients with hemangioma. Pearson correlation was used to analyze the correlation between the expression of HNF1A-AS1 and miR-363-3p in tumor tissues. HemEC were isolated and cultured in vitro.Dual luciferase reporter gene experiment was used to study the regulatory effect between HNF1A-AS1 and miR-363-3p. IL-6 was added to HemEC transfected with si-NC, si-HNF1A-AS1, si-HNF1A-AS1 and anti-miR-NC, or si-HNF1A-AS1 and anti-miR-363-3p, respectively. CCK-8 method and clone formation experiment were used to detect cell proliferation in each group. Transwell method was used to detect cell migration and invasion in each group. Western blotting was used to detect the expression of Ki67, MMP-2 and MMP-9 proteins in each group.

RESULTS: Compared with normal skin tissues, the expression of IL-6 mRNA in hemangioma tissues was increased (P<0.05), and the expression of IL-6 mRNA in the proliferative phase was lower than that in the degenerative phase (P<0.05). Expression of HNF1A-AS1 in hemangioma tissue was increased (P<0.05), while that of miR-363-3p was decreased (P<0.05), and the two were negatively correlated (r=-0.758, P<0.05). HNF1A-AS1 down-regulated the expression of miR-363-3p in HemEC.IL-6 promoted the expression of HNF1A-AS1, OD value, number of colonies, number of migration and invasion of HemEC cells, and the expression of Ki67, MMP-2 and MMP-9proteins (P<0.05), while reduced the expression of miR-363-3p (P<0.05). Down-regulating si-HNF1A-AS1 reduced the IL-6-induced HemEC cell OD value, colony numbers, migration and invasion and the expression of Ki67, MMP-2 and MMP-9 proteins (P<0.05). Down-regulating miR-363-3p attenuated the inhibitory effect of down-regulating si-HNF1A-AS1 on the proliferation, migration and invasion of HemEC cells induced by IL-6 (P<0.05).

CONCLUSION: Expression of HNF1A-AS1 is increased in hemangioma tissues. Down-regulating HNF1A-AS1 may inhibit proliferation, migration and invasion of IL-6-induced hemangioma endothelial cells by targeted up-regulation of miR-363-3p.

PMID:34729750 | DOI:10.3760/cma.j.cn511374-20200830-00633

Haematemesis in an infant: a rare tumour in a rare site

Fetched: November 4th, 2021, 5:01am GMT by Jiangyuan Zhou

Gastroenterology. 2021 Oct 30:S0016-5085(21)03705-7. doi: 10.1053/j.gastro.2021.10.033. Online ahead of print.

NO ABSTRACT

PMID:34728184 | DOI:10.1053/j.gastro.2021.10.033

Permalink for 'Oral reactive capillary hemangiomas induced by SHR-1210 in the treatment of non-small cell lung cancer: a case report and literature review'

Oral reactive capillary hemangiomas induced by SHR-1210 in the treatment of non-small cell lung cancer: a case report and literature review

Fetched: November 4th, 2021, 5:01am GMT by Jinhan Zhou

BMC Oral Health. 2021 Nov 2;21(1):559. doi: 10.1186/s12903-021-01901-9.

ABSTRACT

BACKGROUND: Antibodies to PD-1 and PD-L1 have remarkably improved the overall survival of many patients with advanced solid tumors. SHR-1210 is an anti-PD-1 monoclonal antibody. Dermatologic reactive capillary hemangiomas (RCH) were the most common and unique drug-related AEs of SHR-1210, but rare on oral mucosa and gastrointestinal mucosa. Herein we report a case of RCH occurred in oral mucosa during the clinical trials of SHR-1210 in the treatment of non-small cell lung cancer.

CASE PRESENTATION: A male in his 60 s with a history of non-small cell lung cancer received injection of anti-PD-1 monoclonal antibodies SHR-1210. The patient developed drug-related RCH on skin after the first injection and began to have gingival hyperplasia one year after the first injection which gradually increased in size and affect eating and speaking. Anti-PD-1 treatments were continued. After periodontal treatment, two oral lesions and one skin lesion were surgically removed. Similar histological manifestation was found in all three lesions as reactive capillary hemangiomas. All lesions had a good prognosis without recurrence on oral mucosa within one year after surgery.

CONCLUSIONS: Oral reactive capillary hemangiomas could be induced by SHR-1210 in the treatment of non-small cell lung cancer. Surgical resection is an effective treatment with a good prognosis.

PMID:34727912 | PMC:PMC8561900 | DOI:10.1186/s12903-021-01901-9

Primary sternal haemangioma

Fetched: November 3rd, 2021, 5:01am GMT by Veysel Ayyildiz

Br J Hosp Med (Lond). 2021 Oct 2;82(10):1. doi: 10.12968/hmed.2021.0162. Epub 2021 Oct 14.

NO ABSTRACT

PMID:34726942 | DOI:10.12968/hmed.2021.0162

Cutaneous epithelioid hemangiomas show somatic mutations in the MAPK pathway

Fetched: November 3rd, 2021, 5:01am GMT by K Maurus

Br J Dermatol. 2021 Nov 2. doi: 10.1111/bjd.20869. Online ahead of print.

ABSTRACT

BACKGROUND: Epithelioid hemangioma (EH) arising from the skin is a benign vascular tumor with marked inflammatory cell infiltration, which exhibits a high tendency to persist and frequently recurs after resection. So far, the underlying pathogenesis is largely elusive.

OBJECTIVES: To identify genetic alterations by next-generation-sequencing and/or droplet digital PCR (ddPCR) in cutaneous EH.

METHODS: DNA and RNA from an EH lesion of an index patient were subjected to whole genome and RNA sequencing. Multiplex PCR-based panel sequencing of genomic DNA isolated from archival formalin-fixed paraffin-embedded (FFPE) tissue of 18 cutaneous EH patients was performed. ddPCR was used to confirm mutations.

RESULTS: We identified somatic mutations in genes of the MAPK pathway (MAP2K1 and KRAS) in cutaneous EH biopsies. By ddPCR we could confirm the recurrent presence of activating, low-frequency mutations affecting MAP2K1. In total, 9 out of 18 analyzed patients showed activating MAPK pathway mutations, which were mutually exclusive. Comparative analysis of tissue areas enriched for lymphatic infiltrate or aberrant endothelial cells, respectively, revealed an association of these mutations with the presence of endothelial cells.

CONCLUSIONS: Taken together, our data suggest that EH shows somatic mutations in genes of the MAPK pathway which might contribute to the formation of this benign tumor.

PMID:34726260 | DOI:10.1111/bjd.20869

Contralateral Interhemispheric Transcallosal Approach for Thalamic Cystic Cavernous Malformation

Fetched: November 3rd, 2021, 5:01am GMT by Tak Gun Jang

Brain Tumor Res Treat. 2021 Oct;9(2):87-92. doi: 10.14791/btrt.2021.9.e15.

ABSTRACT

A 42-year-old man presented with a headache and right hemiparesis and was found to have a cystic mass with a calcified mural nodule in the left thalamus. Because the thalamus is surrounded by vital neurovascular structures, the surgical approach to thalamic lesions can be challenging. We decided to remove the mass for decompression and pathological diagnosis. The mass was removed through a contralateral interhemispheric transcallosal transchoroidal approach with less retraction and parenchymal injury than other approaches to avoid brain retraction and cortical injury. The pathological diagnosis was cavernous malformation. Temporary worsening of the preoperative hemiparesis was recovered over two months following surgery. Tolerable thalamic pain syndrome remained. Here, we report a rare case of thalamic cavernous malformation with a favorable outcome through a contralateral surgical approach.

PMID:34725990 | PMC:PMC8561222 | DOI:10.14791/btrt.2021.9.e15

The sternum: An important review area when reporting pre-surgical staging breast MRI studies

Fetched: November 3rd, 2021, 5:01am GMT by Jeremy Price

J Med Imaging Radiat Oncol. 2021 Nov 1. doi: 10.1111/1754-9485.13344. Online ahead of print.

ABSTRACT

Identification and assessment of extramammary findings on contrast-enhanced breast MRI scans is particularly important in the setting of newly diagnosed invasive cancer as metastatic lesions may be encountered in the liver, lungs, pleural cavity or bones. Establishing that stage IV disease is present has a profound effect on patient management. The sternum is routinely included on breast MRI studies and can be an early site for breast cancer metastases. These appear as enhancing lesions with high signal on fat-suppressed T2-weighted images. However, incidental benign lesions, notably haemangiomas, may also be encountered, and careful analysis is required to avoid false-positive results. Clinical context is important with a much lower likelihood of malignancy in the setting of routine screening of young women with no personal history of breast cancer. This pictorial essay illustrates findings encountered with lesions in the sternum and offers insights into how to interpret and manage them.

PMID:34725929 | DOI:10.1111/1754-9485.13344

Portal hypertension as an uncommon presentation of hepatic epithelioid hemangioendothelioma: a case report

Fetched: November 2nd, 2021, 5:01am GMT by Rommel Zambrano-Huailla

Rev Gastroenterol Peru. 2021 Apr-Jun;41(2):103-106.

ABSTRACT

Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.

PMID:34724691

Contrast-Enhanced Ultrasound as a Main Radiological Diagnostic Method for Primary Liver Neoplasms and Hemangiomas

Fetched: November 2nd, 2021, 5:01am GMT by Knkush Hakobyan

Cureus. 2021 Sep 25;13(9):e18288. doi: 10.7759/cureus.18288. eCollection 2021 Sep.

ABSTRACT

Contrast-enhanced ultrasound (CEUS) is a relatively new approach for the definitive diagnosis of focal liver lesions (FLL). The essential advantages of CEUS are affordability, absence of radiation, and negligible nephrotoxicity-making this diagnostic approach more preferable. This review includes data from 39 different research studies published during the last 10 years, selected through the MeSH strategy in PubMed. We conclude that CEUS is a promising approach for diagnosing primary liver neoplasms and it is an excellent radiological approach for children and pregnant women because of the absence of radiation and nephrotoxicity. Studies showed that CEUS is a very good approach for the differentiation of a variety of hemangiomas and for a detailed description of those findings. Therefore, CEUS is an important and progressive method for the diagnosis of liver neoplasms. The regular use of CEUS will facilitate the diagnosis of primary liver lesions.

PMID:34722064 | PMC:PMC8547375 | DOI:10.7759/cureus.18288

Maffucci Syndrome with Intrahepatic Cholangiocarcinoma: A Case Report

Fetched: November 2nd, 2021, 5:01am GMT by Ryoichiro Kobayashi

Case Rep Oncol. 2021 Sep 20;14(3):1347-1352. doi: 10.1159/000515779. eCollection 2021 Sep-Dec.

ABSTRACT

Maffucci syndrome is characterized by multiple hemangiomas and enchondromas. Somatic mutations in IDH1 and IDH2 are associated with the development of Maffucci syndrome, and these patients develop various malignant nonskeletal tumors in addition to malignant skeletal tumors. We report a case of Maffucci syndrome with IDH1 mutation complicated by intrahepatic cholangiocarcinoma. The patient was a 35-year-old woman who was diagnosed with Maffucci syndrome in childhood. She was referred to our department because of a large hepatic tumor. Serum carcinoembryonic antigen was 27.1 ng/mL upon laboratory examination. CT scanning showed a large low-density tumor (90 × 70 mm) in the right lobe of the liver, and MRI revealed a multilobulated and fibrous tumor, which was observed as high signal intensity on T2- and diffusion-weighted images and low signal intensity on T1-weighted images. Positron emission tomography-CT revealed peritoneal dissemination and cancer spread to the muscles of the back. Finally, she was diagnosed with intrahepatic cholangiocarcinoma with dissemination and metastases. We performed a tumor biopsy to determine a treatment plan for chemotherapy. Sanger sequencing of a tumor biopsy identified a mutation in IDH1 at c.394C>T (R132C), but the patient died of rapid cancer progression before the chemotherapy could be initiated. Although rare, malignant tumors can develop in patients with Maffucci syndrome; therefore, it is necessary to monitor these tumors through careful and periodic observation.

PMID:34720940 | PMC:PMC8525296 | DOI:10.1159/000515779

Efficacy and safety of propranolol gel for infantile hemangioma: A randomized, double-blind study

Fetched: November 2nd, 2021, 5:01am GMT by Naoaki Rikihisa

Biol Pharm Bull. 2021 Oct 30. doi: 10.1248/bpb.b21-00500. Online ahead of print.

ABSTRACT

We aimed to evaluate the efficacy and safety of propranolol gel at various concentrations with infantile hemangiomas after proliferative phases. We designed a single-center, randomized, double-blind, dose-dependent trial with placebo control and randomized patients to receive propranolol gel at 0%, 1%, or 5%, twice daily for 24 weeks. The primary efficacy endpoint was the percentage change in redness of the tumors. Safety endpoints were skin characteristics changes and systemic symptoms. We made two comparisons to evaluate the superiority of 1% and 5% propranolol gels against placebo for primary endpoint analysis and used the t-test to compare parents' satisfaction with these treatments. Initially, 19 patients were enrolled, but 8 were excluded from the analysis. We were underpowered to answer the question of efficacy. In the per-protocol set, we found similar results for the redness percentage change among the patients on placebo, 1% and 5% gel. However, the difference in redness before and after treatment suggested a slight decreasing trend of lesion's redness as the propranolol concentration increased. The difference in parents' satisfaction between the placebo and 5% propranolol gel groups was significant (p = 0.08). We observed no serious adverse events. We did not find an obvious dose-dependent effect for the propranolol gel treatment against infantile hemangiomas after the proliferative phase. However, external applications twice daily were less burdensome for parents and led to good compliances. It had a favorable safety profile in Japanese pediatric patients with infantile hemangiomas.

PMID:34719577 | DOI:10.1248/bpb.b21-00500

Permalink for 'Long-term outcomes of staged Gamma Knife radiosurgery for giant cavernous sinus hemangiomas: a single-center retrospective study'

Long-term outcomes of staged Gamma Knife radiosurgery for giant cavernous sinus hemangiomas: a single-center retrospective study

Fetched: October 31st, 2021, 5:01am GMT by Ruyi Yang

J Neurosurg. 2021 Oct 29:1-7. doi: 10.3171/2021.7.JNS21955. Online ahead of print.

ABSTRACT

OBJECTIVE: Cavernous sinus hemangiomas (CSHs) are rare benign tumors originating from the cavernous sinus. Gamma Knife radiosurgery (GKRS) has been recommended as a primary treatment for small- to medium-sized CSHs. The optimal treatment for giant CSHs is still controversial. In this study, the authors retrospectively reviewed the effectiveness and safety of staged GKRS treatment for giant CSHs.

METHODS: Twenty-two patients with giant CSH who received staged GKRS treatment in the Gamma Knife Treatment Center of Henan Province during the period from January 1, 2011, to December 31, 2018, were enrolled in this study. Six patients had received microsurgery before GKRS, the other 16 patients were diagnosed according to clinical symptoms and MR images. All of the enrolled patients received 2-stage GKRS, and the mean interval between the two GKRS treatments was 6.5 months (range 6-12 months). For the first GKRS, the median isodose line was 48% (range 45%-50%), the median marginal dose was 13 Gy (range 11.5-14 Gy), and the median coverage of CSHs was 80% (range 70%-88%). For the second GKRS treatment, the median isodose line was 50% (range 45%-55%), the median marginal dose to the CSHs was 10.5 Gy (range 9-12.5 Gy), and the median coverage of the CSHs was 88% (range 80%-94%).

RESULTS: All of the patients received an outpatient review of an enhanced MR image of the head and a clinical physical check every 6 months after the first GKRS treatment. The mean follow-up duration was 52 months (range 24-84 months). The tumor control rate was 100% 24 months after staged GKRS, and at the last follow-up the mean tumor shrinkage rate was 96.7% (range 90.6%-100%) and the mean residual CSH volume was 2.1 ml (range 0-8.5 ml). Twenty patients suffered central nervous system (CNS) injury symptoms to varying degrees before staged GKRS treatment. Complete symptom recovery was found in 11 (55%) patients, improved symptoms in 5 (25%) patients, and no change in 4 (20%) patients after treatment. Only 1 patient suffered temporary preexisting headache aggravation and 1 patient suffered temporary preexisting diplopia aggravation 1 week after receiving the first GKRS treatment. Subacute or chronic complications were not detected after staged GKRS.

CONCLUSIONS: Staged GKRS is an effective treatment for giant CSHs. Because of the impressively low incidence of adverse effects, staged GKRS may be considered as a primary treatment for giant CSHs.

PMID:34715663 | DOI:10.3171/2021.7.JNS21955

Permalink for 'Knockdown of lncRNA MEG8 inhibits cell proliferation and invasion, but promotes cell apoptosis in hemangioma, via miR-203-induced mediation of the Notch signaling pathway'

Knockdown of lncRNA MEG8 inhibits cell proliferation and invasion, but promotes cell apoptosis in hemangioma, via miR-203-induced mediation of the Notch signaling pathway

Fetched: October 30th, 2021, 5:01am GMT by Zhenfeng Hu

Mol Med Rep. 2021 Dec;24(6):872. doi: 10.3892/mmr.2021.12512. Epub 2021 Oct 29.

ABSTRACT

As a member of the long non‑coding (lnc)RNA family, lncRNA maternally expressed 8, small nucleolar RNA host gene (MEG8), has been reported to serve an oncogenic role in several types of malignancies, including hepatocellular carcinoma, non‑small cell lung cancer and pancreatic cancer. The current study aimed to investigate the effect of the knockdown of MEG8 on human hemangioma endothelial cell (HemEC) proliferation, apoptosis and invasion, in addition to determining the underlying molecular mechanism. The knockdown of lncRNA MEG8 was achieved by transfecting lncRNA MEG8 small interfering (si)RNA into HemECs, while the combined knockdown of lncRNA MEG8 knockdown and microRNA (miR)‑203 was established by co‑transfecting lncRNA MEG8 siRNA and a miR‑203 inhibitor into HemECs. The cell proliferation, apoptosis and invasion and the expression levels of miR‑34a, miR‑200b, miR‑200b and Notch signaling pathway‑related factors were detected via CCK‑8 Kit, flow cytometry, Transwell, reverse transcription‑quantitative PCR and western blot assay, respectively. The knockdown of lncRNA MEG8 significantly inhibited proliferation (P<0.05) and invasion (P<0.05), but promoted apoptosis (P<0.01) in HemECs. Furthermore, lncRNA MEG8 knockdown upregulated miR‑203 (P<0.01) expression, but did not alter miR‑34a or miR‑200b expression (both P>0.05). Subsequent experiments revealed that miR‑203 silencing exerted no significant effect on the expression levels of lncRNA MEG8 (P>0.05) in HemECs. In addition, miR‑203 silencing increased cell proliferation (P<0.05) and invasion (P<0.01), but suppressed apoptosis (P<0.05). miR‑203 silencing also reversed the effect of lncRNA MEG8 knockdown on the proliferation (P<0.05), apoptosis (P<0.001) and invasion (P<0.01) of HemECs. Moreover, lncRNA MEG8 knockdown downregulated jagged canonical notch ligand 1 (JAG1; P<0.05) and Notch1 (P<0.05) expression levels, while miR‑203 silencing upregulated JAG1 (P<0.01) and Notch1 (P<0.01) expression levels and reversed the effects of lncRNA MEG8 knockdown on JAG1 (P<0.01) and Notch1 (P<0.01) expression in HemECs. In conclusion, the findings of the present study suggested that lncRNA MEG8 knockdown may inhibit cell proliferation and invasion, but promote cell apoptosis in hemangioma via miR‑203‑induced mediation of the Notch signaling pathway.

PMID:34713294 | DOI:10.3892/mmr.2021.12512

Endoscopic Transcanal and Transmastoid Laser-Assisted Resection of Middle Ear Capillary Hemangioma

Fetched: October 30th, 2021, 5:01am GMT by Evan J Patel

Otol Neurotol. 2021 Oct 28. doi: 10.1097/MAO.0000000000003386. Online ahead of print.

ABSTRACT

OBJECTIVE: To present a rare case of a middle ear capillary hemangioma in an adult.

PATIENT: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss.

INTERVENTION: Endoscopic laser-assisted resection.

MAIN OUTCOME MEASURES: Clinical, radiographic, and histopathological findings of a capillary hemangioma.

RESULTS: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively.

CONCLUSIONS: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization.

PMID:34711778 | DOI:10.1097/MAO.0000000000003386

Diode laser photocoagulation of intraoral (and perioral) venous malformations: Cases series

Fetched: October 30th, 2021, 5:01am GMT by Dounia Sarfi

Int J Surg Case Rep. 2021 Sep 28;88:106436. doi: 10.1016/j.ijscr.2021.106436. Online ahead of print.

ABSTRACT

Vascular anomalies are subdivided into vascular tumors (hemangiomas) and vascular malformations. They are frequently located in the head, neck, and oral cavity. They are common complaints reported in patients seeking treatment for aesthetic or functional issues. However, recent advances in the diagnosis and management of these lesions are improving treatment strategies. This review provides both basic and up-to-date knowledge on the most common vascular anomalies encountered by practitioners. Due to the wide variability of treatment options which often generates debate, this paper work aims to provide a comprehensive approach of these lesions based upon current concepts and practical clinical experience. Our article is about 4 patients who had consulted for one or several purplish, elevated, well limited and soft lesion. These lesions was not painful, but worrying for patients. Therapies for VAs continue to generate a dilemma for oral surgeons. Several treatment options were reported, including conventional surgery with or without adjunctive preoperative embolization, and drug therapies, such corticosteroids, intralesional injection of corticosteroids and intralesional injections of sclerosing agents. All of these therapeutic approaches carry a high risk of severe side effects such as scars, pain, and bleeding [7]. Nowadays, advances in the use of lasers have allowed doctors an effective treatment with minimal side effects [9]. All our cases described in this article were done by Pr Haitami, using the 980 nm Diode laser, and a complete healing was observed in about 8 months at the most. The laser is therefore a great help in the management of this type of lesion.

PMID:34710772 | DOI:10.1016/j.ijscr.2021.106436

Bean's syndrome (blue rubber bleb nevus syndrome - BRBNS) as a gastrointestinal bleeding - case report and review of the literature

Fetched: October 29th, 2021, 5:01am GMT by Małgorzata Lenarcik

Pol J Pathol. 2021;72(2):190-194. doi: 10.5114/pjp.2021.109524.

ABSTRACT

BRBNS is a rare syndrome of vascular malformations caused by the TEK mutation associated with numerous lesions of the skin and gastrointestinal tract. We present a case report of 41 year old man with severe anemia with recurrent bleedings. The detailed clinical, endoscopical and histopathological description is given as a wide range of differential diagnosis of vascular lesions based on pathophysiology and updated classification of vascular lesions. Clinicopathological diagnosis and treatment options of BRBNS are discussed.

PMID:34706529 | DOI:10.5114/pjp.2021.109524

Phace Syndrome in Children: Two Case Reports

Fetched: October 28th, 2021, 5:01am GMT by Kaoutar Imrani

Radiol Case Rep. 2021 Oct 14;16(12):3882-3886. doi: 10.1016/j.radcr.2021.09.023. eCollection 2021 Dec.

ABSTRACT

PHACE(S) syndrome combines: posterior fossa brain malformations, face hemangioma, arterial cerebrovascular abnormalities, cardiovascular abnormalities, eye abnormalities, and ventral developmental defects (Sternal defects or supra-umbilical rope). The diagnosis is based on the association of an infant hemangioma exceeding 5cm in size on the face, neck, scalp with 1 major criteria or 2 minor criteria. Imaging, especially Gadolinium MRI and MRA of the brain, neck, and aortic arch, transthoracic echocardiography, or even cardiac MRI play a key role in the detection of associated neurological and cardiovascular abnormalities. We report 2 cases of PHACE syndrome revealed by 2 different clinical presentations.

PMID:34703511 | PMC:PMC8523872 | DOI:10.1016/j.radcr.2021.09.023

Primary thyroid hemangioma: a case report and literature review

Fetched: October 28th, 2021, 5:01am GMT by Seyed Hossein Fattahi Masuom

Kardiochir Torakochirurgia Pol. 2021 Sep;18(3):186-189. doi: 10.5114/kitp.2021.109385. Epub 2021 Oct 5.

NO ABSTRACT

PMID:34703479 | PMC:PMC8525273 | DOI:10.5114/kitp.2021.109385

Primary Pleural Hemangioendothelioma: A Case Report and Literature Review

Fetched: October 28th, 2021, 5:01am GMT by Elham Askari

Case Rep Oncol. 2021 Aug 16;14(2):1201-1211. doi: 10.1159/000518243. eCollection 2021 May-Aug.

ABSTRACT

Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular endothelial cells. It can originate from any tissue; however, it occurs most frequently in the liver and lung. Pleural epithelioid hemangioendothelioma (PEH), EHE developing from pleura, is even more infrequent and less reported in the literature. In the following report, we discuss a 40-year-old man who initially presented with right-sided chest pain. Computed tomography revealed pleural thickening and effusion in his right thoracic cavity. PEH diagnosis was confirmed with immunohistochemistry. In order to provide the readers with an inclusive understanding of the disease, we collected the PEH cases reported in the literature. Despite the scarcity of the reported PEH cases (to our best knowledge), the compiled literature review of the disease enables the readers to grasp a better comprehension of the disease.

PMID:34703437 | PMC:PMC8460936 | DOI:10.1159/000518243

Current Perspectives of Prenatal Sonography of Umbilical Cord Morphology

Fetched: October 28th, 2021, 5:01am GMT by David M Sherer

Int J Womens Health. 2021 Oct 18;13:939-971. doi: 10.2147/IJWH.S278747. eCollection 2021.

ABSTRACT

The umbilical cord constitutes a continuation of the fetal cardiovascular system anatomically bridging between the placenta and the fetus. This structure, critical in human development, enables mobility of the developing fetus within the gestational sac in contrast to the placenta, which is anchored to the uterine wall. The umbilical cord is protected by unique, robust anatomical features, which include: length of the umbilical cord, Wharton's jelly, two umbilical arteries, coiling, and suspension in amniotic fluid. These features all contribute to protect and buffer this essential structure from potential detrimental twisting, shearing, torsion, and compression forces throughout gestation, and specifically during labor and delivery. The arterial components of the umbilical cord are further protected by the presence of Hyrtl's anastomosis between the two respective umbilical arteries. Abnormalities of the umbilical cord are uncommon yet include excessively long or short cords, hyper or hypocoiling, cysts, single umbilical artery, supernumerary vessels, rarely an absent umbilical cord, stricture, furcate and velamentous insertions (including vasa previa), umbilical vein and arterial thrombosis, umbilical artery aneurysm, hematomas, and tumors (including hemangioma angiomyxoma and teratoma). This commentary will address current perspectives of prenatal sonography of the umbilical cord, including structural anomalies and the potential impact of future imaging technologies.

PMID:34703323 | PMC:PMC8541738 | DOI:10.2147/IJWH.S278747

Frameless Multisession Radiosurgery for Symptomatic Circumscribed Choroidal Hemangioma

Fetched: October 28th, 2021, 5:01am GMT by V Sai Shreya

Int J Radiat Oncol Biol Phys. 2021 Nov 1;111(3S):e72-e73. doi: 10.1016/j.ijrobp.2021.07.432.

ABSTRACT

PURPOSE/OBJECTIVE(S): Choroidal hemangioma (CH) is a benign vascular tumor that induces exudative retinal detachment and consequent visual symptoms. Current standard treatments for CH include cryotherapy, diathermy, photocoagulation, PDT, transpupillary thermotherapy, and Radiation. SRS is preferred in the treatment of CH because of its characteristic stiff dose-fall-off and accuracy. Current retrospective study evaluated tumor volume reductions and improvements to visual acuity following multisession Radiosurgery in the treatment of symptomatic circumscribed choroidal hemangioma.

MATERIALS/METHODS: Study comprised of Thirteen patients with symptomatic circumscribed CHs diagnosed between August 2012 to December 2019 (8 Male & 5 female). 3 pts. had Sturge-Weber syndrome. All patients underwent frameless SRS following retrobulbar block, Thermoplastic immobilization & Imaging (CT & MRI). A total of 15-18gy / 3 frcs was planned using a 5mm collimator, prescribed to 90% isodose and treatment delivered on a frameless robotic radiosurgery system with skull tracking or C arm Linac equipped with hexapod robotics. Best corrected visual acuity, funduscopic findings, basal tumor diameter and tumor thickness were recorded at baseline and at follow-up visits every 6 -month intervals.

RESULTS: The mean age of patients was 33 years (range: 12-61 years) and the mean duration of clinical or radiological follow-up was 33 months (range: 16-80 months). The mean volume, Basal diameter, tumor height & tumor thickness at baseline were 533.5 mm³ (range: 124-1150 mm³); 8 mm (range: 5-12mm, ± 2.3); 2.8mm (range, 2.0 - 4.5mm); tumor thickness was 5.1 (range: 3.0-11.0, ± 2.7) mm respectively. 6 tumors were subfoveolar, 4 were juxtafoveolar, and 3 were extrafoveolar. Exudative retinal detachment resolved within a median of 5.5 months (response rate, 100%; 95% CI, 48%-100%). Median best-corrected visual acuity was 20/50 (range, 20/22-20/90) at diagnosis and 20/25 (range, 20/20-20/60) 18 months after treatment. The visual acuity of the affected eye had improved at the latest follow-up examination (P = .018) in all patients. Tumor height had decreased a median of 32% (range, 10-40%) by 6 months and 41% (range, 17%-59%) by 20 months. No recurrence of exudative RD occurred. Thinning of the CHs was observed in most patients; however, symptomatic radiation toxicity had not developed in any of the patients.

CONCLUSION: Multisession radiosurgery could be an acceptable alternative treatment for symptomatic circumscribed CH with resolution of exudative RD being achieved within 5-6mo.

PMID:34701955 | DOI:10.1016/j.ijrobp.2021.07.432

Propranolol Suppresses Proliferation and Migration of HUVECs through Regulation of the miR-206/VEGFA Axis

Fetched: October 27th, 2021, 5:01am GMT by Ting Zhang

Biomed Res Int. 2021 Oct 16;2021:7629176. doi: 10.1155/2021/7629176. eCollection 2021.

ABSTRACT

Propranolol has been used in the first-line therapy of infantile hemangioma (IH) for a number of years; however, the mechanisms through which propranolol regulates IH are not yet fully understood. In the present study, microRNA (miRNA/miR) sequencing analysis was performed to identify differentially expressed miRNAs in human umbilical vascular endothelial cells (HUVECs) treated with propranolol. Cell viability and apoptosis were detected using CCK-8 assay and flow cytometry, respectively. Cell migration was assessed using wound healing, Transwell, and tube formation assays. Methylation-specific PCR was then used to investigate the promoter methylation status. The levels of oxidative stress indicators, including superoxide dismutase, glutathione, and malondialdehyde were also detected. Finally, cell cycle analysis was performed using flow cytometry and western blotting. It was observed that propranolol induced the upregulation of miR-206 in HUVECs, which was caused by demethylation of the miR-206 promoter. Moreover, propranolol significantly inhibited the proliferation of HUVECs by inducing apoptosis, while these phenomena were reversed by miR-206 antagomir. VEGFA was found to be a target gene of miR-206. In addition, propranolol notably inhibited the migration and induced G1 arrest of the HUVECs, whereas these results were eliminated by miR-206 antagomir. Collectively, the findings of the present study demonstrated that propranolol may inhibit the proliferation and migration in HUVECs via modulating the miR-206/VEGFA axis. These findings suggest a novel mechanism through which propranolol suppresses the progression of IH.

PMID:34697590 | PMC:PMC8541866 | DOI:10.1155/2021/7629176

Vascular Birthmarks as a Clue for Complex and Syndromic Vascular Anomalies

Fetched: October 26th, 2021, 5:01am GMT by Andrea Diociaiuti

Front Pediatr. 2021 Oct 7;9:730393. doi: 10.3389/fped.2021.730393. eCollection 2021.

ABSTRACT

Vascular birthmarks are common in neonates (prevalence: 20-30%) and mostly incidental findings sometimes with spontaneous regression (salmon patch and nevus simplex). Capillary malformations are found in about 1% and infantile hemangiomas are found in 4% of mature newborns. Vascular malformations are classified according to their most prominent vessel type. The term "capillary malformation" (port wine stain) includes a wide range of vascular lesions with different characteristics; they may be isolated or part of specific syndromic conditions. Part of the infantile hemangiomas and of the vascular malformations may require treatment for functional or cosmetic reasons, and in rare cases, investigations are also necessary as they represent a clue for the diagnosis of complex vascular malformation or tumors associated with extracutaneous abnormalities. Complex vascular malformations are mostly mosaicism due to early somatic mutations. Genetic advances have led to identify the main pathogenic pathways involved in this disease group. Diffuse capillary malformation with overgrowth, Klippel-Trenaunay syndrome, CLAPO syndrome, CLOVES syndrome, and megalencephaly-capillary malformation belong to the PIK3CA-related overgrowth. Capillary malformation-arteriovenous malformation underlies a fast-flow vascular malformation, sometimes manifesting as Parkes-Weber syndrome. Recognition of these different types of capillary vascular stains is sometimes difficult; however, associated findings may orient the clinicians while genetic testing may confirm the diagnosis. Lymphatic malformation frequently manifests as large masses that compress and/or infiltrate the surrounding tissues, representing a neonatal emergency when airways are involved. Infantile hemangiomas may cause functional and/or permanent esthetical damage, depending on their localization (such as periorbital area, lip, nose); large (more than 5 cm) infantile hemangiomas with a segmental distribution can be associated with obstruction or malformations of the underneath organs with complications: PHACE syndrome, LUMBAR/SACRAL syndrome, and beard infantile hemangioma. In our review, we discuss controversies regarding the international classification and emerging concepts in the field of vascular anomalies. Finally, we discuss potential developments of new, non-invasive diagnostic techniques and repurposing of target therapies from oncology. Complex and/or life-threatening vascular tumors and malformations are extremely rare events and they represent a considerable therapeutic challenge. Early recognition of clinical signs suggestive for a specific disease may improve therapeutic outcomes and avoid severe complications.

PMID:34692608 | PMC:PMC8529251 | DOI:10.3389/fped.2021.730393

Permalink for 'Blood Supply of Cranial Nerves Passing Through the Cavernous Sinus: An Anatomical Study and Its Implications for Microsurgical and Endoscopic Cavernous Sinus Surgery'

Blood Supply of Cranial Nerves Passing Through the Cavernous Sinus: An Anatomical Study and Its Implications for Microsurgical and Endoscopic Cavernous Sinus Surgery

Fetched: October 26th, 2021, 5:01am GMT by Edinson Najera

Front Oncol. 2021 Oct 8;11:702574. doi: 10.3389/fonc.2021.702574. eCollection 2021.

ABSTRACT

BACKGROUND: Despite improvements in surgical techniques, cranial nerve (CN) deficits remain the most frequent cause of disability following cavernous sinus (CS) surgery. The most common tumor affecting the CS is meningioma. They originate from lateral wall and have their blood supply from meningohypophyseal trunk (MHT) and inferolateral trunk (ILT). Pituitary adenomas commonly invade the CS through its medial wall and receive blood supply form medial branches of the internal carotid artery (ICA) (superior and inferior hypophyseal arteries). Some tumors may grow within the CS (e.g. trigeminal schwannomas, hemangiomas). These tumors are fed by all the intracavernous ICA branches. Tumors involving the CS may also displace the neurovascular structures, therefore, a better understanding of intracavernous neurovascular anatomy may reduce the postoperative morbidity associated with approaching CS tumors. In this anatomical study, the anatomic variations and their clinical implications of the intracavernous CNs' blood supply were evaluated through transcranial and endonasal routes.

METHODS: Twenty sides of ten adult cadaveric formalin-fixed, latex-injected specimens were dissected in stepwise fashion under microscopic and endoscopic magnification. The origin and course of the intracavernous ICA branches supplying the intracavernous CNs are studied.

RESULTS: The proximal segment of the oculomotor nerve receives blood supply from the ILT in 85%, and the tentorial artery of the MHT in 15% of specimens. The distal segment is exclusively supplied by the ILT. The proximal trochlear nerve receives blood supply from the ILT (75%) and the tentorial artery (25%); the distal segment is exclusively supplied by the superior orbital branch. The proximal third of the abducens nerve receives its vascularity exclusively from the dorsal meningeal artery, and its middle and distal thirds from the ILT. The ophthalmic and proximal maxillary segments of the trigeminal nerve also receive blood supply from the ILT. The distal maxillary segment is supplied by the artery of the foramen rotundum. All ILT branches terminate on the inferomedial aspects of the intra-cavernous CNs. Extensive anastomoses are found between ILT branches and the branches arising from external carotid artery.

CONCLUSION: Understanding the anatomy of the intracavernous ICA's branches is important to improving surgical outcomes with tumors involving the CS.

PMID:34692480 | PMC:PMC8531550 | DOI:10.3389/fonc.2021.702574

Hepatic epithelioid hemangioendothelioma simulating liver metastasis: A case report

Fetched: October 26th, 2021, 5:01am GMT by Badr Serji

Ann Med Surg (Lond). 2021 Sep 22;70:102885. doi: 10.1016/j.amsu.2021.102885. eCollection 2021 Oct.

ABSTRACT

INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Liver involvement is often multinodular simulating metastases. Herein, we report a rare case of HEHE mimicking liver metastases in a female patient.

CASE PRESENTATION: A 43-years-old female patient, presented with complaints of pain in the right upper quadrant. Laboratory tests were all within the normal range. Abdominal ultrasound revealed multiple heterogeneous hypoechoic liver lesions. A thoracic and abdominopelvic computed tomography showed bilateral pulmonary micronodules with multiple hypodense hepatic nodules involving both lobes. Percutaneous ultrasound-guided biopsy with pathological study and immunohistochemistry staining revealed the diagnosis of hepatic epithelioid hemangioendothelioma.

DISCUSSION: HEHE usually involves both liver lobes with three radiological presentations: single nodular, multiple nodular, or diffuse types, the diffuse type reflects an advanced stage. 3/4 of the cases are initially misdiagnosed as liver metastases or primary liver tumors. The pathological study with the immunochemistry stainings confirms the diagnosis. There is no standard treatment for HEHE due to its rarity and lack of prospective randomized studies.

CONCLUSION: HEHE is a rare tumor of vascular origin of unknown etiology with malignant potential and unpredictable course. The therapeutic management of this rare condition is not codified and is discussed on a case-by-case basis. Surgical treatment remains the best option with an excellent outcome.

PMID:34691426 | PMC:PMC8519773 | DOI:10.1016/j.amsu.2021.102885

Pleural hemangioma: A case report and review of the literature

Fetched: October 26th, 2021, 5:01am GMT by Binil Mathew Jacob

Pathol Res Pract. 2021 Oct 12;228:153650. doi: 10.1016/j.prp.2021.153650. Online ahead of print.

ABSTRACT

A middle-aged female with history of multinodular goiter, Hashimoto disease, and chronic vitamin B12 deficiency presented with palpitations and subsequent exertional dyspnea. Initial radiographic analysis suggested mediastinal cavernous hemangioma, but biopsy showed features consistent with pleural hemangioma. Pleural hemangioma should be considered among the differential diagnoses for recurrent unilateral pleural effusion. Pleural hemangioma should be distinguished from other more common entities including the similarly benign pulmonary hemangioma and the more aggressive pleural hemangioendothelioma.

PMID:34695620 | DOI:10.1016/j.prp.2021.153650

Ocular adnexal intramuscular hemangioma arising from the eyelid: case report and literature review

Fetched: October 26th, 2021, 5:01am GMT by Kenneth K H Lai

Orbit. 2021 Oct 22:1-5. doi: 10.1080/01676830.2021.1990352. Online ahead of print.

ABSTRACT

Intramuscular hemangioma (IMH) is rare in the ocular adnexa and is uncommonly found in infants. We describe a 1-month-old female infant with an unremarkable birth history presented with subacute onset of right upper eyelid swelling. Clinical examination revealed a purplish subcutaneous mass over the right upper eyelid causing mechanical ptosis. MRI revealed a well-defined subcutaneous mass, which was hyperintense on the T1 weighted images and showed intense enhancement after administration of intravenous gadolinium. Our working diagnosis was infantile capillary hemangioma, and the patient received two intralesional steroid injections without diminishing the lesion size. The lesion had become static in size and the subsequent MRI 14 years later revealed the same lesion had become hyperintense on the T1 weighted image. An excisional biopsy revealed mixed type IMH. IMH should be considered among young patients with upper eyelid swelling, particularly in those who show a poor response to steroid treatment.

PMID:34686104 | DOI:10.1080/01676830.2021.1990352

COVID-19 Associated Choroidopathy

Fetched: October 26th, 2021, 5:01am GMT by Youssef Abdelmassih

J Clin Med. 2021 Oct 13;10(20):4686. doi: 10.3390/jcm10204686.

ABSTRACT

The aim of the study is to report on the indocyanine green angiography (ICGA) and OCT findings in patients hospitalized for severe COVID infection. In this observational prospective monocentric cohort study, we included patients hospitalized for severe COVID infection. The main outcomes were ICGA and OCT findings. A total of 14 patients with a mean age of 58.2 ± 11.4 years and a male predominance (9/14 patients; 64%) were included. The main ICGA findings included hypofluorescent spots in 19 eyes (68%), intervortex shunts in 10 eyes (36%), and characteristic "hemangioma-like" lesions in five eyes (18%). "Hemangioma-like" lesions were both unique and unilateral, and showed no washout on the late phase of the angiogram. The main OCT findings included focal choroidal thickening in seven eyes (25%), caverns in six eyes (21%) and paracentral acute middle maculopathy lesions in one eye (4%). All patients hospitalized for severe COVID infection had anomalies on ICGA and OCT. Lesions to both retinal and choroidal vasculature were found. These anomalies could be secondary to vascular involvement related directly or indirectly to the SARS-CoV2 virus.

PMID:34682810 | PMC:PMC8541653 | DOI:10.3390/jcm10204686

Infantile Hemangiomas: An Update on Pathogenesis and Treatment

Fetched: October 26th, 2021, 5:01am GMT by Małgorzata Kowalska

J Clin Med. 2021 Oct 9;10(20):4631. doi: 10.3390/jcm10204631.

ABSTRACT

Infantile hemangiomas are the most common benign vascular tumors in infancy. This review includes an update on the current knowledge on pathogenesis, a discussion on indications for treatment, and a review of the mechanisms underlying the different treatment methods. Although most infantile hemangiomas require only active observation because of their natural course, which results in involution, about 10% present with complications that require immediate treatment. The basic treatment includes systemic and topical options. In cases of insufficient response or rebound growth, other forms of treatment should be considered. In some cases, combined therapy might be initiated.

PMID:34682753 | PMC:PMC8539430 | DOI:10.3390/jcm10204631

A Two-Year Clinical Description of a Patient with a Rare Type of Low-GGT Cholestasis Caused by a Novel Variant of USP53

Fetched: October 26th, 2021, 5:01am GMT by Olga Shatokhina

Genes (Basel). 2021 Oct 14;12(10):1618. doi: 10.3390/genes12101618.

ABSTRACT

Here, we report a novel truncating mutation in the ubiquitin-specific peptidase gene (USP53) causing low-γ-GT (GGT) cholestasis. Genetic testing was carried out, including clinical exome sequencing for the proband and Sanger sequencing for the proband and his parents. The proband harbored a novel c.1017_1057del (p.(Cys339TrpfsTer7)) mutation in the ubiquitin carboxyl-terminal hydrolase (UCH) domain of USP53; we describe the clinical and laboratory features of the patient with a rare type of low-GGT cholestasis caused by this variant. The clinical presentation was found to be similar to that of phenotypes described in previous studies. However, there was an unusual presence of liver hemangiomas observed in our patient. Thus, our report reinforces the link between USP53 mutations and cholestasis. With this report, we confirm USP53 as the gene for low-GGT cholestasis and describe liver hemangiomas as a possible additional symptom of the phenotype spectrum. The inclusion of USP53 in the OMIM database and liver gene panels can further increase the effectiveness of molecular genetic studies.

PMID:34681012 | PMC:PMC8535307 | DOI:10.3390/genes12101618

Medical Management of Infantile Hemangiomas: An Update

Fetched: October 26th, 2021, 5:01am GMT by Caroline Colmant

Paediatr Drugs. 2021 Oct 22. doi: 10.1007/s40272-021-00477-9. Online ahead of print.

ABSTRACT

Infantile hemangioma (IH) is the most common benign vascular tumor of infancy, affecting about 5% of infants. It has a characteristic growth pattern of early rapid proliferation followed by progressive involution. Although most IH evolve favorably, complications are observed in 10-15% of cases, justifying treatment. For over 10 years now, propranolol has become the first-line therapy for complicated IH, revolutionizing their management and their prognosis. In this article, we review the clinical features, associations, complications/sequelae and therapeutic approaches for IH, focusing on current medical therapy. Indications for treatment and various treatment options, including propranolol and other oral β-blockers, topical timolol, and corticosteroids are presented. Current controversies regarding oral propranolol such as pre-treatment screening, in- vs out-patient initiation of treatment, early and potential long-term side effects and recommended monitoring are discussed.

PMID:34677814 | DOI:10.1007/s40272-021-00477-9

Therapeutic Effect of Polidocanol Sclerotherapy on Oral Vascular Malformations

Fetched: October 26th, 2021, 5:01am GMT by Satoshi Fukuzawa

Dent J (Basel). 2021 Oct 14;9(10):119. doi: 10.3390/dj9100119.

ABSTRACT

Various treatments for oral vascular malformation (VM) have been reported. Polidocanol and absolute ethanol have also been reported for sclerotherapy. However, there are still few reports on the therapeutic effect and dosage of polidocanol sclerotherapy. Therefore, we examined its therapeutic effects on oral VM. There were 17 sites of VMs, with nine patients diagnosed with oral VM at the Department of Dental and Oral Surgery, Tsukuba University Hospital. The medical records were retrospectively investigated to determine the site, hemangioma volume, polidocanol injection volume, and therapeutic effect. The volume of hemangiomas was calculated using magnetic resonance images. Based on the site, oral VMs were observed in the tongue, buccal mucosa, lips, and oral floor in eight, three, five, and one patients, respectively. The average size of the site was 3071 mm³. The average injection dose of polidocanol at one site was 2.86 mL, the average number of administrations was 1.6, and the response rate was 88.2%. No adverse events were observed. The median numerical rating scale scores were 2/10 (0-6/10) and 0/10 (0-1/10) the day after surgery and 1 week after surgery, respectively. Univariate regression analysis of the total dose in successful cases provided the following formula: 1.3 + 0.00025 × volume (mm³) (mg). Polidocanol sclerotherapy is an effective treatment method for oral VM.

PMID:34677181 | PMC:PMC8534538 | DOI:10.3390/dj9100119

Long-Term Observation and Treatment of Epithelioid Haemangioendothelioma of the Mediastinum: A Case Report

Fetched: October 26th, 2021, 5:01am GMT by Qiuli Zhi

Front Surg. 2021 Oct 5;8:678572. doi: 10.3389/fsurg.2021.678572. eCollection 2021.

ABSTRACT

Epithelioid haemangioendothelioma is a rare angiogenic tumour originating from vascular endothelial or pre-endothelial cells, and it can occur anywhere in the body, such as the liver, lung, bone, spleen, lymph nodes, parotid gland, and thyroid. In the fifth revision of the WHO classification, epithelioid haemangioendothelioma (EHE) was described as a malignant vascular neoplasm composed of epithelioid endothelial cells, distinct from epithelioid angiosarcoma. We, herein, report one patient with EHE of the left upper mediastinum who underwent resection and radiotherapy during the first therapeutic process. Multiple metastases occurred in the thoracic vertebrae 6 years later, and resection and multiple radiotherapies were performed. The condition of the patient remained stable at the last review in October 2020, and it has been more than 8 years since her first admission. The reasonable "take-away" lessons from the case are active treatment and prolonged surveillance.

PMID:34676238 | PMC:PMC8525909 | DOI:10.3389/fsurg.2021.678572

Cavernous Hemangioma in the Orbital Cavity: Case Report

Fetched: October 26th, 2021, 5:01am GMT by José Afonso de Almeida

Eur J Dent. 2021 Oct 21. doi: 10.1055/s-0041-1732948. Online ahead of print.

ABSTRACT

Cavernous hemangiomas are benign malformations of vascular origin, usually well circumscribed and slow to grow. These lesions can be asymptomatic, being discovered unintentionally in imaging exams or symptomatic, indicated mainly by the presence of proptosis, diplopia, and visual disturbances by optic nerve compression. The complementary exams involve computed tomography associated with contrast, color Doppler, magnetic resonance, and angiography. Treatment can be conservative or surgical depending on the case, and the open therapy usually involves lateral, supraorbital, transconjunctival, transantral, pterional, transnasal, and extradural endoscopic orbitotomy. The present study aims to report a recurrent case of hemangioma in the orbital cavity signaled by ocular proptosis, hyperemia, and ocular pain.The lesion was achieved through the Weber-Ferguson access with zygomatic osteotomy and preservation of the infraorbital nerve. The excision of the lesion was performed, and the previously displaced fragments were fixed with 1.5 mm mini plates. The patient has a chance of progressing with visual impairment due to considerable manipulation of the optic nerve and is being followed up.The reported case showed a successful diagnosis and therapeutic conduct, remaining now in the evolution and follow-up scenario.

PMID:34674195 | DOI:10.1055/s-0041-1732948

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