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Pediatric case of colonic perivascular epithelioid cell tumor complicated with intussusception and anal incarceration:A case report

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World Journal of Gastrointestinal Oncology 2022年第7期14卷 1348-1355页

作者： Luan Kou Wen-Wen Zheng Li Jia Xiao-Li Wang Ji-Hai Zhou Jiao-Rong Hao Zhu Liu Feng-Yu Gao Department of Gastroenterology Shandong Provincial Maternal and Child Health Care HospitalJinan 250014Shandong ProvinceChina

BACKGROUND perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal *** can occur in many organs but is rare in the colorectum,especially in ***,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent *** describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current *** SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested *** direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by *** pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar *** cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was *** immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spottumor as *** recovery was good at the 4 mo *** The diagnosis of PEComa mainly depends on pathology and *** resection is the preferred treatment method.

关键词： perivascular epithelioid cell tumor Colonic Intussusception Anal incarceration Endoscopic direct-vision intussusception treatment Case report

TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery:A case report and review of literature

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World Journal of Clinical Cases 2020年第18期8卷 4207-4214页

作者： Nah Ihm Kim Ji Shin Lee Yoo Duk Choi U Chul Ju Jong Hee Nam Department of Pathology Chonnam National University Hospital and Medical SchoolGwangju 61469South Korea Department of Pathology Chonnam National University Hwasun Hospital and Medical SchoolHwasun 58128South Korea Department of Obstetrics and Gynecology Chonnam National University Hwasun Hospital and Medical SchoolHwasun 58128South Korea

BACKGROUND perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell *** tumor is characterized by immunoreactivity for melanocytic and myogenic markers but can be misdiagnosed as more common tumors with similar characteristics,including gastrointestinal stroma tumors or ***,a subset of PEComas has been reported to harbor a transcription factor binding to TFE3 ***,we report a rare case of TFE3-expressing malignant PEComa arising from the *** SUMMARY A 50-year-old woman presented with abdominal discomfort for 3 *** of laboratory tests were all within the normal ranges,and the patient had no notable medical *** resonance imaging revealed a large tumor on the right side of the pelvic floor,which was originally suspected to be a primary ovarian ***,during surgery,the tumor was revealed to have originated from the ***,the tumor was composed of bundles of spindle cells and sheets of epithelioid *** coagulative necrosis and numerous mitotic figures were *** revealed that the tumor cells were positive for smooth muscle actin,HMB-45,and TFE3 *** involvement of the rectal serosa was identified,leading to a final diagnosis of malignant PEComa of the *** resection was followed by adjuvant *** recurrence or metastasis was observed over a 6-month follow-up *** Malignant PEComa of the mesentery is extremely rare and should be distinguished from morphological mimics through differential diagnosis and immunohistochemistry.

关键词： perivascular epithelioid cell tumor TFE3 Differential diagnosis Mesentery Histology Case report

Hepatic perivascular epithelioid cell tumor:A case report

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World Journal of Clinical Cases 2022年第13期10卷 4273-4279页

作者： Yong-Fang Li Liang Wang Yi-Jing Xie Department of Hepatology Lanzhou University The Second HospitalLanzhou 730030Gansu ProvinceChina Department of Radiology Lanzhou University The Second HospitalLanzhou 730030Gansu ProvinceChina

BACKGROUND perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple *** originating in the liver is extremely rare,with most cases being benign,and only a few cases are *** outcomes are achieved with radical surgical resection,but there is no effective treatment for some large tumors and specific locations that are contraindicated for *** SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous *** ultrasoundguided percutaneous liver aspiration biopsy was *** pathological immunohistochemical staining was HMB45,Melan-A,and smooth muscle actin *** epithelioid tumor was *** tumor was large and could not be completely resected by *** digital subtraction angiography revealed a rich tumor blood supply,and interventional embolization followed by surgery was ***,the patient underwent transarterial embolization(TAE)combined with sorafenib for four *** reexamination indicated no clear vascular staining of the tumor,and the tumor had *** patient was followed up for a short period of time,achieved a stable condition,and surgery was *** Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.

关键词： perivascular epithelioid cell tumor Liver Treatment Transarterial embolization Sorafenib Case report

A perivascular epithelioid cell tumor of the stomach:An unsuspected diagnosis

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World Journal of Gastroenterology 2010年第4期16卷 522-525页

作者： Cristina Aparecida Troques da Silveira Mitteldorf Dario Birolini Luis Heraldo da Camara-Lopes Laboratory of Surgical and Molecular Pathology Hospital Sírio Libanêsrua Dona Adma Jafet 91So PauloSP 01308-050Brazil Department of Surgery of the University of So Paulo Medical School av. Dr. Enéas de Carvalho Aguiar 25505403-000 So Paulo SP Brazil Hospital Sírio Libanês rua Dona Adma Jafet 91 So Paulo SP 01308-050 Brazil Genoa Biotechnology S.A. Alameda Ministro Rocha Azevedo 346 1st floor 01410-000So Paulo SP 01308-050 Brazil

perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological,immunohistochemical and molecular *** report a case of PEComa arising in the antrum of a 71-year-old female with *** tumor,located predominantly in the submucosa as a well delimited nodule,measured 3.0 cm in diameter and was completely resected,with no evidence of the disease ***,it was composed predominantly of eosinophilic epithelioid cells arranged in small nests commonly related to variably sized vessels,with abundant extracellular material,moderate nuclear variation and discrete mitotic *** necrosis,angiolymphatic invasion or perineural infil-tration was *** cells were uniformly positive for vimentin,smooth muscle actin,desmin and melan *** unusual,PEComa should be considered in the differential diagnosis of gastric neoplasia with characteristic epithelioid and oncocytic features and prominent vasculature.

关键词： perivascular epithelioid cell tumor Stomach Gastrointestinal hemorrhage

Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment:A case report

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World Journal of Gastrointestinal Surgery 2024年第10期16卷 3334-3342页

作者： Han-Teng Yang Fu-Rong Wang Na He Yuan-Hua She Yong-Yue Du Wen-Gui Shi Jing Yang Gang Chen Shu-Ze Zhang Feng Cui Bo Long Ze-Yuan Yu Jun-Min Zhu Geng-Yuan Zhang Department of General Surgery Lanzhou University Second HospitalLanzhou 730030Gansu ProvinceChina Department of Pathology Lanzhou University Second HospitalLanzhou 730030Gansu ProvinceChina Oncology Department Ward Lanzhou University Second HospitalLanzhou 730030Gansu ProvinceChina Cuiying Biomedical Research Center Lanzhou University Second HospitalLanzhou 730030Gansu ProvinceChina

BACKGROUND perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and *** occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly *** diagnosis is the gold *** usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)*** SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were *** addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective *** showed an excellent efficacy in the postoperative treatment of the *** Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.

关键词： perivascular epithelioid cell tumor Hepatic Renal Surgery Case report

Hepatic perivascular epithelioid cell tumor:Two case reports and a literature review

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Hepatic perivascular epithelioid cell tumor:Two case reports...