Ocular PEComas are frequently melanotic and TFE3-translocated: report of two cases including the first description of PRCC-TFE3 fusion in PEComa
Sign Up to like & getrecommendations! Published in 2020 at "Virchows Archiv"
DOI: 10.1007/s00428-020-02890-w
Abstract: Ocular perivascular epithelioid cell tumor (PEComa) is exceedingly rare. We reported two examples involving the choroid and subconjunctival tissue, respectively, in patients aged 17 and 20 years. Both tumors comprised packets and sheets of large polygonal… read more here.
Keywords: pecoma; first description; prcc tfe3; description prcc ... See more keywords
Cytopathology of extra-renal perivascular epithelioid cell tumor (PEComa): a series of 7 cases and review of the literature.
Sign Up to like & getrecommendations! Published in 2020 at "Journal of the American Society of Cytopathology"
DOI: 10.1016/j.jasc.2020.09.006
Abstract: INTRODUCTION Perivascular epithelioid cell tumor (PEComa) is a family of rare mesenchymal tumors consisting of histologically and immunohistochemically distinctive perivascular epithelioid cells. Relatively little is known about the cytopathology of extra-renal PEComas. Because of a… read more here.
Keywords: extra renal; perivascular epithelioid; pecoma; cell ... See more keywords
The potential of emerging new therapeutics for the treatment of perivascular epithelioid cell tumors (PEComa)
Sign Up to like & getrecommendations! Published in 2018 at "Expert Opinion on Orphan Drugs"
DOI: 10.1080/21678707.2018.1493376
Abstract: ABSTRACT Introduction: Perivascular epithelioid cell tumor (PEComa) constitutes a family of rare mesenchymal soft-tissue tumors. These tumors pose a significant diagnostic challenge, given their cytomorphology and characteristic co-expression of both melanocytic and myogenic markers. While… read more here.
Keywords: perivascular epithelioid; mtor; pecoma; epithelioid cell ... See more keywords
Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa
Sign Up to like & getrecommendations! Published in 2022 at "Medicine"
DOI: 10.1097/md.0000000000029506
Abstract: Abstract Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumour (PEComa). Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of… read more here.
Keywords: clinical diagnosis; hepatic pecoma; diagnosis treatment; pecoma ... See more keywords
Abstract B010: Response to treatment with nab-sirolimus among patients with primary uterine tumors: A subgroup analysis from AMPECT
Sign Up to like & getrecommendations! Published in 2024 at "Clinical Cancer Research"
DOI: 10.1158/1557-3265.endo24-b010
Abstract: Introduction/objective: AMPECT (NCT02494570) is a pivotal study of nab-sirolimus in patients with malignant perivascular epithelioid cell tumors (PEComa). Malignant PEComa is an aggressive, rare sarcoma for which cytotoxic chemotherapies provide limited patient benefit. Inactivation of… read more here.
Keywords: treatment; response; pecoma; nab sirolimus ... See more keywords
Characterization of Clinical Cases of Malignant PEComa via Comprehensive Genomic Profiling of DNA and RNA
Sign Up to like & getrecommendations! Published in 2020 at "Oncology"
DOI: 10.1159/000510241
Abstract: Purpose: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal soft tissue neoplasm often linked to mTOR pathway activation via TSC2 mutation. We analyzed a series of 31 consecutive metastatic PEComa (mPEComa) cases using a… read more here.
Keywords: pecoma; comprehensive genomic; mpecoma; dna rna ... See more keywords
Metastatic perirectal PEComa treated by checkpoint inhibitor immunotherapy and multimodal treatment: case report and review of the literature
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DOI: 10.1177/17588359241280541
Abstract: Malignant PEComas are an extremely rare subtype of soft tissue sarcomas. Here, we report the case of a man presenting with a perirectal PEComa and liver metastasis. Since the tumor harbored a tumor mutational burden… read more here.
Keywords: treatment; report; tumor; pecoma ... See more keywords
Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature
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DOI: 10.1186/s12885-019-5383-0
Abstract: BackgroundPerivascular Epithelioid Cell Tumors (PEComa) is an extraordinarily rare mesenchymal neoplasm especially the malignant type originating from the lung. To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. Firm… read more here.
Keywords: tumor; pecoma; primary adenocarcinoma; case ... See more keywords
Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge
Sign Up to like & getrecommendations! Published in 2017 at "European Journal of Medical Research"
DOI: 10.1186/s40001-017-0248-y
Abstract: BackgroundPerivascular epithelioid cell tumor (PEComa) is a rare subtype of mesenchymal origin tumor composed of epithelioid cells which exhibits immunohistochemical co-expressions of melanocytic markers and smooth muscle markers.Case presentationIn the first case, malignant uterine PEComa… read more here.
Keywords: pecoma; uterine pecoma; cell tumor; epithelioid cell ... See more keywords
Clinicopathological features and treatment of perivascular epithelioid cell tumor.
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Clinical Oncology"
DOI: 10.1200/jco.2021.39.15_suppl.e23538
Abstract: e23538 Background: Perivascular epithelioid cell tumor (PEComa) is a rare form of mesenchymal neoplasms. No clinical trial of large sample size regarding medical treatment of PEComa has been reported so far. Previous retrospective studies with… read more here.
Keywords: mtor; treatment; perivascular epithelioid; pecoma ... See more keywords
Perivascular epithelioid cell tumor (PEComa) of the liver. An extremely rare diagnosis.
Sign Up to like & getrecommendations! Published in 2023 at "Revista espanola de enfermedades digestivas"
DOI: 10.17235/reed.2023.9558/2023
Abstract: Hepatic PEComa are tumors that show perivascular epithelioid cell differentiation. Scarcely published, its management is based on small case series and currently its treatment of choice is surgical resection. We present a clinical case of… read more here.
Keywords: pecoma; cell tumor; epithelioid cell; perivascular epithelioid ... See more keywords