ArticleInternational journal of clinical and experimental pathology2019
Histopathologic characteristics and immunotypes of perivascular epithelioid cell tumors (PEComa).
Article in International journal of clinical and experimental pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 25 citations in OpenAlex.
- PEComas: current concepts in diagnosis, molecular pathways, and emerging treatments.The oncologist · 2026Review
- Malignant uterine PEComa: a narrative literature review and challenging case report.Journal of cancer research and clinical oncology · 2026Review
- Perivascular Epithelioid Cell Neoplasm of the Lung: A Patient Case Report.Case reports in pathology · 2026Article
- Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review.Cancers · 2025Review
- Exploring perivascular epithelioid cell tumors (PEComas) in the CNS: insights from two case reports and a comprehensive literature review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Review
- Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis.Frontiers in oncology · 2025Article
- Primary Hepatic Perivascular Epithelioid Tumor (PEComa) With High Risk of Malignancy: A Case Report.Case reports in gastrointestinal medicine · 2025Article
- Article
- Multifocal malignant perivascular epithelioid cell tumor (PEComa) of the peritoneum: A case report.International journal of surgery case reports · 2024Article
- A case report of recurrent primary posterior mediastinal perivascular epithelioid cell tumour compressing the right inferior pulmonary vein, atria, and inferior vena cava.European heart journal. Case reports · 2024Article
- A case report and literature review: leiomyosarcoma or perivascular epithelioid cell neoplasm?Frontiers in oncology · 2024Article
- Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study.Journal of cancer research and clinical oncology · 2023Article
- Primary retroperitoneal PEComa: an incidental finding.BMJ case reports · 2022Article
- Cutaneous Angiomyolipoma-A Distinct Entity That Should Be Separated From Classic Angiomyolipoma: Complete Review of Existing Cases and Defining Fundamental Features.JMIR dermatology · 2022Article
- Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma.BMJ case reports · 2022Article
- Interrogating the Genomic Landscape of Uterine Leiomyosarcoma: A Potential for Patient Benefit.Cancers · 2022Review
- TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review.World journal of surgical oncology · 2022Review
- Efficacy of Sirolimus Treatment in PEComa-10 Years of Practice Perspective.Journal of clinical medicine · 2021Article
- TFE3 activation in a TSC1-altered malignant PEComa: challenging the dichotomy of the underlying pathogenic mechanisms.The journal of pathology. Clinical research · 2021Article
- [Angiomyolipoma of the liver-a case report and review of the literature].Der Pathologe · 2020Review
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesIn order to provide further evidence for the origin and differentiation of PEComa, the clinicopathologic and immunophenotype findings were analyzed in 26 cases with literature review.
methodsImmunohistochemistry and special staining were used.
resultsMultinucleated giant cells and polymorphism were scattered visibly in 53.8% and 76.9% of the cases and spotty necrosis and hemorrhage were observed in 38% of the cases. Capsular micro-invasion was detected in 46% cases accompanied by hemorrhage and/or necrosis in the tumors with diameters larger than 5 cm. It was also found that 100% of cases diffusely expressed SMA, Melan-A, and vimentin except one negative for HMB-45. The tumor cells partly expressed CD56, CD99, desmin, and S-100 and were negative for CK-pan, TFE3, CD117, CD44, and CD34. Clinical follow-up found that 22 out of 23 patients were alive, with no recurrence or progression, ranging from 42 to 82 months. However, one patient died from leukemia.
conclusionsIn this study, the histopathologic features with the co-expressions of SMA and melanin, were the diagnostic basis of PEComas. The interspersed expressions of desmin and S-100 were helpful for the differential diagnosis of leiomyoma and neuroma. The expressions of S-100, CD56, and CD99 supported the origins of the pluripotent cells from the neural crests. Tumors larger than 5 cm in diameter with micro-hemorrhaging/necrosis and micro-capsular invasions should be considered either uncertain or of malignant potential. The spontaneous rupturing of blood vessels may be related to the amyloidosis and desmin negative expression, and broken elastic fibers.
Indexed as
Identifiers
31933841PMC6949869W3005172654What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.