ArticleThe American journal of surgical pathology2018
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Article in The American journal of surgical pathology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 82 papers, 1 of them a synthesis that pooled it.
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Who cites it
82 citing papers in PubMed, 1 synthesis or guideline pooled it, 181 citations in OpenAlex.
- Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence.Archives of gynecology and obstetrics · 2024Pooled it
- Multicenter phase II trial of ipilimumab and nivolumab in metastatic or unresectable perivascular epithelioid cell tumor (PEComa): a substudy ofJournal for immunotherapy of cancer · 2025Trial
- Expression of melanocytic markers in uterine smooth muscle tumours.Histopathology · 2026Article
- Article
- Diagnostic utility of novel marker TRIM63 ISH in the workup of uterine perivascular epithelioid cell tumors.Virchows Archiv : an international journal of pathology · 2026Article
- Ultrasound Features of Uterine Perivascular Epithelioid Cell Tumor (PEComa): A Systematic Review.Journal of imaging · 2026Review
- 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
- An update on the role of SMARCA4 deficiency in gynecological neoplasms: how and where.Virchows Archiv : an international journal of pathology · 2026Review
- Cutaneous soft tissue tumors in the 5th edition of the World Health Organization classification of skin tumors: key updates and new entities.Journal of pathology and translational medicine · 2026Article
- An Update on Uterine Smooth Muscle Tumors: Is It a Leiomyoma, a STUMP, or a Leiomyosarcoma?Biomedicines · 2026Review
- Uterine PEComa With Lymphangioleiomyomatosis (LAM)-Like Features: A Case Report.Case reports in pathology · 2026Article
- Clinicopathological and molecular features of gynecologic perivascular epithelioid cell tumors: a single-center study.Frontiers in oncology · 2026Article
- Diagnosis and grading of adrenal cortical carcinoma.Virchows Archiv : an international journal of pathology · 2026Review
- Hepatic perivascular epithelioid cell tumor: A rare mesenchymal tumor with epithelioid features: A case report.Experimental and therapeutic medicine · 2026Article
- Uterine Angiomyolipoma Presenting as a Rapidly Growing Uterine Mass in a Postmenopausal Woman.Diagnostics (Basel, Switzerland) · 2025Article
- Genetic correlates of malignancy in TFE3-rearranged PEComa: a series of 14 cases.Histopathology · 2025Article
- New histopathological and molecular findings in gynecological cancers.International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics · 2025Review
- Article
- CD34-positive pleomorphic uterine sarcoma with NUDT3::RAD51B fusion.Virchows Archiv : an international journal of pathology · 2025Article
22 more citing papers are in PubMed but not listed here.
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Authors and funding
15 authors at 13 institutions in 8 countries.
Funding
Abstract
Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms that may show overlapping morphology and immunohistochemistry with uterine smooth muscle tumors. In this study, we evaluated the morphologic, immunohistochemical, and molecular features of 32 PEComas, including 11 with aggressive behavior. Two distinct morphologies were observed: classic (n=30) and those with a lymphangioleiomyomatosis appearance (n=2). In the former, patients ranged from 32 to 77 (mean: 51) years and 13% had tuberous sclerosis. Tumors ranged from 0.2 to 17 (mean: 5.5) cm with 77% arising in the corpus. Epithelioid cells were present in 100% and a spindled component was seen in 37%. Nuclear atypia was low (53%), intermediate (17%), or high (30%). Mitoses ranged from 0 to 36 (mean: 6) and 0 to 133 (mean: 19) per 10 and 50 high-power fields, with atypical mitoses present in 30%. Thin and delicate vessels were noted in 100%, clear/eosinophilic and granular cytoplasm in 93%, stromal hyalinization in 73%, necrosis in 30%, and lymphovascular invasion in 10%. All tumors were positive for HMB-45, cathepsin K, and at least one muscle marker, with most expressing melan-A (77%) and/or MiTF (79%). A PSF-TFE3 fusion was identified in one while another showed a RAD51B-OPHN1 fusion. Follow-up ranged from 2 to 175 (mean: 41) months, with 63% of patients alive and well, 20% dead of disease, 13% alive with disease, and 3% dead from other causes. In the latter group (n=2), patients were 39 and 49 years old, one had tuberous sclerosis, while the other had pulmonary lymphangioleiomyomatosis. Both tumors expressed HMB-45, cathepsin K, and muscle markers, but lacked TFE3 and RAD51B rearrangements. The 2 patients are currently alive and well. Application of gynecologic-specific criteria (≥4 features required for malignancy: size ≥5 cm, high-grade atypia, mitoses >1/50 high-power fields, necrosis, and lymphovascular invasion) for predicting outcome misclassified 36% (4/11) of aggressive tumors; thus, a modified algorithm with a threshold of 3 of these features is recommended to classify a PEComa as malignant.
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