ArticleJournal of cancer research and clinical oncology2023
Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study.
Article in Journal of cancer research and clinical oncology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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Who cites it
10 citing papers in PubMed, 1 synthesis or guideline pooled it, 8 citations in OpenAlex.
- MRI findings for differentiating benign and malignant soft tissue tumors: a systematic review-part 2: key imaging findings.Skeletal radiology · 2026Pooled it
- Perivascular epithelioid cell (PEComa) tumors of the musculoskeletal system: a case series with MR imaging and histopathologic review.Skeletal radiology · 2026Article
- Soft tissue reporting and data system (soft tissue-RADS): framework for radiologists specializing in imaging of musculoskeletal tumors and tumor-like lesions.Skeletal radiology · 2026Review
- From the spectrum of PEComa: a renal angiomyolipoma.Journal of surgical case reports · 2026Article
- Beyond Chordoma: A Comprehensive Review of Sacral Lesions.Current oncology (Toronto, Ont.) · 2026Review
- Duodenal perforation after retroperitoneal laparoscopic radical nephrectomy for ruptured malignant renal PEComa with spontaneous retroperitoneal hemorrhage: a case report.Frontiers in oncology · 2026Article
- Article
- Femoral PEComa Treated With Combined Surgery and Radiotherapy: A Case Report.Clinical case reports · 2026Article
- Article
- Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis.Frontiers in oncology · 2025Article
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Authors and funding
14 authors at 10 institutions in 1 country.
Funding
Abstract
purposePerivascular epithelioid cell tumors (PEComas) of the bone and soft tissues are rare mesenchymal neoplasms, some of which are malignant. However, their clinical and pathological characteristics remain unclear. This study was performed to investigate the clinical and pathological characteristics of PEComas in bone and soft tissues by leveraging information from the Japanese Musculoskeletal Oncology Group.
methodsNine patients, including four male and five female patients with a median age of 50 years, were retrospectively reviewed. PEComas of the visceral organs, including the uterus and retroperitoneum, were excluded.
resultsEight tumors arose in the soft tissue and one in the bone, with a mean size of 8.8 cm. Four patients showed local recurrence or distant metastasis. The 1-year survival rate was 78%. Pathologically, eight tumors were classified as malignant and one as having uncertain malignancy potential. Half of the tumors showed high MIB-1 index values of > 30%. Immunohistochemically, the melanocyte marker HMB45 was expressed in 89% of the cases, and muscle-specific markers were expressed only in 30-50% of the cases. Transcription factor binding to IGHM enhancer 3 (TFE3) expression was positive in 100% of the patients. Tumors with high expression of TFE3 were classified as PEComas with malignant potential according to Folpe's classification.
conclusionsBone and soft tissue PEComas may have a higher malignancy potential than other visceral PEComas and are more likely to develop as TFE3-rearranged PEComas.
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