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Journal of Cancer Research and Clinical Oncology

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Open Access 20-07-2023 | Research

Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Authors: Yuya Izubuchi, Shunsuke Hamada, Yoshikazu Tanzawa, Ikuo Fujita, Jungo Imanishi, Hirotaka Koyanagi, Akiyoshi Shimatani, Tadashi Komatsubara, Takaaki Tanaka, Mana Fukushima, Yoshiaki Imamura, Takafumi Ueda, Hirotaka Kawano, Akihiko Matsumine

Published in: Journal of Cancer Research and Clinical Oncology | Issue 14/2023

Abstract

Purpose

Perivascular 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.

Methods

Nine 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.

Results

Eight 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.

Conclusions

Bone 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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Title: Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study
Authors: Yuya Izubuchi
Shunsuke Hamada
Yoshikazu Tanzawa
Ikuo Fujita
Jungo Imanishi
Hirotaka Koyanagi
Akiyoshi Shimatani
Tadashi Komatsubara
Takaaki Tanaka
Mana Fukushima
Yoshiaki Imamura
Takafumi Ueda
Hirotaka Kawano
Akihiko Matsumine
Publication date: 20-07-2023
Publisher: Springer Berlin Heidelberg
Published in: Journal of Cancer Research and Clinical Oncology / Issue 14/2023
Print ISSN: 0171-5216
Electronic ISSN: 1432-1335
DOI: https://doi.org/10.1007/s00432-023-05114-1

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