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PEComa of the talus: a unique case of a soft tissue tumor within bone

par Gebhart, Michaël ;Coltofeanu, Andrei
Référence Acta chirurgica Belgica (Ed. bilingue), 119, 2, page (118-122)
Publication Publié, 2019-03

Article révisé par les pairs

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Résumé :

Introduction: Perivascular epithelioid cell tumor (PEComa) is a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs). These tumors have been described at multiple anatomic sites. PEComas presenting in bone are especially rare and they have been reported to be either primary or metastatic lesions. None of them is located at the level of the foot. Patient: We report on a case of primary bone PEComa of the talus, the first one to be published to the best of our knowledge. Treatment and result: Limb sparing surgical treatment has been done using intralesional tumor removal, burring and application of argon beam at the tumor walls and primary filling of the tumor cavity by bone cement with so far absence of any tumor recurrence documented by multiple histological sections at second look operation nine months after primary surgery, where cement has been replaced by autologous bone graft, and by subsequent follow-up. Conclusions: PEComa is a very rare tumor, and its presence in bone is especially rare. The here reported case of PEComa presenting at the level of the talus is, to the best of our knowledge, the first case to be reported in the literature.