Gebhart Michael, Coltofeanu Andrei
a Service de chirurgie orthopédique, de l'Institut Bordet , Université libre de Bruxelles , Brussels , Belgium.
b Service de chirurgie orthopédique , Andrei Clinic Hospital, Regina Maria, Medicova , Bucarest , Romania.
Acta Chir Belg. 2019 Apr;119(2):118-122. doi: 10.1080/00015458.2017.1394658. Epub 2019 Feb 17.
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.
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by multiple histological sections at second look operation nine months after primary surgery, where cement has been replaced by autologous bone graft, and by subsequent follow-up.
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.
血管周上皮样细胞肿瘤（PEComa）是一类罕见的间叶组织肿瘤，由组织学和免疫组织化学上独特的血管周上皮样细胞（PEC）组成。这些肿瘤已在多个解剖部位被描述。发生于骨的PEComa尤其罕见，据报道可为原发性或转移性病变。其中无一位于足部。
我们报告一例距骨原发性骨PEComa病例，据我们所知这是首例发表的此类病例。治疗及结果：采用病损内肿瘤切除、磨钻处理、在肿瘤壁应用氩气刀以及用骨水泥对肿瘤腔进行一期填充的保肢手术治疗，初次手术后九个月的二次探查手术中经多次组织学切片检查均未发现肿瘤复发，此时骨水泥已被自体骨移植替代，并进行了后续随访。
PEComa是一种非常罕见的肿瘤，其在骨中的出现尤其罕见。据我们所知，此处报告的距骨部位的PEComa病例是文献中首例报道的病例。
