Department of Plastic and Reconstructive Surgery, Medical School of Jeonbuk National University, Jeonju, Republic of Korea.
Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju, Republic of Korea.
Medicine (Baltimore). 2023 Aug 11;102(32):e34679. doi: 10.1097/MD.0000000000034679.
The World Health Organization defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. Although PEComas have the potential to behave in a malignant fashion, malignant PEComas are extremely rare.
An 83-year-old man visited our clinic presented with palpable, painless, and movable mass in the right knee area.
Malignant PEComa was diagnosed by incisional biopsy. No metastases was confirmed by radiologic imaging including PET/CT, magnetic resonance imaging, high resolution computed tomography.
We performed wide excision of the mass and used an anterolateral thigh free flap to reconstruct the defect on the right knee.
The permanent histopathology showed malignant PEComa was totally resected. The flap which was performed to cover the defect was survived and the patient discharge without any complications.
PEComa can metastasize to various anatomical regions. Although there is no established standardized treatment, radical resection is still considered the cornerstone of treatment. Rapid and appropriate defect coverage is important to improve the patient's prognosis.
世界卫生组织将血管周上皮样细胞瘤（PEComa）定义为一种间叶性肿瘤，由具有特征性形态学和免疫组织化学特征的血管周上皮样细胞组成。虽然 PEComa 有恶性行为的潜力，但恶性 PEComa 极为罕见。
一名 83 岁男性因右膝区可触及、无痛、可移动的肿块就诊于我院。
通过切开活检诊断为恶性 PEComa。影像学检查（包括 PET/CT、磁共振成像、高分辨率计算机断层扫描）未发现转移。
我们对肿块进行广泛切除，并使用股前外侧游离皮瓣修复右膝的缺损。
永久性组织病理学显示恶性 PEComa 已完全切除。用于覆盖缺损的皮瓣存活，患者无任何并发症出院。
PEComa 可转移至各个解剖区域。虽然没有既定的标准化治疗方法，但根治性切除仍被认为是治疗的基石。快速、适当的缺损覆盖对于改善患者预后至关重要。
