Gan David Eng Yeow, Choy Rebecca Xin Yi, Sellappan Harivinthan, Hayati Firdaus, Azizan Nornazirah
Department of Surgery, Queen Elizabeth Hospital, Ministry of Health Malaysia, Kota Kinabalu, Sabah, Malaysia.
Department of Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, Jalan Yaacob Latif, Bandar Tun Razak, Kuala Lumpur, Malaysia.
Oman Med J. 2021 Mar 23;36(2):e239. doi: 10.5001/omj.2021.21. eCollection 2021 Mar.
Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal tumors with discrete histological and immunohistochemical characteristics. Even rarer among them are cutaneous and subcutaneous PEComas. We describe a 34-year-old woman who presented with a large anterior abdominal subcutaneous lesion showing intact overlying skin and no obvious invasion of the abdominal musculature. A wide local excision was performed. Histopathology revealed a solitary tumor measuring 75 × 55 × 90 mm with epithelioid cells in nests with thin fibrovascular septa and spindle cells. Resection margins were clear with no invasion to the skin or rectus sheath. Tumor cells were positive for HMB-45 but negative for other markers. This is the largest subcutaneous PEComa reported to date.
血管周上皮样细胞肿瘤（PEComas）是一类罕见的间叶性肿瘤，具有独特的组织学和免疫组化特征。其中，皮肤和皮下PEComas更为罕见。我们报告一例34岁女性，其腹部前侧皮下有一巨大肿物，表面皮肤完整，未见明显侵犯腹部肌肉组织。行广泛局部切除。组织病理学检查显示为一孤立性肿瘤，大小为75×55×90mm，上皮样细胞呈巢状排列，有薄的纤维血管间隔，还有梭形细胞。切除边缘清晰，未侵犯皮肤或腹直肌鞘。肿瘤细胞HMB-45呈阳性，但其他标志物呈阴性。这是迄今为止报道的最大的皮下PEComa。
