Sassi Farah, Boujelbene Nadia, Abbes Imen, Naija Lamia, Khessairi Nayssem, Mrad Karima
Department of Pathology, Salah Azaiz Institute, Faculty of Medicine, University Tunis El Manar.
Department of Pathology, Salah Azaiz Institute, Faculty of Medicine, University Tunis El Manar.
Int J Surg Case Rep. 2024 Nov;124:110455. doi: 10.1016/j.ijscr.2024.110455. Epub 2024 Oct 12.
Neoplasms with perivascular epithelioid-cell differentiation (PEComa) are rare. We present a case of multifocal malignant PEComas arising in the peritoneum.
A 61-year-old woman presented with a painful abdominal mass that had progressed over the past year and recently increased in size. Pelvic magnetic resonance imaging (MRI) showed two masses: an 11-cm partially solid and cystic tumor, with moderate contrast uptake probably originating from the left ovary, and an 18-cm sized, intraperitoneal abdominal heterogeneous enhancing mass. Two nodules in the mesentery and the bladder peritoneum were discovered intraoperatively. The patient underwent Hudson's intervention with resection of the epiploic mass and the two nodules. Microscopic examination revealed a tumor proliferation of epithelioid clear cells with delicate vascular septae. Immunohistochemistry was positive for HMB45, MelanA and SMA. The final diagnosis of a malignant PEComa was retained. The patient developed two local peritoneal recurrences and died after 26 months of follow-up.
Our case is original by its multifocal presentation being the seventh case of a multifocal malignant PEComa reported in the literature. PEComa's clinical presentation and radiographic appearance can often mimic a soft tissue clear cell sarcoma.
Clinical and pathological correlations are mandatory to make a precise diagnosis and adapt the treatment of PEComas.
伴有血管周上皮样细胞分化的肿瘤（PEComa）较为罕见。我们报告一例发生于腹膜的多灶性恶性PEComa病例。
一名61岁女性因腹部疼痛性肿块就诊，该肿块在过去一年中逐渐增大，近期体积进一步增加。盆腔磁共振成像（MRI）显示两个肿块：一个11厘米大小的部分实性和囊性肿瘤，造影剂摄取中等，可能起源于左侧卵巢，另一个18厘米大小的腹腔内不均匀强化肿块。术中发现肠系膜和膀胱腹膜有两个结节。患者接受了Hudson手术，切除了网膜肿块和两个结节。显微镜检查显示上皮样透明细胞肿瘤增殖，伴有纤细的血管间隔。免疫组化HMB45、MelanA和SMA呈阳性。最终诊断为恶性PEComa。患者出现两次局部腹膜复发，随访26个月后死亡。
我们的病例具有多灶性表现，是文献报道的第七例多灶性恶性PEComa，具有独特性。PEComa的临床表现和影像学表现常可模仿软组织透明细胞肉瘤。
临床与病理相关性对于准确诊断和调整PEComa的治疗至关重要。
