Wilhite Annelise M, Dal Zotto Valeria, Pettus Paige, Jeansonne Julie, Scalici Jennifer
University of South Alabama, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, USA.
East Carolina University, Department of Pathology, USA.
Gynecol Oncol Rep. 2022 Mar 21;40:100962. doi: 10.1016/j.gore.2022.100962. eCollection 2022 Apr.
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that exhibits immunohistochemical evidence of smooth muscle and melanocytic differentiation. We report a case of uterine PEComa in a 21 year-old primigravida, presenting at time of c-section as a small subserosal lesion that expressed soft tan-brown tissue fragments. Microscopically the cells were epithelioid, staining positive for TFE3 and HMB45. Significant cytologic atypia and mitotic activity were concerning for malignancy. The patient was treated post-partum with total robotic hysterectomy and right salpingo-oopherectomy, and is currently without evidence of disease.
This case of PEComa diagnosed during pregnancy highlights the importance of intra-operative biopsy and the difficulty of predicting malignant potential of PEComa in the setting of a gravid uterus with a dynamic smooth muscle architecture.
血管周上皮样细胞瘤（PEComa）是一种罕见的间叶性肿瘤，具有平滑肌和黑素细胞分化的免疫组化证据。我们报告一例21岁初产妇的子宫PEComa病例，剖宫产时表现为一个小的浆膜下病变，呈现软的棕褐色组织碎片。显微镜下，细胞为上皮样，TFE3和HMB45染色呈阳性。显著的细胞异型性和有丝分裂活性提示恶性可能。患者产后接受了全机器人子宫切除术和右侧输卵管卵巢切除术，目前无疾病证据。
该例妊娠期诊断的PEComa病例突出了术中活检的重要性，以及在妊娠子宫平滑肌结构动态变化的情况下预测PEComa恶性潜能的困难。
