Department of Pathology and Laboratory Medicine, Miller School of Medicine University of Miami, Miami, FL.
Department of Pathology, The Johns Hopkins University Hospital, Baltimore, MD.
Am J Clin Pathol. 2021 Apr 26;155(5):729-737. doi: 10.1093/ajcp/aqaa180.
Epithelioid sarcoma (ES) rarely arises in the nerve. To increase our understanding of this unusual tumor originating in the nerve, we describe the features of three cases and review the literature.
Clinical data, imaging, pathology, treatment, and follow-up are detailed. A systematic literature review was conducted.
Two patients were male and one female; the median age was 24 years. The patients had neurologic symptoms, and the tumors arose in large nerves and ranged from 2.4 to 5.8 cm. The tumors were avid on positron emission tomography-computed tomography and showed increased signal intensity on T2-weighted magnetic resonance imaging. Centered in the nerve, the tumors grew with an infiltrative pattern and encased the nerve fascicles. All were treated with wide resection, and adjuvant treatment included combinations of chemotherapy and radiation. One recurred, and the limb was amputated. Metastases were documented to lymph nodes, lung, pleura, and skin. One patient died of disease after 54 months. Literature review including our cases showed that tumors stained with pancytokeratin (9/9), EMA (4/4), and CD34 (7/7); there was loss of INI1 in all six cases tested.
ES rarely arises in the peripheral nerve, and its infiltrative nature often requires morbid surgery. The differential includes a variety of benign and malignant epithelioid neoplasms.
上皮样肉瘤（ES）很少发生在神经。为了增加我们对起源于神经的这种不常见肿瘤的理解，我们描述了三例病例的特征并复习了文献。
详细介绍了临床资料、影像学、病理学、治疗和随访情况。进行了系统的文献复习。
两名男性和一名女性患者；中位年龄为 24 岁。患者有神经症状，肿瘤发生在大神经上，大小为 2.4 至 5.8cm。肿瘤在正电子发射断层扫描 - 计算机断层扫描上摄取增加，并在 T2 加权磁共振成像上显示信号强度增加。肿瘤位于神经中央，呈浸润性生长，包裹神经束。所有患者均接受广泛切除术治疗，辅助治疗包括化疗和放疗的联合应用。1 例复发，行截肢术。记录到淋巴结、肺、胸膜和皮肤转移。1 例患者在 54 个月后死于疾病。包括我们病例在内的文献复习显示，肿瘤对广谱细胞角蛋白（9/9）、EMA（4/4）和 CD34（7/7）染色阳性；所有 6 例检测到 INI1 缺失。
ES 很少发生在周围神经，其浸润性性质通常需要进行恶性手术。鉴别诊断包括多种良性和恶性上皮样肿瘤。
