Khattab Mohamed H, Sherry Alexander D, Ahlers Carolyn G, Kirschner Austin N
Department of Radiation Oncology, Vanderbilt University Medical Center, Nashville, TN 37232, United States.
Vanderbilt University School of Medicine, Nashville, TN 37232, United States.
World J Clin Oncol. 2018 Dec 20;9(8):200-207. doi: 10.5306/wjco.v9.i8.200.
Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade, malignant tumor that constitutes less than one percent of all salivary gland tumors. To date, only one other case report has described radiation-associated EMC in the English language medical literature.
In this report, we describe the case of a 56-year-old male patient who presented with a neck mass diagnosed as EMC of the left submandibular gland approximately 30 years after mantle field radiation and chemotherapy for Hodgkin lymphoma. Treatment included resection, re-resection with nodal dissection, and adjuvant chemoradiotherapy. This patient was also diagnosed with 4 other secondary malignancies, including stage IV diffuse large B cell lymphoma in the abdomen with subsequent brain metastases, low-grade neuroendocrine carcinoma of the lung, Hurthle cell adenoma, and small B cell lymphoma before the patient expired. This case provides important information regarding the pathology, clinical sequelae, and management of a patient diagnosed with radiation-associated EMC amidst four concurrent malignancies.
Further investigation is needed on the efficacy of adjuvant radiotherapy in EMC, especially atypical EMC.
上皮-肌上皮癌（EMC）是一种罕见的低级别恶性肿瘤，在所有涎腺肿瘤中占比不到1%。迄今为止，英文医学文献中仅有另一例关于放疗相关EMC的病例报告。
在本报告中，我们描述了一名56岁男性患者的病例，该患者在接受霍奇金淋巴瘤斗篷野放疗和化疗约30年后，出现颈部肿块，被诊断为左下颌下腺EMC。治疗包括切除、再次切除并进行淋巴结清扫，以及辅助放化疗。该患者在去世前还被诊断出患有其他4种继发性恶性肿瘤，包括腹部IV期弥漫性大B细胞淋巴瘤并伴有脑转移、肺低级别神经内分泌癌、许特耳细胞腺瘤和小B细胞淋巴瘤。该病例提供了关于一名在同时患有四种恶性肿瘤的情况下被诊断为放疗相关EMC患者的病理学、临床后遗症及治疗方面的重要信息。
需要进一步研究辅助放疗在EMC，尤其是非典型EMC中的疗效。
