Quiroga-Garza Gabriela, Lee Jeong Hyeon, El-Naggar Adel, Black Jennifer O, Amrikachi Mojgan, Zhai Qihui J, Ayala Alberto G, Ro Jae Y
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX 77030.
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX 77030; Department of Pathology, Korea University Medical Center, Anam Hospital, Seoul, 152-703, Republic of Korea.
Hum Pathol. 2015 May;46(5):725-31. doi: 10.1016/j.humpath.2015.01.012. Epub 2015 Feb 12.
Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid is a rare traditionally "low-grade" tumor that predominantly occurs in women. Approximately 50 cases have been reported in the literature. It arises in a background of Hashimoto thyroiditis and is characterized by nests of epidermoid and mucin-secreting cells located within an eosinophil-rich sclerotic stroma. Herein, we outline the clinicopathological and immunohistochemical characteristics of 6 cases of thyroid SMECE. All tumors were detected in women (age, 36-89 years; average, 59 years), and all patients underwent total thyroidectomies. Clinicopathological findings included extensive tumor invasion into the adjacent soft tissues, trachea, pharynx, and esophagus. Of 6 specimens, 5 had positive surgical margins. Cervical lymph node metastases were seen in 4, and distant metastases were in 3 patients. Immunohistochemically, all tumors were positive for CK19, galectin 3, and p63 and negative for calcitonin, calponin, S-100, and smooth muscle actin. Interestingly, 2 tumors also showed faint focal staining for thyroglobulin, and 2 others had focal positivity for thyroid transcription factor 1. Together, galectin 3 and CK19 expression supported the malignancy of these lesions, and p63 expression raised the possibility that these tumors originated from the ultimobranchial body. In summary, SMECE tumors in our series exhibit a clear female predominance with aggressive behavior and appear to arise from pluripotent solid cell nests. A correct diagnosis is crucial to providing SMECE patients with the appropriate treatment options, and we recommend a closer follow-up schedule than previously considered.
甲状腺嗜酸性硬化性黏液表皮样癌（SMECE）是一种罕见的传统“低级别”肿瘤，主要发生于女性。文献报道约50例。它发生于桥本甲状腺炎背景下，其特征为富含嗜酸性粒细胞的硬化性间质内存在表皮样细胞巢和黏液分泌细胞巢。在此，我们概述6例甲状腺SMECE的临床病理及免疫组化特征。所有肿瘤均在女性中发现（年龄36 - 89岁；平均59岁），所有患者均接受了甲状腺全切术。临床病理表现包括肿瘤广泛侵犯相邻软组织、气管、咽部和食管。6例标本中，5例手术切缘阳性。4例出现颈部淋巴结转移，3例有远处转移。免疫组化方面，所有肿瘤CK19、半乳糖凝集素3和p63均呈阳性，而降钙素、钙调蛋白、S - 100和平滑肌肌动蛋白均为阴性。有趣的是，2例肿瘤甲状腺球蛋白呈微弱局灶性染色，另外2例甲状腺转录因子1呈局灶性阳性。总之，半乳糖凝集素3和CK19表达支持这些病变的恶性性质，p63表达提示这些肿瘤可能起源于最后鳃体。总之，我们系列中的SMECE肿瘤明显以女性为主，具有侵袭性行为，似乎起源于多能实性细胞巢。正确诊断对于为SMECE患者提供合适的治疗方案至关重要，我们建议采用比以往认为的更密切的随访计划。
