Brandt Hannes Hermann, Baumhoer Daniel, Tetter Nora
Department of Otolaryngology, Kantonsspital Aarau AG, Aarau, Switzerland.
Bone Tumor Reference Centre, Institute of Medical Genetics and Pathology, University Hospital Basel, University of Basel, Basel, Switzerland.
Biomed Hub. 2022 Feb 25;7(1):31-35. doi: 10.1159/000521830. eCollection 2022.
Minor salivary gland malignancies are a rare entity among head and neck tumors. As in major gland neoplasms, adenoid cystic carcinoma and mucoepidermoid carcinoma are the most common histological subtypes. Malignant tumors affecting minor salivary glands include a wide range of histopathologic types. Localization in the epipharynx and hyalinizing clear cell carcinoma subtype are exceedingly rare. A 47 year-old male presented to our clinic with a complaint of slowly progressing left-sided nasal obstruction. Endoscopy revealed a well-defined nodular epipharyngeal mass. Radiographic evaluation discovered a nonvascularized tumor of the tubal protuberance. The tumor was treated with wide local excision. Staging at the time found no evidence of regional lymph node metastases. Histologic examination revealed a hyalinizing clear cell salivary gland carcinoma demonstrating an EWSR1-ATF1 gene fusion. Restaging endoscopy and radiographic imaging 3 months after initial therapy did not reveal any signs of tumor persistence. The patient is currently in follow-up.
小唾液腺恶性肿瘤在头颈部肿瘤中是一种罕见的疾病。与大唾液腺肿瘤一样，腺样囊性癌和黏液表皮样癌是最常见的组织学亚型。影响小唾液腺的恶性肿瘤包括多种组织病理学类型。发生于下咽的肿瘤以及透明变性透明细胞癌亚型极为罕见。一名47岁男性因左侧鼻塞进展缓慢前来我院就诊。内镜检查发现一个边界清晰的结节状下咽肿物。影像学评估发现咽鼓管隆突处有一个无血管化的肿瘤。该肿瘤接受了广泛局部切除治疗。当时分期未发现区域淋巴结转移的证据。组织学检查显示为透明变性透明细胞唾液腺癌，存在EWSR1-ATF1基因融合。初始治疗3个月后复查内镜及影像学检查未发现肿瘤持续存在的迹象。该患者目前正在接受随访。
