咽旁上皮-肌上皮癌与双侧沃辛氏瘤共存

Zuzana Mateášiková, R. Salzman, I. Stárek, J. Michálek
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摘要

上皮-肌上皮癌(EMEC)是一种非常罕见的低级别恶性肿瘤,发病率不到所有唾液腺肿瘤的1%。在咽旁间隙,它可以来自腮腺的深叶或口咽的小唾液腺。我们报告一位70岁女性病患,因进行性吞咽困难及旁旁扁桃体肿大。CT扫描显示咽旁间隙囊性形成和双侧腮腺不均匀肿瘤。术前FNAB(细针穿刺活检)未确诊。手术切除后的最终组织学检查证实咽旁间隙上皮-肌上皮癌和两腮腺Warthin肿瘤。EMEC的免疫组化检测显示BRAF、COSM476、COSM775和PIK3CA突变,但未检测到HRAS突变。这个病例报告显示了一个罕见的临床和放射学表现,可能类似于良性诊断。多发性唾液腺肿瘤是相对罕见的疾病。沃辛氏肿瘤是最常见的具有这种行为的肿瘤。双侧腮腺Warthin肿瘤与咽旁上皮-肌上皮癌的同时发生至今尚未见文献报道。关键词:上皮-肌上皮癌咽旁间隙囊肿唾液腺沃辛瘤
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Coexistent parapharyngeal epithelial-myoepithelial carcinoma and bilateral Warthin’s tumors
Epithelial-myoepithelial carcinoma (EMEC) is a very rare low-grade malignancy with an incidence of less than 1% of all salivary gland tumors. In the parapharyngeal space, it can arise either from the deep lobe of the parotid gland or from minor salivary glands of the oropharynx. We present a case of a 70-year-old female patient with progressive dysphagia and a bulging of paratonsillar space. A CT scan revealed a cystic formation in the parapharyngeal space and bilateral inhomogeneous tumors in parotid glands. Preoperative FNAB (fine needle aspiration bio­psy) was undiagnostic. Definitive histologic examination after surgical removal confirmed epithelial-myoepithelial carcinoma in the parapharyngeal space and Warthin’s tumors in both parotid glands. Immunohistochemical tests of the EMEC revealed BRAF, COSM476 a COSM775 and PIK3CA mutations, however, no HRAS mutation was detected. This case report shows a rare clinical and radiological presentation that could resemble benign dia­gnosis. Multiple salivary gland tumors are relatively rare conditions. Warthin’s tumor is the most common tumor with this type of behaviour. The coincidence of bilateral Warthin’s tumor of parotid glands with a parapharyngeal epithelial-myoepithelial carcinoma has not been documented in literature so far. Keywords: epithelial-myoepithelial carcinoma – parapharyngeal space cyst – salivary gland – Warthin’s tumor
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