斜坡区恶性腺肌上皮癌1例并文献复习

Myoepithelial Carcinoma in Clivus Region: A Case Report and Literature Review

摘要: 目的：报道1例罕见的斜坡区恶性肌上皮癌，以提高对该疾病的认识。方法：回顾性分析2020年3月我科收治的1例斜坡恶性腺肌上皮癌患者的诊疗经过，包括症状、影像学特点、病理免疫组化、手术治疗及预后等内容；同时进行文献复习，总结该疾病的研究进展。结果：患者女，78岁，因头痛复视，伴左侧鼻腔嗅觉减退、血性分泌物30天来诊，颅脑MRI提示斜坡团块状长T1短T2混杂密度影，边界较清。综合评估患者疾病后行鼻内镜下颅底手术，术中见肿瘤位于中颅底，累及蝶窦、斜坡、双侧岩尖区，深达局部硬脑膜，组织病理提示恶性腺肌上皮瘤伴坏死，免疫组化示：CK+、CK7+、CK5/6+、P63+、CK20−、CDX2−、TTF1−、S100−、Pax8−。患者术中并发脑梗(非术区)，术后不明原因反复发热，经抗凝、调脂、抗感染治疗后逐渐好转出院。结论：肌上皮癌细胞形态学表现多样，免疫组化检查是较为可靠的诊断依据。治疗以手术切除为主，良好预后的关键是确保肿瘤切缘阴性和做好头颈部淋巴结的清扫。

Abstract: Objective: To improve the understanding of the disease, we reported a rare case of malignant my-oepithelial carcinoma in clivus region. Methods: A patient with malignant myoepithelial carcinoma in clivus region was admitted to our department in March 2020. The patient’s diagnosis and treat-ment process were analyzed retrospectively, including symptoms, imaging features, immunohisto-chemical, characteristics, procedure and prognosis; We also reviewed the literature about myoepi-thelial carcinoma to summarize the research progress of the disease. Results: A 78-year-old female patient came to our hospital for headache and diplopia, accompanied by decreased olfaction and bloody secretions in the left nasal cavity for 30 days. MR manifested a hypo-intense mixed mass shadow with clear boundary on T1WI and T2WI in clivus region. After a comprehensive assessment of the patient’s disease, endoscopic skull base surgery was performed. The tumor was found to be located at the base of the middle skull, involving sphenoid sinus, slope, bilateral rock apices, and deep into the local dura. The histopathology suggested malignant adenomyoepithelial tumor with necrosis, and immunohistochemistry showed: CK+, CK7+, CK5/6+, P63+, CK20−, CDX2−, TTF1−, S100−, Pax8−. The patient was complicated with cerebral infarction (non-operative area) during the operation and repeated fever for unknown reasons after the operation. After anticoagulation, lipid regulation and anti-infection treatment, the patient was gradually improved and discharged. Con-clusion: The morphologic manifestations of myoepithelial cancer cells are varied, and immuno-histochemical examination is a reliable diagnostic basis. Surgical resection is the main treatment, the key point in prognosis is to ensure that the tumor margin is negative and do a good job of head and neck lymph node dissection.