外耳道原发性腺样囊性癌2例并文献复习

Two Cases of Primary Adenoid Cystic Carcinoma of the External Auditory Canal and Review of the Literature

摘要: 目的：探讨外耳道原发腺样囊性癌的临床病理特征及其鉴别诊断。方法：外耳道原发性腺样囊性癌是一种发生于外耳道的一种附属器源性的恶性肿瘤，我们报道2例罕见外耳道原发性腺样囊性癌，结合文献对其临床特征、组织学形态、免疫表型及鉴别诊断等进行综合分析。结果：两例均为左侧外耳道新生物，临床表现侵袭性生长并伴有明显按压痛，一例出现明显听力下降并伴流脓，CT示软组织密度灶，与周围组织界限欠清，并有一例见有骨质破坏。免疫组化：CK7(+)，CD117(+)，GCDFP-15(−)，Ki-67 (<10%)。结论：目前国内外报道的外耳道原发性腺样囊性癌不超过80例，其组织学形态分型有筛状型、管状型、实性型等。本文两例以不规则的腺腔结构为主，高倍镜下细胞异型性明显并伴有明显的核分裂象，但Ki-67增殖指数并不高。目前的治疗方式仍以手术切除为主。但局部复发及转移可能性大，一经确诊，应以扩大切除并辅以局部放疗的综合治疗为主。

Abstract: Objective: To investigate the clinicopathologic features of primary adenoid cystic carcinoma of the external auditory canal and its differential diagnosis. Methods: Primary adenoid cystic carcinoma of the external auditory canal is a malignant tumor of appendicular origin that occurs in the external auditory canal. We report two rare cases of primary adenoid cystic carcinoma of the external audi-tory canal, and comprehensively analyze their clinical features, histological patterns, immunophe-notypes, and differential diagnosis with the literature. Results: Both cases were neoplasm of the left external auditory canal, with clinical manifestations of invasive growth accompanied by obvious pressure pain, one case showed obvious hearing loss with pus, and CT showed soft tissue density foci with poorly defined boundaries with the surrounding tissues, and bone destruction in one case. Immunohistochemistry: CK7(+), CD117(+), GCDFP-15(−), Ki-67 (<10%). Conclusion: No more than 80 cases of primary adenoid cystic carcinoma of the external auditory canal have been reported at home and abroad, and their histologic morphology is typed as sieve-like, tubular, and solid types. The two cases in this paper were dominated by irregular glandular cavity structure, with obvious cellular heterogeneity and obvious nuclear schizophrenia under high magnification, but with low Ki-67 proliferation index. The current treatment is still based on surgical resection. However, the possibility of local recurrence and metastasis is high, and once diagnosed, comprehensive treat-ment with extended resection supplemented by local radiotherapy should be the mainstay.