12例内淋巴囊肿瘤的临床病理分析暨文献复习

Clinicopathological analysis of 12 cases of endolymphatic Sac tumors and literature review

目的观察12例内淋巴囊肿瘤(ELST)的临床病理学特征,并结合文献探讨其病理诊断与鉴别诊断,以提高病理医生及临床医生对此病的认识。方法收集2013年8月至2022年2月昆明医科大学第一附属医院病理科12例内淋巴囊肿瘤患者的临床资料,描述病例的显微镜下特点、免疫表型及预后,并复习相关文献。结果12例ELST中男性7例,女性5例,年龄21~58岁。12例患者肿块最大径2.5 cm,平均直径(1.9±0.33)cm。临床上多表现为渐进性听力下降、耳鸣、眩晕等。影像学显示颞骨岩部中后部软组织肿物,边界不清、密度稍高并可出现骨质破坏。镜下肿瘤细胞主要排列呈乳头-囊样结构,乳头轴心为血管丰富的纤维间质,可见大小不一的腺腔样结构,被覆单层柱状上皮,细胞温和,胞质较丰富,核圆形或卵圆形,可见核仁,无明显核分裂象。12例ELST中CK、VIM均呈阳性表达,KI-67指数1%~2%,2例有CD56强弱不等的表达,3例EMA仅表现为局灶细胞膜腔缘阳性。12例GFAP、Olig-1、PR、S-100、Syn、TTF-1、TG、CD10均呈阴性表达。12例患者均进行肿瘤切除术且完整切除,电话随访至今,所有患者均尚存,有1例有复发。结论ELST的发生与性别无关,所有年龄段均可发病,病理学诊断依据其典型的显微镜下特征,结合免疫组织化学检测结果及影像学资料可明确诊断,手术完整切除可治愈。

Objective To investigate the clinicopathological features of 12 cases of endolymph sac tumor(ELST),and to explore its pathological diagnosis and differential diagnosis based on the literature,in or⁃derto improve the understanding of pathologists and clinicians.Methods The clinical data of 12 patients with endolymphatic sac tumor in the Department of Pathology,The First Affiliated Hospital of Kunming Medical Uni⁃versity from August 2013 to February 2022 were collected.The microscopic characteristics,immunophenotypes and prognosis of the cases were described,and relevant literatures were reviewed.Results Among the 12 ELST patients,7 were male and 5 were female,aged 21⁃58 years.The maximum diameter and average diameter of 12 patients were 2.5 cm and(1.9±0.33 cm).Clinical manifestations are progressive hearing loss,tinnitus,vertigo.Imaging studies showed a soft tissue mass in the middle and posterior part of the petropetros of the temporal bone,with ill⁃defined boundary,slightly higher density and possible bone destruction.Under the microscope,the tumor cells are mainly arranged in a papillary⁃cystic structure,the axis of the papilla is a fibrous stroma with rich blood vessels,and glandular cavity⁃like structures of various sizes can be seen,covered with a single layer of columnar epithelium,the cells are mild,the cytoplasm is rich,and the nucleus is round Shaped or oval,with visi⁃ble nucleoli and no obvious mitotic figures.12 cases of ELST showed positive expression of CK and VIM,ki⁃67 index 1%~2%,2 cases showed different expression of CD56,3 cases of EMA only showed positive expression of focal membrane margin.GFAP,OLig⁃1,PR,S⁃100,Syn,TTF⁃1,TG and CD10 were all negative in 12 cases.All 12 patients underwent tumor resection and complete resection.Up to now,all patients are still alive,and 1 pa⁃tient has recurrence.Conclusion The occurrence of ELST is not related to gender,and it can occur in all ages.The pathological diagnosis is based on its typical microscopic features,combined with immunohistochemical test results and imaging data.The diagnosis can be confirmed,and complete surgical resection can be cured.