内淋巴囊低度恶性腺癌21例临床病理特征

Clinicopathological features of low-grade malignant endolymphatic sac adenocaricinomas

目的探讨内淋巴囊低度恶性腺癌的临床病理特点。方法收集并分析21例内淋巴囊低度恶性腺癌临床病理资料。结果患者16~71岁不等,平均40.3岁,中位年龄39岁;8例男性,13例女性,男女之比为1.1∶1.6,女性多发。左侧发生13例,右侧7例,1例双侧发生,左侧发生占绝对优势。O型及B型血共占71.4%。免疫组化:CK、EMA和vimtin均(+),S-100(10/14)(+),CD56(9/12)(+),NSE(2/4)(+),GFAP(1/11)(+),Syn、Cg A、TTF-1、TG、CD34和calcitonin均(-)。Ki-67指数平均4.3%。镜下细胞呈乳头状排列可伴有分支,纤维轴心血管多丰富。部分呈扩张的大小不等的甲状腺滤泡样结构,滤泡内含红染的胶质样物,可有扇贝样分泌空泡。有扩张的腔隙。部分病例呈腺性排列,个别呈巢状、腺囊样排列。大部分被覆单层立方上皮,少数细胞扁平或柱状,胞质透亮、淡染。细胞核卵圆形,核仁不明显,染色质细腻,少数有小的核仁。组织易出血,有新鲜及陈旧性出血。约一半患者有坏死骨形成,部分病例见肿瘤组织破坏周围骨。背景纤维组织增生伴玻璃样变性,部分有钙化及砂砾体形成。临床主要表现为听力下降或丧失,其次表现为耳鸣,伴有不同程度的颅神经损伤。随访期间无患者死亡,2例分别于术后1年和7年后复发。结论所有患者进展缓慢,无死亡,2例复发。易误诊。

Objective To explore the clinical-pathological characteristics of endolymphatic sac tumors（ ELSTs）.Methods The clinicopathological data were collected and analyzed in 21 cases of ELSTs. Results The patients were aged 16-71 years（ median： 39 years; mean： 40. 3 years）. There were 13 famales and 8 males,yielding a female/male ratio of 1. 6∶ 1. 1 cases were located left side which were predominante,7 for right side,l for bilateral one. Type O and B blood of the female patients occupied 71. 4%. Immunohistochemically,CK,EMA and vimentin were all positive. S-100（ 10/14）,CD56（ 9/12）,NSE（ 2/4）,GFAP（ 1/11） were partially positive; Syn,Cg A,TTF-1,TG,CD34 and calcitonin were negative. The average Ki-67 indexes were 4. 3%. The histologic figures showed that tumor cells were arraged for papillary with branches. Fiberous axis often contained rich small blood vessels. Some cases had expanded thyroid follicular structures with varying sizes which contained eosinophilic staining gelatinous substance around scallop-like secretory vacuoles,and a few of follicules contained light blue staining mucoid. Some had cystic-wall-like structure which included papillary and glandular structure. Some showed glandular arrangement. Individual case showed nest and adenoid cystic stucture. Major were coated simple cuboidal epithelium,minority for tabular and or columnar. Cytoplasm was clear and or light red. Cell nucleus was oval with fine and smooth chromatin. Nucleolus was not obvious with small one for minority.The tumor tissue was easy to note fresh and old bleeding. About a half of patients had necrostic bone in tumors. The tumors destroyed surrounding bone in some cases. There were fiberous hyperplasia with hyaline degeneration with some histocytes and lymphocytes infiltration in some patients. Calcification and gravel body were seen in a proportion of cases. Main clinical manifestations were partial or total hearing loss for all of patients,followed by the performance of tinnitus,with different degrees of cranial nerve damage. 2 cases relapsed after one year and 7 years,respectively. Conclusion All of the patients show slow progress without death,and 2 cases relapse.