皮肤原发分泌性癌临床病理分析及文献复习

Clinicopathological features of secretory carcinoma of skin and review of the literatures

目的探讨皮肤原发分泌性癌(PSCS)的临床病理特点、免疫表型及预后。方法收集2例PSCS的临床病理资料及随访资料,光镜下观察HE切片,采用免疫组化、FISH技术检测ETV6基因,并复习相关文献。结果 2例均为男性,年龄30~52岁,平均41岁,共4个标本,均为无痛性肿块。例1首发于右下睑,术后2个月复发,术后6个月及11个月分别出现右腮腺及右下颌肿块;例2发生于左侧胸壁。4个标本肿瘤直径0.5~2.1 cm,边界清或欠清,无包膜。镜下肿瘤细胞排列成微囊/囊状、管状及实性结构,细胞巢之间被玻璃样变的纤维组织分隔,呈分叶状生长;细胞淡染,腔内含特征性抗淀粉酶的嗜酸性分泌物。其中例1的下睑原发标本可见脉管侵犯。免疫表型示肿瘤细胞均弥漫表达mammaglobin、vimentin、S-100及CK7,Ki-67增殖指数5%~15%。其中3个标本(例1的下睑原发和腮腺转移标本,例2标本)行FISH检测到ETV6基因重排。随访4~44个月,例1术后出现复发及转移。结论 PSCS属于临床经过相对惰性、预后较好的罕见肿瘤,但极少数病例也可复发及转移。其具有与乳腺及涎腺分泌性癌相似的病理学形态、免疫表型及分子遗传学特征,ETV6基因重排有助于明确诊断。

Objective To investigate the clinicalpathological features, immunophenotype and prognosis of primary secretory carcinoma of the skin(PSCS). Methods Clinical and follow-up data of 2 cases of PSCS were collected. HE staining, immunohistochemical staining(IHC) and fluorescence in situ hybridization(FISH) were used to observe the histopathological features of tumors. With review of the relevant literatures. Results Two patients were male, aged from 30 to 52 years, with a mean age of 41 years. There were 4 specimens in 2 patients, all of which were painless.Case 1 first appeared on the right lower eyelid and recurred 2 months after operation. After 6 months and 11 months, there were masses in right parotid gland and right mandible respectively. Case 2 occurred in the left chest wall. The tumor diameter of 4 specimens ranged from 0.5 cm to 2.1 cm, with clear or unclear boundary and no capsule. Histopathologically, tumor cells were arranged in tubular and solid structures. Cell nests were separated by fibrous tissue, growing in a lobulated manner. The tumor cells were light stained and contained eosinophilic secretions with characteristic anti-amylase in the cavity. Vascular invasion could be found in the primary specimen of the lower eyelid of case 1. Immunohistochemically, all tumors showed strong positivity for mammaglobin, vimentin, S-100 and CK7. The Ki-67 proliferation rate was about 5%~15%.The rearrangement of ETV6 gene was detected by FISH in 3 specimens(primary lower eyelid and parotid gland in case 1 and case 2). During the follow-up of 4-44 months, recurrence and metastasis occurred in case 1 after operation. Conclusion PSCS is a rare tumor with relatively indolent clinical course and good prognosis, but very few cases can also recur and metastasize. It is similar to secretory carcinoma of the breast and salivary glands in pathological morphology, immunophenotype and molecular genetic characteristics. ETV6 gene rearrangement is helpful for the definite diagnosis.