宫颈浸润性复层产黏液的癌8例临床病理学分析

Invasive stratified mucin-producing carcinoma:a clinicopathological analysis of eight cases

目的探讨宫颈浸润性复层产黏液的癌(ISMC)的临床病理学特点、诊断和鉴别诊断。方法回顾性分析8例ISMC的临床病理资料和免疫表型,并复习相关文献。结果 8例ISMC的患者年龄为32~68岁,平均年龄46岁,中位年龄43.5岁。2例因体检发现,4例以不规则阴道出血就诊,2例因绝经后阴道出血就诊。8例ISMC患者HPV检测均提示高危型感染。镜下组织病变与周围组织界限清楚,主要特点为实性巢团状,巢团内细胞镶嵌式或叠瓦状排列,其内可见散在分布、数量不等的产黏液细胞,周边细胞栅栏状排列。4例为ISMC伴子宫颈高度鳞状上皮内病变(HSIL),1例为ISMC伴微小浸润性鳞状细胞癌,1例为ISMC伴普通型腺癌,1例为ISMC伴腺鳞癌,1例为单纯性ISMC。8例ISMC均表达CK7、p16和CEA,CK5/6弥漫性表达或部分表达,p40散在表达或不表达,p63散在表达,不表达Pax-8、p53、ER和PR,Ki-67增殖指数均90%以上。腺癌的特殊染色过碘酸-雪夫染色(PAS染色)阳性。8例均行子宫颈活检,1例行子宫颈锥切,7例行全子宫+双附件切除。随访后,均未复发。结论 ISMC是新近发现的一种少见的宫颈浸润性黏液性腺癌,与人类乳头状病毒(HPV)感染高度相关,组织学特征独特,可独立发生或伴发于其他类型宫颈癌,临床预后好于胃型黏液腺癌,差于普通型腺癌。需与腺鳞癌、腺样囊性癌/基底细胞癌、黏液表皮样癌、伴有少许黏液细胞的鳞状细胞癌相鉴别。

Objective To investigate the clinical pathological characteristics, diagnosis and differential diagnosis of invasive stratified mucin-producing carcinoma(ISMC). Methods The clinical pathological data and immune phenotype were retrospectively analyzed in 8 cases of ISMC, with review of the relevant literature. Results Eight cases of ISMC patients aged from 32 to 68 years, with an average of 46 years and median age 43.5 years. 2 cases for medical examination found that 4 cases presented with irregular vaginal bleeding, 2 case with postmenopausal vaginal bleeding. 8 patients with ISMC high risk type HPV testing suggested infection. Pathological changes showed the tumors had clear boundaries with the surrounding tissue. The main characteristics were solid or nest patterns, a nest of cells with mosaic or palisade arrangement. 3 cases of ISMC had high-grade cervical squamous intraepithelial lesion(HSIL), 1 case of ISMC presented with tiny invasive squamous cell carcinoma, 1 case with ordinary adenocarcinoma, 1 case with adenosquamous carcinoma and 1 case with simple ISMC. 8 cases of ISMC expressed CK7, p16 and CEA. CK5/6, p63 and p40 were diffuse or partially positive but Pax-8, p53, ER and PR were negative. Ki-67 proliferation index was more than 90%. PAS stain was postive. Cervical biopsy was performed in 8 cases, routine cervical cone cutting in 1 case, and uteroovarian resection in 7 cases. Follow-up showed no relapse in all the cases. Conclusion ISMC is a newly discovered and rare cervical invasive mucinous adenocarcinoma, highly associated with the human papilloma virus(HPV) infection. Histologic features are unique. ISMC occurrs independently or with other types of cervical cancer. Clinical outcomes is favouble in gastric mucous adenocarcinoma, but worse than ordinary adenocarcinoma. Differential diagnosis includes squamous cell carcinomas, adenoid cystic carcinoma/basal cell carcinoma, mucoepidermoid carcinoma and squamous cell carcinoma with a little mucous production.