卵巢伴环状小管性索肿瘤4例临床病理分析

Ovary sex cord tumor with annular tubules:a clinical and pathologic analysis

目的探讨卵巢伴环状小管性索肿瘤的临床病理特征及鉴别诊断要点。方法对4例卵巢伴环状小管性索肿瘤进行临床病理、免疫组化及特殊染色研究。结果4例患者中1例伴发黏膜黑斑-息肉综合征(PJS),其余3例中2例发生肿瘤复发转移。临床上表现为腹部不适、腹部肿物、月经不规律、不孕等;肿物大体标本均具有黄色、质地细腻的特点;镜下均可见特征性的环状小管结构,小管中心为PAS(+)的基底膜样物质;免疫组化:inhibin、CD99v、imentin和Melan-A(+),EMA(-),CK局灶(+)。结论卵巢伴环状小管性索肿瘤少见,易误诊,主要依据其典型的镜下形态并结合免疫组化做出诊断。

Objective To study the clinicopathologic characteristics and differential diagnosis of ovary sex cord tumor with annular tubules （SCTAT）. Methods Four cases of SCTAT were studied on their histopathologic and histochemical characteristics. Results One of the 4 cases was associated with Peutz-Jegher syndrome （PJS）. Two of the 3 cases without PJS relapsed. Clinical manifestation was characterised by an abdominal mass, dysmenorrhea and sterility; gross appearance showed that the tumor was yellow with dedicated texture; examination under microscope showed single or complex annular tubules with PAS-positive basement membrane-like material around the tubules; tumor cells were positive for inhibin, CD99, vimentin, melan A and CK locally, but negative for EMA. Conclusions SCTAT is rare and easily misdiagnosed. Its diagnosis mainly bases on the characteristics of histopathology and immunohistochemistry.