原发性卵巢高钙血症型小细胞癌伴肾功能不全临床病理分析

Ovarian small cell carcinoma hypercalcemic type with acute renal dysfunction

目的探讨卵巢高钙血症型小细胞癌(ovarian small cell carcinoma hypercalcemic type,OSCCHT)伴急性肾功能不全的临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例OSCCHT伴急性肾脏功能不全患者的临床资料、组织学形态、免疫表型、超微结构等,并复习相关文献。结果患者女性,29岁。因无明显诱因出现食欲不振伴明显消瘦1个月余。实验室检查示肾功能异常;影像学示盆腔肿块,考虑附件来源。血液透析肾功能部分恢复后行手术。右侧卵巢见一12 cm×10 cm×10 cm大小肿瘤,镜下见瘤细胞呈弥漫实性分布,被纤维条索分割成多结节状,部分细胞间可见小滤泡样、假腺样结构,腔内可见嗜酸性分泌物,瘤细胞中等偏大,胞质嗜酸性,核圆形、卵圆形,核呈空泡状,核质比高,核分裂象多见。免疫表型:癌细胞EMA和CK、C-erb B-2均(+),CA125(灶+),ER、PR、Syn、Cg A、PTH、Inhibin、CD99、AFP、PLAP、CD30和CD20均(-),Ki-67增殖指数约60%。结论 OSCCHT临床少见,同时伴急性肾脏功能不全更为罕见。OSCCHT是一种进展迅速、高度恶性的卵巢恶性肿瘤,确诊主要根据其临床特点(血钙升高等)、病理学特征及免疫表型,必要时可结合电镜和分子生物学检测辅助诊断。

Purpose To investigate the clinicopathlogical characteristics, diagnosis and differential diagnosis of ovarian small cell car-cinoma hypercalcemic type （ OSCCHT） associated with acute renal dysfunction. Methods A case of OSCCHT associated with acute renal dysfunction was reported. The clinical and pathologic data, treatment and pathological examinations were analyzed and the related literatures were reviewed. Results A 29-year-olds women was presented to hospital with inappetence and significant weight loss for 2 months. The laboratory examination showed abnormal renal function, and pelvic cavity mass, possibly coming from adnexa of the uterus was seen by radiography. Serum levels showed significantly increased serum calcium with acute renal dysfunction. An emergency opera-tion was performed just after renal function partially recovered by 2 times hemodialysis. During surgery, right ovary tumor with a size of 12 cm × 10 cm × 10 cm was inspected. Microscopically, the tumor cells were arranged in a diffuse solid pattern, mutiple nodules were separated by fibrous tissue. some small folliculars and pseudoglandular cavities with acidophilia secretion within nodules could been ob-served. Tumor cells were medium to large with eosinophilic cytoplasm, round or oval vesicular nucleus, increased karyoplasmic ratio and pathologic mitosis. Immunohistochemistry revealed that the tumor cells expressed EMA, CKpan, C-erbB-2 （ ＋） , CA125 （ focal＋） and Ki-67 proliferation index was about 60%, while ER, PR, Syn, CgA, PTH, Inhibin, CD99, AFP, PLAP, CD30 and CD20 were not expressed. Conclusion Ovarian small cell carcinoma hypercalcemic type with acute renal dysfunction is a very rare with a rapidly progressive and highly malignant tumor. The final diagnosis mainly lies on clinical information （ hypercalcaemia） , morphology and immunohistochemistry, combined with electron microscopy and molecular biological detection when necessary.