卵巢高钙血症型小细胞癌临床病理分析

Clinicopathological features of small cell carcinoma of ovary hypercalcemic type

目的探讨卵巢高钙血症型小细胞癌(SCCOHT)的临床病理特征、诊断及鉴别诊断。方法回顾性分析我院2000-2018年以来收治的4例SCCOHT患者的临床资料、组织病理学形态及免疫表型,并复习相关文献。结果 SCCOHT好发于年轻女性,平均发病年龄为25. 5岁。患者多以腹痛、腹胀等腹部症状为主诉就诊。肿瘤多为单侧发生,多数患者术前有血清CA125及血钙的异常升高。镜下见肿瘤细胞以小细胞成分为主,细胞弥漫性生长,呈片状、巢状或条索状分布。细胞胞质少,核深染,核仁不明显。核分裂象约20~30个/10HPF。多数SCCOHT免疫组化示CK(AE1/AE3)、EMA、WT-1及CD99(+),Cg A、Syn、Calretinin及α-Inhibin(-)。结论 SCCOHT是一种临床罕见的高度恶性肿瘤,好发于年轻女性,需要与卵巢肺型小细胞癌、幼年型颗粒细胞瘤、淋巴瘤、生殖细胞肿瘤等鉴别。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of small cell carcinoma of ovary hypercalcemic type( SCCOHT). Methods A retrospective analysis was performed on the clinical data,histopathological and immunophenotype features in 4 cases of pathologically confirmed SCCOHT between 2000 and2018,and the related literatures were reviewed. Results The mean age of patients was 25. 5 years. The presenting symptoms included abdominal pain,distention and so on. Tumors were mostly unilateral. Most of the patients had abnormal elevated serum CA125 and blood calcium before operation. Microscopically,the tumor cells showed diffuse sheets or insular,trabecular and funicular distribution. Mitotic figures were about 20-30/HPF. Immunohistochemically,the tumor cells were positive for CK( AE1/AE3),EMA,WT-1 and CD99,but most commonly negative for Cg A,Syn,Calretinin and α-Inhibin. Conclusions SCCOHT is a rare and highly malignant tumor that usually occurs in young females. The differential diagnosis includes small cell carcinoma of pulmonary type,juvenile granulosa cell tumor,lymphoma,germ cell tumor and others.