摘要
目的 探讨幼年性粒层细胞瘤和Maffucci综合征的临床病理特征及两者伴发的原因。方法 对 1例伴Maffucci综合征的幼年性粒层细胞瘤进行HE、组织化学及免疫组织化学染色观察 ,并复习文献。结果 幼年性粒层细胞瘤多发生于 2 0岁以下患者 ,儿童患者常表现为青春期前假性性早熟 ,生育期妇女常表现为月经紊乱。组织学上具特征性滤泡结构 ,丰富的嗜酸性或空泡化胞质 ,无核沟及Call Exner小体 ,核分裂象多见。Inhibin免疫组化染色有助于诊断和鉴别诊断。Maffucci综合征为先天性软骨发育异常 ,易合并恶性肿瘤。 14例卵巢幼年性粒层细胞瘤伴Maffucci综合征或Ollier病中卵巢肿瘤和骨病变有同侧分布倾向。
Purpose To investigate the clinicopathological features of juvenile granulosa cell tumor (JGCT) and Maffuccis syndrome (MS) and the possible common cause of both disorders. Methods A case of ovarian JGCT with MS was studied by HE staining and histochemical, immunohistochemical techniques, and the literatures on JGCT and MS were reviewed. Results JGCT occurred predominantly before the age of 20 years, and premenarchal patients usually manifested signs of precocious pseudopuberty, postmenarchal patients usually presented menstrual abnormalities. Histologically JGCT had characteristic follicular pattern, abundant eosinophilic or vacuolated cytoplasm, grooved nuclei or Call Exner bodies, and mitotic figures were frequent. Inhibin immunostaining could be used to help the diagnosis and differential diagnosis of JGCT. MS was a congenital dysplastic disease of cartilage, and the emergence of malignant neoplasms was a well recognized complication in MS. In the reported 14 cases of JGCT with MS or OD, the ovarian tumors originated from the same side as the pronounced skeletal abnormalities. Conclusion The association of JGCT with MS is more than mere coincidence and indicates generalized mesodermal dysplasia.
出处
《临床与实验病理学杂志》
CAS
CSCD
2003年第4期364-367,共4页
Chinese Journal of Clinical and Experimental Pathology
