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伴有合体滋养层巨细胞的松果体生殖细胞瘤:一例全垂体功能减退和同性假性性早熟病例

Pineal germinoma with syncytiotrophoblastic giant cells: a case with panhypopituitarism and isosexual pseudopuberty.

作者信息

Laidler P, Pounder D J

出版信息

Hum Pathol. 1984 Mar;15(3):285-7. doi: 10.1016/s0046-8177(84)80192-6.

Abstract

A previously healthy boy, aged 12 years, developed hypopituitarism due to suprasellar metastases from a pineal germinoma with syncytiotrophoblastic giant cells (SGC). Despite the hypopituitarism, the patient showed pubertal development, which was associated with abnormal levels of human chorionic gonadotropin (hCG) in the cerebrospinal fluid. This case adds support to the theory that precocious puberty in children with pineal germinomas may be due to the secretion of hCG by SGC, a minor component of the germinoma. It also illustrates the point that, although abnormal levels of circulating hCG in children with pineal tumors may indicate a diagnosis of choriocarcinoma with its encumbent poor prognosis, other pineal germ cell tumors may also secrete hCG. Although this patient had abnormal levels of hCG, his cerebral tumor exhibited behavior more commonly associated with pineal germinoma than with choriocarcinoma.

摘要

一名既往健康的12岁男孩因松果体生殖细胞瘤伴合体滋养层巨细胞(SGC)发生鞍上转移而出现垂体功能减退。尽管存在垂体功能减退,但该患者仍出现了青春期发育,这与脑脊液中人绒毛膜促性腺激素(hCG)水平异常有关。该病例支持了以下理论:松果体生殖细胞瘤患儿的性早熟可能是由于生殖细胞瘤的次要成分SGC分泌hCG所致。它还说明了一点,即尽管松果体肿瘤患儿循环hCG水平异常可能提示绒毛膜癌的诊断及其预后不良,但其他松果体生殖细胞肿瘤也可能分泌hCG。尽管该患者hCG水平异常,但其脑肿瘤的行为更符合松果体生殖细胞瘤而非绒毛膜癌。

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