Liu Y C, Wei T C, Hsu Y H, Fang J S, Lee M L
Center for Medical Genetics, Tzu Chi General Hospital, Hualien, Taiwan.
J Formos Med Assoc. 1995 Sep;94(9):568-71.
Dysgenetic gonads are well known to be associated with gonadoblastoma and various germ cell tumors, but very rarely with choriocarcinoma. We describe a 16-year-old, phenotypic female patient with a 46XY karyotype who developed gonadoblastoma on the right gonad and choriocarcinoma on the left. There was no co-existence of the two tumor cell types in the same gonad. This case emphasizes the importance of bilateral gonadectomy for "46XY female" patients, as soon as the cytogenetic evidence is known.
发育不全的性腺与性腺母细胞瘤及各种生殖细胞肿瘤相关,这是众所周知的,但与绒毛膜癌的关联则非常罕见。我们描述了一名16岁的表型女性患者,其核型为46XY,右侧性腺发生了性腺母细胞瘤,左侧发生了绒毛膜癌。同一性腺中不存在两种肿瘤细胞类型。该病例强调了一旦得知细胞遗传学证据,对“46XY女性”患者进行双侧性腺切除术的重要性。