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J Gynecol Obstet Hum Reprod ; 48(4): 283-286, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30690088

RESUMO

Swyer syndrome also known as pure or complete gonadal dysgenesis is a very rare disorder of sex development wherein the individuals are phenotypically females with 46, XY genotype and preserved mullerian structures. These individuals characteristically have dysgenetic streak gonads which carry an increased risk of malignant transformation. Prophylactic gonadectomy is highly recommended as soon as a clinical diagnosis is established to diminish the chances of tumor development. We present a case of complete gonadal dysgenesis with bilateral small gonads with a dysgerminoma arising in a background of gonadoblastoma in one gonad and immature teratoma in the other. The present case, besides adding a rare case to the literature, highlights the importance of detailed pre-operative assessment of gonadal size and prompt prophylactic gonadectomy in cases with gonadal dysgenesis.


Assuntos
Castração , Disgenesia Gonadal 46 XY/patologia , Disgenesia Gonadal 46 XY/cirurgia , Ovário/patologia , Adulto , Disgerminoma/patologia , Disgerminoma/cirurgia , Feminino , Gonadoblastoma/patologia , Gonadoblastoma/cirurgia , Humanos , Masculino , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Procedimentos Cirúrgicos Profiláticos , Teratoma/patologia , Teratoma/cirurgia
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