Background: Swyer syndrome (46XY complete gonadal dysgenesis) is an uncommonly encountered condition in our population. Gonadectomy is recommended upon diagnosis due to a significant risk of malignant transformation of the dysgenetic gonads, typically to dysgerminoma.
Cases: We present 3 cases of women who underwent gonadectomy following a diagnosis of Swyer syndrome. Two of these patients had dysgerminoma confirmed on histopathology. In particular we discuss the macroscopic appearance of the affected gonads and the further management of each case.
Summary and conclusion: Individuals with Swyer syndrome require gonadectomy upon diagnosis of their condition, as part of their multidisciplinary management. For treatment of early stage dysgerminoma, surgical resection of the involved gonad and fallopian tube is curative, again highlighting the need for early intervention.
Keywords: 46 XY; Dysgerminoma; Gonadal dysgenesis; Gonadoblastoma.
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