Malignant Germ Cell Tumors and Their Precursor Gonadal Lesions in Patients with XY-DSD: A Case Series and Review of the Literature

Sahra Steinmacher, Sara Y. Brucker, Andrina Kölle, Bernhard Krämer, Dorit Schöller and Katharina Rall
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Sahra Steinmacher: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany
Sara Y. Brucker: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany
Andrina Kölle: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany
Bernhard Krämer: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany
Dorit Schöller: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany
Katharina Rall: Department of Women’s Health, Tübingen University Hospital, Calwerstr. 7, 72076 Tübingen, Germany

IJERPH, 2021, vol. 18, issue 11, 1-12

Abstract: The risk of gonadal germ cell tumors is increased over the lifetime of patients with XY-disorders of sex development (XY-DSD). The aim of this study was to evaluate clinical features and histopathological outcome after gonadectomy in patients with XY-DSD to assess the risk of malignant transformation to gonadal germ cell tumors. Thirty-five women treated for XY-DSD at our hospital between 2003 and 2020 were enrolled in this study. Twenty-seven (77%) underwent prophylactic gonadectomy, 10 (29%) at our department and 17 (48%) at external hospitals. Eight (23%) patients didn’t receive gonadectomy. Of the patients who underwent a surgical procedure at our hospital, two patients were diagnosed with a unilateral seminoma, one patient with a bilateral and one patient with a unilateral Sertoli cell adenoma. According to these findings, preventive gonadectomy in patients with XY-DSD should be taken into consideration. Guidelines concerning the necessity of gonadectomy to avoid malignant transformation are still lacking. The risk of malignant germ cell tumors from rudimentary gonads has not been investigated sufficiently to date, as it is mostly based on case series due to the rarity of the condition. In our study we retrospectively analyzed patients who partly underwent bilateral gonadectomy, aiming to fill this gap. Concerning the ideal point of time for gonadectomy, further studies with a higher number of patients are needed.

Keywords: XY-DSD; gonadectomy; malignant transformation (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2021
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