Riadh Ben Temime
Charles Nicolle University Hospital, Tunisia
Tunis El Manar University, Tunisia
Posters & Accepted Abstracts: J Cancer Sci Ther
46 XY pure gonadal dysgenesis known as Swyer syndrome is a disorder of sexual differentiation characterized by the association of a female phenotype and a 46 XY karyotype with fibrous gonads. It is generally revealed by a primary amenorrhea. This syndrome represents a ground of predilection for the development of germ cell tumors. We present the clinical, sonographic and endocrine findings in 10 cases of Swyer syndrome managed in Charles Nicolle University Hospital Tunis, Tunisia. We study the risks of malignant transformation and analyze the histopathological findings of the tumors arising on these dysgenesis gonads. The treatment options and the follow up are discussed through this largest series in the world. A bilateral gonadectomy was decided by laparoscopy in nine cases and by laparotomy for adnexal torsion of a 20 cm tumor in one case. Histopathology showed: fibrous gonads in five cases, a bilateral gonadoblastoma in three cases and a dysgerminoma associated to a gonadoblastoma in two cases. The presence of Y chromosome in the karyotype of a patient presenting a gonadal dysgenesis must lead to prophylactic bilateral gonadectomy in order to avoid a malignant transformation. Gonadectomy must be followed by a hormone replacement therapy. A screening of similar cases in sisters is imperative.
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