Abstract

EMBRYONIC CARCINOMA REVEALING FEMINIZING TESTIS SYNDROME: A CASE REPORT

Dr. G. Benjallloun, Dr. M. Sefrioui, *Dr. M. Mourabbih, Dr. M. Charkaoui, Pr. H. Bouffetal, Pr. S. Mahdaoui and Pr. N. Samouh

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Abstract

Introduction: The feminizing testis (TF) is the most complete form of androgynoid pseudohermaphrodisms.[1] Observation: our patient is a 28 year old girl from a first degree consanguineous marriage. As a surgical history, the patient is operated on for inguinal hernia. At around 13–14 years of age, breast development began, but sexual hair did not appear and the patient remained amenorrheic, leading her to see an endocrinologist. On clinical examination, breast development was normal, puboaxillary hair was almost absent, the external genitalia were unambiguously female, with a rectal cul-de-sac 2 cm deep. Presence of an abdomino-pelvic mass extending beyond the umbilicus. The hormonal balance revealed a very high plasma testosterone concentration. The karyotype was male type 46 XY. Discussion: The peripheral androgenic effect involves a defect in the development of the Wolffian ducts: the seminal vesicles and the prostate are generally absent, while a poorly developed epididymis is found in 9% of cases.[2,3] Wolffian or Müllerian cysts have been reported and are sometimes so dilated that they form pelvic cystic masses.[3,5] Histologically, TF is formed of immature seminiferous tubules containing rare germ cells and bordered only by Sertoli cells.[3,4] The number of germ cells can be normal in children under five and tends to decrease or even cancel out with age, the residual germ cells often have an abnormal morphological appearance with sometimes lesions of seminoma in situ.[7]

Keywords: As a surgical history, the patient is operated on for inguinal hernia.