PERSISTENT MULLERIAN DUCT SYNDROME WITH NON SEMINOMATOUS GERM CELL TUMOR A RARE PRESENTATION

INTRODUCTION The persistent Mullerian duct syndrome is a rare disorder of male sexual development, characterized by the presence of the uterus, fallopian tubes and upper vagina in otherwise normally virilized males1. Case history A 30 year old father of two children presented to our department with abdominal pain and distension of a fortnight duration. Examination revealed moderate abdominal distension with a tender firm hypogastric, right iliac and lumbar mass of size 15x12 cm. Right scrotum was empty whereas left testis and cord structures were normal. Per rectal examination revealed the lower extent of mass felt through the anterior rectal wall. Serum AFP was 13903 ngml with LDH of 757 UL and Beta HcG- 7.17 mIU. We performed Tru-cut biopsy of the mass which revealed Germcell tumour (with Yolk Sac element) of the testes. Due to the advanced stage (Stage IIIC T4N0M0S3) chemotherapy with four cycles of BEP was given. Post chemotherapy levels of serum AFP came down.Diagnostic laparoscopy revealed residual mass with female reproductive internal organs. A formal laparotomy was performed which revealed a 6x3 cm residual mass present with uterus, fallopian tube and no enlarged nodes. Histopathology revealed the female organs and no evidence of malignancy.Discussion PMDS is a uncommon cause of intersex in males. The malignant transformation rate is about 15 percent with seminoma being the commonest. Very few reports of yolk sac tumors can be quoted from literature. Management is based on the same protocols for testicular malignancies. Urologists need to be aware of this syndrome in cryptorchidism and childhood hernia in order to avoid future problems of infertility and malignant transformation.