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

Abstract

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.