|Year : 2004 | Volume
| Issue : 2 | Page : 180-181
Seminoma arising in a true hermaphrodite with normal male external genitalia
Abraham Kurien1, Kim Mammen1, Sunitha Jacob2
1 Department of Urology, Christian Medical College and Hospital, Ludhiana, India
2 Department of Pathology, Christian Medical College and Hospital, Ludhiana, India
Department of Urology, Christian Medical College and Hospital, Ludhiana - 141 008
Keywords: True hermaphrodite, seminoma, male external genitalia, cryptorchidism.
|How to cite this article:|
Kurien A, Mammen K, Jacob S. Seminoma arising in a true hermaphrodite with normal male external genitalia. Indian J Urol 2004;20:180-1
| Case Report|| |
A 24-year-old phenotypic man with bilateral cryptorchidism presented with pain and mass in the right iliac fossa. On examination there was a right iliac fossa mass, an empty scrotum and a normal penis. Contrast enhanced CT scan confirmed a large retroperitoneal mass of 16 x 12 x 8 cm [Figure - 1]. No metastatic disease was detected. A true-cut needle biopsy demonstrated seminoma. His serum b-HCG and LDH were elevated while the serum AFP levels were normal. With four cycles of platinum based chemotherapy there was a remarkable reduction in size of the mass. His serum levels of b-HCG and LDH came down to normal levels. A further CECT showed a residual mass in the pelvis. Laparotomy and histopathology revealed a rudimentary uterus with a pair of tubes extending on to a pair of ovotestes with no residual tumour. The uterus along with the gonads was resected [Figure - 2]. Chromosomal analysis showed 46 XY karyotype. The follow up on him over two years has not revealed any recurrence.
| Comments|| |
The presence of ovarian and testicular tissue in an individual, whether as a single tissue type gonad or an ovotestis, constitutes true hermaphroditism a rare form of intersex. The reported incidence of 46 XY karyotype in a true hermaphrodite is 7% and the incidence of gonadal malignancy in a true hermaphrodite is 4.6%.  The Y chromosome is implicated in the incidence of germ cell tumours in these patients.  The risk may reflect the ectopic location of the gonads in these individuals. The completely normal male external genitalia except for the empty scrotum corresponded with a Luks type VI classification.  While genital ambiguity is the frequent finding in a true hermaphrodite, clinicians should suspect, although rare, the possibility of an intersex condition in a patient with bilateral cryptorchidism and normal external genitalia. To the authors' knowledge, this is the first reported case of seminoma occurring in a true hermaphrodite with normal male genitalia.
| References|| |
|1.||Krob G. Braun A. Kuhnle U. True hermaphroditism: geographical distribution, clinical findings, chromosomes and gonadal histology. Eur J Pediatr 1994; 153: 2-10. |
|2.||Manuel M. Katayama PK. Jones HW Jr. The age of occurrence of gonadal tumours in intersex patients with a Y chromosome. Am J Obstet Gynecol 1976; 124: 293-300. |
|3.||Luks Fl. Hansbrough F, Klotz DH Jr. Kottmeier PK. Tolete-Velcek F. Early gender assignment in true hermaphroditism. J Ped Sure 1988: 23: 1122-6. |
[Figure - 1], [Figure - 2]