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CASE REPORT |
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| Year : 2004 | Volume
: 20
| Issue : 2 | Page : 180-181 |
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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
Correspondence Address:
Kim Mammen
Department of Urology, Christian Medical College and Hospital, Ludhiana - 141 008
India
 Source of Support: None, Conflict of Interest: None  | Check |
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%. [1] The Y chromosome is implicated in the incidence of germ cell tumours in these patients. [2] 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. [3] 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]
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