|
|
CASE REPORT |
|
|
|
| Year : 2002 | Volume
: 18
| Issue : 2 | Page : 181-183 |
| |
Carcinoma in third testis in a case of polyorchidism and persistent mullerian structure syndrome : A case report and review of literature
S Kumar, R Tandon, AN Srivastava, D Dalela
Departments of Surgical Oncology, Pathology and Urology, K. G. Medical College, Lucknow, India
Correspondence Address:
A N Srivastava
Department of Pathology, K.G. Medical College, Lucknow
India
 Source of Support: None, Conflict of Interest: None  | Check |
 Abstract | | |
Polyorchidism is a rare but distinct genital anomaly in which supernumerary testes are present usually within the scrotum. A unique case of germ cell tumour arising in one of the two cryptorchid abdominal testes with persistent mullerian structure syndrome is presented. He had single testicle in the scrotum, inguinal hernia and a single fallopian tube on exploration. This case is being reported for its extreme rarity and clinical issues involved.
Keywords: Polyorchidism; Persistent Mullerian Duct
How to cite this article:
Kumar S, Tandon R, Srivastava A N, Dalela D. Carcinoma in third testis in a case of polyorchidism and persistent mullerian structure syndrome : A case report and review of literature. Indian J Urol 2002;18:181-3 |
How to cite this URL:
Kumar S, Tandon R, Srivastava A N, Dalela D. Carcinoma in third testis in a case of polyorchidism and persistent mullerian structure syndrome : A case report and review of literature. Indian J Urol [serial online] 2002 [cited 2023 Mar 25];18:181-3. Available from: https://www.indianjurol.com/text.asp?2002/18/2/181/37639 |
| Case Report | |  |
A 24-year-old male presented to us with large left lower abdominal mass. On examination there was hard hypogastric retroperitoneal lump of about 20 x 18 cm. He had fully developed secondary sexual characteristics and a normal circumcised phallus. The third testis was small and located in well-developed ipsilateral scrotal sac [Figure - 1]. On the right side, however, the testis was absent and scrotum was underdeveloped. The abdominal scans and serum markers (AFP-1800 iu/ml & HCG-680 ng/ml) were suggestive of Non-seminomatous Germ Cell Tumour of testis. The subsequent FNAC from abdominal mass was suggestive of NSGCT. 3 courses of combination chemotherapy (Cisplat + Etopo + Bleo) were given and after that exploration was done which revealed a cryptorchid smaller but morphologically normal testis on right side. On the left side, there was another testicle from which the tumour was arising. There was an unicornuate uterus having rightsided Fallopian tube More Details which was herniating in the left inguinal hernial sac. The malignant mass, right cryptorchid testis and uterus alongwith the fallopian tube were resected. Left inguinal hernia was repaired. The histopathological examination revealed post-chemotherapeutic extensive necrosis and degeneration in germ cell tumour in cryptorchid abdominal testis with rudimentary uterus with one fallopian tube [Figure - 2],[Figure - 3],[Figure - 4].
| Comments | | |
Preoperative diagnosis of polyorchidism is difficult because of its extreme rarity. [1],[2] The scrotum is the location of supernumerary testis in 75% of the reported cases. Other sites include the inguinal region (20%) and retroperitoneum. [6] Various abnormalities and malformations are known to be associated with polyorchidism and include maldescent (15-23%) either of supernumerary testicle or ipsilateral normal testicle, hernia (23-30%), torsion (13%) and hydrocoele (9%). [2],[7] Despite the high association of maldescent in polyorchidism, the incidence of malignancy is low. [2] A review of literature shows that the first reported case of teratoma in a supernumerary testicle was reported by D'Oplando. [8] Subsequently others have reported seminoma and teratoma in one case each. [9],[11] Because the incidence of malignancy is low prophylactic orchiectomy is deemed unnecessary. [2] However, exploration of scrotal mass is mandatory, especially when there is suspicion of tumour. [2],[11]
Our case is truly extra-ordinary, in that not only did this patient manifest with the rare extra-scrotal variant of polyorchidism, which itself is an uncommon disorder, but developed tumour in the supernumerary testicle with Persistent Mullerian Duct Syndrome with single right-sided fallopian tube. The case also proves the point that a supernumerary gonad in the abdomen is also at risk of developing malignancy. The behaviour of malignancy in such a testis follows the same pattern as that of other abdominal testes.[12]
| References | | |
| 1. | Mehan DJ, Chehval MJ, Ullah S. Polyorchidism. J Urol 1976; 116: 530-532.  |
| 2. | Pelander WM. Luna G, Lilly JR. Polyorchidism : Case report and literature review. J Urol 1978; 119: 705-706. |
| 3. | Renton CJC. A case of polyorchidism with intersex. J Urol 1975:113: 720-724. |
| 4. | Corbi P. Houlgatte A, Auberget JL, Rodier J, Timbal Y. A rare diagnosis of an intrascrotal tumor : Polyorchidism. Ann Urol (Paris) 1988;22:140. |
| 5. | Yashida T. Yabumoto H, Shima H et al. Polyorchidism : Pre-operative diagnosis by ultrasonography. Urol Int 1989; 44: 47. |
| 6. | Burgers JK, Gearhart JP. Abdominal polyorchidism : An unusual variant. J Urol 1988: 140: 582-583. |
| 7. | Thum G. Polyorchidism: Case report and review of literature. J Urol 1991; 145: 370-372. |
| 8. | D'Oplando C. cited by Parks TG. Chromosome studies in polyorchidism. Br J Surg 1967; 54: 113. |
| 9. | Grechi G. Zampi C, Selli C, Carini M. Ucci M. Polyorchidism and seminoma in a child. J Urol 1980: 1. |
| 10. | Scott KWM. A case of Polyorchidism with testicular teratoma. I Urol 1980: 124: 930-931. |
| 11. | Gonchar MA. A germ cell tumour of an ectopic testis in polyorchidism. Klin K 1989; 5: 64. |
| 12. | Kulkarni IN. Kamat MR, Borges AM. Bilateral synchronous tumors in testes in unrecognised mixed gonadal dysgenesis: A case report and review of literature. J Urol 1990: 143: 362-364. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
[Table - 1]
|
