Indian Journal of Urology
: 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


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.

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;18:181-183

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 2020 Oct 22 ];18:181-183
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 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 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].


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]


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