Bilateral polyorchidism with ipsilateral two undescended testes: a rare congenital anomaly

Polyorchidism is defined as the presence of more than two testes. This rare congenital anomaly has been reported with only 140 pathologically proven cases in the published literature to date. While triorchidism is the most common variation and generally affects the left side, bilateral polyorchidism is even rare and only seven cases of patients with four testes have been reported in the literature. There is no consensus in the literature regarding the management of supernumerary testis due to its rareness. We report such a rare case of a 20-year-old male patient, who was presented with left-sided scrotal mass and right inguinal swelling and, diagnosed as polyorchidism including four distinct testes, with two of them in left hemiscrotum and the other two testes in the right inguinal canal. The patient underwent orchiopexy for the normal looking right-sided testis, and orchiectomy for the right-sided supernumerary testis being dysmorphic and potential malignancy risk. Histopathological examination confirmed the excised tissue to be severely atrophic testicle. The patient is still following with regular self-examination and scrotal ultrasonography. Polyorchidism should be keep in mind especially for the differential diagnosis of extratesticular and paratesticular masses. Physical examination may not be sufficient, and radiologic examination can provide accurate diagnosis. Conservative, extirpative or reconstructive approaches could be performed based on individual basis by reproductive potential and location of supernumerary testis, coexistence of other disorders and suspicion of malignancy.