Orchialgia secondary to polyorchidism: a case report

Introduction

Polyorchidism is a rare congenital condition defined by the presence of more than two testes. Although still quite rare, triorchidism, the most common form of polyorchidism, is defined as the presence of three testes (1). Although the exact etiology of polyorchidism is unclear, it is hypothesized that the one or more supernumerary testicles are a result of abnormal division of the genital ridge before the eighth week of intrauterine development (2). Most cases of polyorchidism were discovered incidentally during the evaluation and treatment of other conditions including inguinal hernia, cryptorchidism, testicular torsion, and orchialgia (2). In the majority of the reported cases of polyorchidism, the supernumerary testicles are found on the left side and present without symptoms (1). The current report describes an extremely rare case of orchialgia secondary to polyorchidism with quadorchidism in a 30-year-old man with three separate left testicles and a solitary right testicle. We present this case in accordance with the CARE reporting checklist (available at https://tau.amegroups.com/article/view/10.21037/tau-24-179/rc).

Case presentation

A 30-year-old man presented with a history of left chronic orchialgia and left testicular masses. The patient reported testicular trauma at 11 years of age, which resulted in pain and swelling. He has since felt three left testicular masses that he believed to be a result of “incorrect healing” and desired removal. Additionally, the patient does not have children, was not interested in future fertility potential, and in fact was interested in vasectomy for permanent sterilization.

Physical examination revealed a normal right testis, approximately 22 CC in volume, and a high riding left testis, approximately 10 CC in volume. Two 3.5-cm extratesticular masses were palpated on the left side, one superior to the testis extending from the caput of the epididymis and one inferior to the testis extending from the cauda of the epididymis. Scrotal ultrasound was recommended to further define the masses, but the patient declined due to cost as he was uninsured. It was agreed upon to proceed with a left inguinal orchiectomy abiding by oncologic principles as the masses were undefined, with a plan for placement of a left testicular prosthesis and a right vasectomy. Through a left inguinal incision with control of spermatic cord vessels, exploration revealed polyorchidism with three separate left testicles attached to two epididymides coming off of the left spermatic cord (Figure 1). Pathologic findings confirmed polyorchidism, with variable levels of atrophy of the testicular parenchyma and no evidence of malignancy (Figure 2). The patient had an uncomplicated recovery and has had complete resolution of orchialgia. The patient relayed a great deal of relief at his follow up that he had resolution of pain for the first time in a number of years and was pleased with the cosmesis and comfort of the testicular prosthesis.

Figure 1 Three separate left testicles attached to two epididymides with a common spermatic cord.

Figure 2 Gross pathologic appearance of the bivalved polyorchid specimen demonstrating the 3 testicles (labeled T1–T3) and the 2 separate epididymides (labeled E1 and E2).

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Polyorchidism is an extremely rare congenital urological condition that is typically an incidental finding in childhood or early adulthood. The exact etiology of polyorchidism is not defined, however; an abnormal division of the genital ridge prior to the eighth week of gestation is responsible for the development of one or more supernumerary testes (1). Bergholz et al. classified polyorchidism on an anatomical basis categorized by two major groups: type A: a supernumerary testis is connected to and drained by a single vas deferens and type B: is not drained by a vas (3). Type A and type B were also separated into the subgroups as follows: Type A1 supernumerary testis has its own epididymis and vas. Type A2 supernumerary testis has its own epididymis, but a shared vas deferens. Type A3 supernumerary testis shares an epididymis and vas deferens. Type B1 supernumerary testis has its own epididymis and no vas deferens. Type B2 supernumerary testis does not have an epididymis or vas deferens.

In the current case being reported, the patient presented with a rare combination of Type A1 and B2. The optimal management of polyorchidism remains ill-defined, as the differences in type and location of supernumerary testes yield a wide range of clinical presentations (4). Although the majority of cases present asymptomatically, a recent systematic review conducted in 2023 by Balawender et al. found that almost a third of patients with polyorchidism reported associated scrotal pain (5). Although a hypothesis of the etiology of scrotal pain due to polyorchidism has not been established previously, in this specific case the patient’s supernumerary testes were high riding and he described the pain as being consistent with a constant dull ache due to pressure of too much scrotal contents in a limited space with compression and worsening of pain with movement causing jostling such as when he ran. In the current case report, orchiectomy of the supernumerary testes completely resolved the patient’s scrotal pain.

Conclusions

Polyorchidism is a rare congenital condition and the literature related to the management of polyorchidism remains unclear. The current case report demonstrates the individualized treatment of the patient’s clinical presentation, with orchiectomy of the supernumerary testes in an extremely rare case of quadorchidism leading to complete resolution of the patient’s scrotal pain. However, counseling on fertility and testosterone deficiency is indicated prior to consideration of orchiectomy.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tau.amegroups.com/article/view/10.21037/tau-24-179/rc

Peer Review File: Available at https://tau.amegroups.com/article/view/10.21037/tau-24-179/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tau.amegroups.com/article/view/10.21037/tau-24-179/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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