Cystic dysplasia of the testis is a rare congenital defect that results in the formation of numerous irregularly shaped cystic spaces in the mediastinum testis. The clinical presentation of such a disorder usually occurs in the paediatric age group as an asymptomatic scrotal swelling that can mimic testicular tumours. The case presented in this paper is the first identified and diagnosed case of cystic dysplasia in Kashmir, India.
A 2‐year‐old boy presented with a history of scrotal swelling since birth and was admitted to the Department of Surgery, Shri Mahraja Hari Sirgh Hospital, Srinagar, Kashmir, associated with Government Medical College, Srinagar, Kashmir, India.
On examination, the scrotum showed an enlarged right testis with a cystic feel. A trans‐illumination test was positive. Scrotal ultrasound also showed cystic spaces in the testis. The testis was 7 × 3 cm with all three coverings and had a cystic feel, with small cystic spaces ranging in size from 0.2 to 1.5 cm on the cut section, affecting the whole testicular mass. Microscopic examination showed that the cysts were lined by low cuboidal flattened epithelium resembling the epithelium of the rete testis, separated from each other by fibrous septae with a complete replacement of normal testicular tissue.
Cystic dysplasia of the rete testis is a rare abnormality often associated with ipsilateral agenesis of the kidney. This malformation is due to a developmental defect of the mesonephric duct, which is the cause of both the dilatation of testicular rete testis and renal agenesis.1 It is characterised by multiple irregular cystic spaces in the mediastinum of the testis that may affect the whole gonad and is associated with renal malformations (agenesis or cystic dysplasia2).
Embryologically, when the duct system of the testis is formed, seminiferous tubules anastomose with one another to form the rete testis. The rete testis in turn establishes contact with persisting mesonephric tubules, which form vasa‐efferentia, the cranial part of the mesonephric duct becomes highly coiled on itself to form the epididymis and the distal part becomes the vas deferens. The seminal vesicle arises on either side of the mesonephric duct as a diverticulum. The part of the mesonephric duct between the opening into the prostatic urethra and the origin of the diverticulum forms the ejaculatory duct.3
A defect in the connection between efferent ductules derived from the mesonephric epithelium and the rete testis and seminiferous tubules seems to be the most likely explanation for the pathogenesis of cystic dysplasia of the testis.3,4
In our patient, teratoma was clinically suspected and orchidectomy was carried out on the right side. The histopathological diagnosis was cystic dysplasia of the testis, but evaluation of the patient for other urogenital anomalies was not possible as the patient did not come for follow‐up.
Today, the non‐operative management of cystic dysplasia of the testis represents an effective alternative option in these patients and the primary benefit of this approach is the preservation of endocrine function and spermiogenic activity. The natural history of untreated cystic dysplasia of the testis and its effect on normal testicular tissue are, however, still unknown. Therefore, a long‐term follow‐up is recommended,5 along with evaluation for associated urological anomalies.
References
- 1.Cimador M, Rosone G, Castagnetti M.et al Cystic dysplasia of rete testis associated with ipsilateral renal agenesis. Case Rep Minerva Pediatr 200355175–179. [PubMed] [Google Scholar]
- 2.Camassei F D, Francalanci P, Ferro F.et al Cystic dysplasia of the rete testis: report of two cases and review of the literature. Pediatr Dev Pathol 20025206–210. [DOI] [PubMed] [Google Scholar]
- 3.Glantz L, Hansen K, Caldamone A.et al Cystic dysplasia of the testis. Hum Pathol 1993241142–1145. [DOI] [PubMed] [Google Scholar]
- 4.Nistal M, Regadera J, Paniagua R. Cystic dysplasia of the testis. Light and electron microscopic study of three cases. Arch Pathol Lab Med 1984108579–583. [PubMed] [Google Scholar]
- 5.Rovellini P, Bianchi A, Bottanelli A.et al Cystic dysplasia of the testis: report of a case and review of the literature. Arch Ital Urol Androl 200173193–198. [PubMed] [Google Scholar]