Xanthogranulomatous epididymo-orchitis: A single-institutional case series and systematic review

Abstract

Introduction: Xanthogranulomatous inflammation is a rare nonneoplastic and chronic inflammatory process, characterized by proliferation of foamy macrophages resulting in damage and necrosis of the affected tissue. Involvement of the testis/epididymis by the disease is a rare event. Methods: A case series of four male patients diagnosed with xanthogranulomatous epididymitis/orchitis (XGEO) at our institute was reviewed. In addition, a systematic review of XGEO was carried out using PRISMA Guidelines 2020. Twenty-nine articles describing 38 patients of XGEO were included in the study. Results: XGEO usually has a subacute or chronic presentation and affects male individuals in the 5th or 6th decades of life. The disease is also known to occur in the pediatric age group. The patients present with swelling, tenderness, or pain in the scrotal region. Bilateral involvement has also been documented. Thirty patients were known to have one or more causal risk factors including diabetes mellitus (23.7%), spinal cord injury/neuropathic bladder (7.9%), prostatectomy (7.9%), trauma (4.1%), and transurethral resection of prostate procedure (4.1%). Complications observed were scrotal fistula, adhesions, and abscess formation. Radiological features reported are nonspecific and include heterogeneous echotexture, hypoechoic areas, and/or scrotal wall collections. Bacterial microorganisms isolated from the affected tissue demonstrated the presence of Escherichia coli, Pseudomonas aeruginosa, and Staphylococcus aureus. Histological subtypes of XGEO are diffuse and focal. In the diffuse subtype, which is more common, there is extensive parenchymal destruction by inflammatory process accompanied by widespread ischemic necrosis. Conclusion: The mainstay of treatment in XGEO cases is surgical excision preferably orchidectomy. Conservative management has been attempted in young individuals and in patients with focal XGEO, but there is limited supporting evidence. We present data of four cases along with detailed systematic review of the disease examining its clinicopathological behavior and associated risk factors followed by operative approach.