Primary Epididymal Adenocarcinoma: A Case with Extended Follow-Up and Literature Review

Introduction: Primary malignant tumors of the epididymis are extremely rare and are associated with a poor prognosis. Since the first description by Scholl in 1928, fewer than 100 cases have been reported [JAMA. 1928;91(8):560–564]. Because presentations are nonspecific and misdiagnosis is common, clinical suspicion remains essential. Standardized diagnostic and therapeutic protocols are lacking, and there is no systematic evaluation of available treatment modalities. Case Presentation: This report describes a 52-year-old man who presented with left testicular swelling and pain. Imaging initially suggested epididymitis. He underwent radical orchiectomy, and histopathology confirmed primary epididymal adenocarcinoma (PEA). Pathological staging was pT2 N1 M0, S0 [20,21]. A subsequent laparoscopic retroperitoneal lymph node dissection (RPLND) identified metastasis in 1 of 10 nodes. The patient declined adjuvant chemotherapy; therefore, postoperative radiotherapy (50 Gy in 25 fractions to the pelvic and para-aortic regions) was administered. The patient has remained disease-free for 10 years of follow-up, indicating a clinical cure. Conclusion: This case represents one of the longest reported follow-ups for node-positive PEA. It highlights that an aggressive multimodal strategy – integrating radical orchiectomy, RPLND for pathologic staging and potential therapeutic benefit, and adjuvant radiotherapy when chemotherapy is declined – can be curative. This long-term survival outcome provides a management framework for this orphan disease, though validation in larger cohorts is needed.