Lymphoma presenting as a genitourinary lesion in children: A case series highlighting diagnostic challenges and the risk of unnecessary organ resection.

BACKGROUND: Pediatric lymphoma rarely arises from or initially presents as a solitary urogenital mass. Because imaging features often resemble those of primary genitourinary malignancies, misdiagnosis may lead to unnecessary radical organ resection. OBJECTIVE: To describe the clinical features, diagnostic challenges, and treatment outcomes of children with lymphoma initially presenting as a genitourinary lesion, emphasizing the importance of early recognition and a systematic, stepwise diagnostic approach-one that optimizes evaluation to avoid missed diagnoses while minimizing unnecessary radiation exposure in children-with the ultimate goal of preventing unnecessary radical organ resection. STUDY DESIGN: This retrospective study included five boys (6-13 years old) who presented between January 2016 and April 2025 with a solitary urogenital mass later diagnosed as lymphoma. Clinical presentation, imaging, pathology, treatment, and outcomes were analyzed to identify key diagnostic and therapeutic patterns. RESULTS: Initial sites of presentation were the kidney (n = 1), retroperitoneum (n = 1), ureter (n = 1), penis (n = 1), and abdominal mass involving the bladder (n = 1). Imaging typically showed homogeneous soft-tissue masses with mild enhancement, without necrosis or calcification. PET-CT indicated systemic involvement in all patients. Histologic subtypes included aggressive mature B-cell (n = 2), Burkitt (n = 1), B-lymphoblastic (n = 1), and ALK-positive anaplastic large-cell lymphoma (n = 1). Four patients were diagnosed by percutaneous biopsy, and one after local excision. All received systemic chemotherapy, achieving complete remission in four and partial remission in one case complicated by vesical fistula. No deaths occurred during a median follow-up of 2.8 years. DISCUSSION: Lymphoma should be considered when evaluating pediatric genitourinary lesions in children, as the presentation may mimic primary malignancy and delay diagnosis. A complete imaging workup, including cross-sectional imaging with a low threshold for PET-CT, is essential to assess for systemic involvement. When diagnostic doubt persists after imaging, image-guided core-needle biopsy with multidisciplinary input can prevent unnecessary radical surgery. Study limitations include the small sample size and single-institution experience; however, the findings align with published evidence supporting high chemosensitivity and excellent outcomes with systemic therapy. CONCLUSION: Pediatric lymphomas presenting as genitourinary lesions are rare but highly treatable. Heightened diagnostic awareness, followed by a structured approach-comprehensive imaging and, when indicated image-guided biopsy enables accurate diagnosis, organ preservation, and favorable prognosis, meeting the study's aim of improving diagnostic awareness and management.