Lymphoblastic lymphoma (LBL) is a rare malignancy of precursor lymphoid cells, typically presenting with mediastinal or nodal disease. Isolated testicular involvement is exceptionally uncommon. Due to its rarity, there is limited guidance on diagnostic workup, staging, treatment, and follow-up. We present a case of bilateral primary testicular lymphoblastic lymphoma in an adult and review the current evidence on its management.

A targeted literature review was conducted using PubMed and Google Scholar to identify reported cases of lymphoblastic lymphoma with primary or isolated testicular involvement, including both B-cell and T-cell subtypes. Data were extracted on presenting features, diagnostic approach, imaging, treatment regimens, and outcomes. These findings were synthesised with an illustrative case of adult bilateral testicular LBL to highlight diagnostic and therapeutic considerations.

Nine cases of localised primary testicular lymphoblastic lymphoma were identified, comprising two T-cell and seven B-cell subtypes. Presentation most commonly involved testicular swelling (n=7), with pain reported in five cases and constitutional symptoms in one. Eight cases were unilateral; only one, our case, demonstrated bilateral involvement. Ages ranged from 3 to 39 years, with five paediatric and four adult patients. Diagnosis and staging involved ultrasound (n=7), CT (n=7), bone marrow biopsy (all), lumbar puncture (n=6), and FDG-PET scan (n=3). Local control was achieved with orchidectomy in seven cases, including one bilateral orchidectomy (this case); one case was managed with biopsy alone and one did not specify local treatment. Radiotherapy to the contralateral testis was conducted in two cases. All patients received systemic and intrathecal chemotherapy using variable regimens based on Acute Lymphoblastic Leukaemia protocols, despite no confirmed CNS involvement. Follow-up duration ranged from 3 to 36 months. One patient experienced relapse at 3 months; all others remained disease-free during follow-up.

Testicular lymphoblastic lymphoma is a rare and diagnostically challenging entity. Accurate histopathological diagnosis and thorough staging are essential. Local control with orchidectomy is commonly used alongside systemic and intrathecal chemotherapy, though no standardised treatment or follow-up protocols exist. Bilateral involvement, as in our case, highlights the need for early consideration of sperm preservation and potential requirement for testosterone replacement, particularly when bilateral disease is suspected or when radiotherapy to the contralateral testis is planned. Further multicentre studies are needed to guide optimal management and long-term surveillance.