Bilateral adrenal masses and adrenal insufficiency: a rare case of primary adrenal lymphoma.

Summary: Primary adrenal lymphoma (PAL) is a rare malignancy typically considered in patients presenting with features of adrenal insufficiency and bilateral adrenal gland enlargement. Early diagnosis and differentiation from other causes of bilateral adrenal masses are essential to guide appropriate management. Prompt initiation of chemotherapy can significantly improve survival outcomes. We present the case of a 72-year-old patient with adrenal insufficiency and imaging-confirmed large bilateral adrenal masses with widespread lymph node involvement. Core biopsy of the adrenal mass confirmed high-grade diffuse large B-cell lymphoma (DLBCL), consistent with a diagnosis of PAL. She was initially managed with systemic chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), achieving a complete metabolic response. Two months later, she presented with relapsed central nervous system disease and died despite intrathecal cytarabine and methotrexate chemotherapy. Given the rarity of this condition, we use the case to illustrate and discuss key aspects of PAL, including demographics, pathogenesis, diagnosis, management, and prognosis. Learning points: PAL is a rare but important diagnosis to consider in patients presenting with bilateral adrenal masses and adrenal insufficiency, especially in the absence of a known primary. In this case, the patient presented repeatedly with constitutional symptoms before the diagnosis was made through imaging. Whether an earlier diagnosis would have changed the clinical outcome remains unclear. This suggests that early imaging is important to initiate the diagnostic and management cascade in patients presenting with persistent, unexplained symptoms. Following exclusion of phaeochromocytoma, early biopsy and histopathological confirmation is crucial for accurate diagnosis and guiding management. Immunochemotherapy is the mainstay of treatment and may improve survival even in the setting of widespread disease. CNS involvement is important to exclude through MR scan of the brain and CSF sampling. Further research into predictors of recurrence and CNS relapse may improve long-term outcomes in PAL.