Bilateral primary adrenal lymphoma

Abstract

A 51-year-old man presented with abdominal pain. Abdominopelvic magnetic resonance imaging (MRI) revealed inhomogeneous tumours involving both adrenal glands, with the right gland measuring about 14 cm and the left 11 cm in maximum diameter (Figure). A percutaneous ultrasound-guided biopsy of the right adrenal gland was performed. Diffuse large B-cell non-Hodgkin lymphoma was diagnosed by histopathological examination. The serum biochemistry profile, adrenocorticotropic hormone (ACTH) stimulation test and urine analysis were normal. The patient received six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy; complete remission was achieved after three cycles. At present, 6 months after chemotherapy, the patient is considered tumour-free. Primary adrenal lymphoma is rare. The majority of the patients are elderly men with bilateral adrenal involvement and the most common histology is diffuse large B-cell lymphoma. The importance of early awareness of the possibility of this diagnosis in patients with bilateral adrenal masses complicated by adrenal insufficiency should be emphasized. Definitive diagnosis is established by image-guided biopsy or by surgical excisional biopsy. On computed tomography and MRI imaging, primary adrenal lymphomas tend to appear as complex masses of variable density, often with areas of necrosis and/or haemorrhage; most tumours are bilateral. An appropriate treatment of this lymphoma is not well established. Therapeutic modalities include multi-agent chemotherapy and surgery followed by chemotherapy and/or radiation therapy. Most of these tumours are highly aggressive and treatment is not satisfactory. Usually, the prognosis is poor with early death occurring during chemotherapy. However, one-third of patients achieve partial or complete remission during chemotherapy.