Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature

Lymphomas are cancers that arise from the white blood cells and have been traditionally divided into two large subtypes: Hodgkin and non-Hodgkin lymphoma. B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma; almost 85% of patients with lymphoma have this variant. Lymphomas can potentially arise from any lymphoid tissue located in the body; however, primary adrenal non-Hodgkin lymphoma is extremely rare. We report the history, examination findings, and laboratory results of a 50-year-old man diagnosed with a primary left adrenal diffuse large B-cell lymphoma.

A 50-year-old Pakistani man presented to our hospital with progressively increasing pain and fullness in the left upper quadrant of his abdomen, generalized weakness, easy fatigability, and decreased appetite of 1.5 months’ duration. On examination, he had a blood pressure of 140/80 mmHg with no postural drop, a pulse rate of 106 beats/minute, and no fever. His past medical history was significant for pulmonary tuberculosis 2 years earlier, for which he received antituberculous therapy. Computed tomography revealed a heterogeneous enhancing soft tissue density mass in the left adrenal gland. It measured 7.1 × 5.6 × 9.5 cm. Further laboratory workup revealed the following levels: sodium 135 mEq/L, potassium 4.5 mEq/L, lactate dehydrogenase 905 IU/L, renin 364 IU/ml, aldosterone 5.79 ng/dl, dehydroepiandrosterone sulfate 79.20 μg/dl, urinary vanillylmandelic acid 6.4 mg/24 hours, and a low-dose overnight dexamethasone suppression test result of 3.20 μg/dl. The patient underwent left adrenalectomy. Histopathological test results showed a diffuse large B-cell lymphoma. Immunohistochemical stains were strongly positive for CD20 and negative for CD3, CD5, CD10, and cyclin D1. The patient’s Ki-67 (Mib-1) index was approximately 80%. He received a total of six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy (rituximab was not given, owing to financial constraints) and was routinely followed pre- and postchemotherapy at our hematology clinic with complete blood count and serum lactate dehydrogenase evaluations. The patient responded to chemotherapy and is currently doing well.

Primary adrenal lymphoma is an extremely rare but rapidly progressive disease. It generally carries a poor prognosis, partly because an optimal treatment protocol has not yet been established. Further studies with larger sample sizes are needed to establish the best treatment option and increase overall survival.