Case History: 54 year old man was admitted with 3 week history of dizziness and feeling unsteady on feet. He also had persistent central abdominal pain with no vomiting or diarrhoea. On examination, his lying and standing BP were 117/75 and 73/43 mm Hg respectively. He had tenderness over the right hypochondrium and right lumbar region. His initial investigations showed sodium 128 mmol/L and potassium 4.2 mmol/L. Short synacthen test was arranged and the cortisol values were 102, 113 and 136 nmol/L at 0′, 30′ and 60′. The patient was commenced on replacement with hydrocortisone. CT abdomen was ordered to investigate abdominal pain which showed bilateral large adrenal masses. No other mass were seen in the chest or anywhere in the abdomen. The histology and immunohistochemistry confirmed the diagnosis of large B-cell lymphoma. He was treated with chemotherapy.
Discussion: Primary Adrenal (extra nodal) Lymphoma (PAL) is an extremely rare entity and so far there have only been a few cases reported. PAL shows a predilection for males and usually presents above the age of 40. A significant percentage of patients present with symptoms and signs of adrenal insufficiency, while other symptoms include abdominal or back pain, fever, weight loss, malaise etc. More than two thirds of patients have bilateral enlargement of the adrenal glands. Diagnosis is primarily made with CT scan and guided biopsy of the mass. 90% of these lymphomas were of B-cell in origin and the vast majority of them are high grade malignancies. Multiagent chemotherapy is the main modality of treatment for PAL. Bilateral adrenalectomy, radiotherapy or a combination of them were tried before without much success. Addisonian crisis has severe life-threatening consequence, so there should be low threshold to investigate for PAL in patients presenting with abdominal pain, weight loss and symptoms of hypoadrenalism.