Endocrine Abstracts

Adrenal Lymphoma is extremely rare with fewer than 200 cases reported. The most common subtype is the diffuse large B cell lymphoma. Malignant lymphoma arising in the endocrine glands represents only 3% of extranodal malignant lymphomas and is usually confined to the thyroid gland. NK/T-cell lymphoma presents primarily in the nasopharynx and sinuses. Although involvement of the adrenal glands can be seen in disseminated disease, primary presentation with adrenal masses and adrenal insufficiency is extremely rare. We report a case of 34 years old gentleman presenting with prolonged fever, constitutional symptoms and symptoms and signs of adrenal insufficiency. He was treated as adrenal crisis in the district hospital with hypotension, hyponatremia, and hyperkalemia. He appeared cachexic, with no hyperpigmentation or palpable lymph nodes. His lactate dehydrogenase enzymes (LDH) was elevated. Cortisol taken during this stressful period was deemed too low. This was confirmed by an inadequate response to the Synacthen test. CT Adrenal was performed and reported as bilateral enlarged adrenal, likely hyperplasia. He developed multiple cranial nerves neuropathies that progressively worsened. CT Brain was normal. Lumbar puncture revealed high opening pressure and CSF biochemistry revealed no cells and negative for tuberculosis. With the possibility of disseminated tuberculosis, empirical anti-tuberculosis medications were started but failed to abate his symptoms. Laparoscopic left adrenal excision biopsy was performed and histology features are in keeping with high grade NK/T cell lymphoma. His condition deteriorated fast and he succumbed to his illness. This case illustrates the need to include Primary Adrenal Lymphoma in the differential diagnosis of adrenal masses especially when adrenal contour is preserved on imaging, hypoadrenalism and high LDH.