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Natural killer T-cell lymphoma presenting as adrenal mass and partial adrenal insufficiency followed by CNS involvement.

Natural killer (NK) T-cell lymphoma is a rare, Epstein-Barr virus (EBV) –related lymphoma that primarily affects the paranasal sinuses and CNS. It is seen in Asian and Central and South American populations, but is extremely rare in North America. A typical presentation includes a mass of the nasal septum, sinuses, and palate. Extranasal involvement of the adrenal gland, lymph nodes, skin, or lung is rare and portends a poor prognosis. A 70-year-old man presented with chest pain, nausea, vomiting, abdominal pain, a 30-pound weight loss, and acute on chronic renal insufficiency. The symptoms were progressive over a 4-month period. An evaluation for abdominal pain one month before admission, which included computed tomography (CT) scan of the abdomen, did not show any intra-abdominal pathology. Repeat CT of abdomen on admission showed new bilateral adrenal masses measuring 4.2 3 cm on right side and 3.7 2.8 cm on left side (Fig 1). An outpatient biopsy was planned, but he returned with progressive symptoms. A third CT scan showed further enlargement of the adrenal masses (8.1 4.5 cm on the left and 5.5 3.0 cm on the right), but CT scan of the chest was negative for primary lung malignancy. The differential diagnosis included primary adrenal malignancy therefore serum levels of adrenal hormones were drawn. The results were as follows: epinephrine 29 pg/mL (normal 84 pg/mL), metanephrine 0.46 nmol/L (normal 0.0 to 0.49 nmol/L), norepinephrine 2,122 pg/mL (normal 420 pg/mL), normetanephrine 2.70 nmol/L (normal 0.0 to 0.89 nmol/L), total catecholamine 2,151 pg/mL (normal 504 pg/mL), and dopamine lower than 30 pg/mL (normal 60 pg/mL). A repeat metanephrine level was 0.17nmol/L (normal 0.0 to 0.49 nmol/L) and normetanephrine was 1.36 nmol/L (normal 0.0 to 0.89 nmol/L). Cushing’s syndrome was ruled out with a negative dexamethasone suppression test. Suspicion for pheochromocytoma was low since metanephrine and normetanephrine levels were declining. He was started on phenoxybenzamine and CTguided biopsy was performed. The patient was discharged while waiting for the biopsy results. The interpretation of the biopsy was delayed because of the unusual nature of the neoplasm. He then presented for readmission with nausea, vomiting, abdominal pain, and a blood pressure of 95/65 mmHg. Laboratory studies revealed hyponatremia (sodium 127 mmol), hyperkalemia (potassium 5.2 mmol), and renal failure (blood urea nitrogen/creatinine 120/4.2 mg/dL). Thirty minutes after adrenocorticotropic hormone stimulation, the basal cortisol level increased from 9.42 mcg/dL to 15.67 mcg/dL, confirming the diagnosis of primary, partial adrenal insufficiency. The final pathology report revealed a lymphoproliferative neoplasm positive for CD3, CD 56, and EBV and negative for CD4 and CD8, consistent with a diagnosis of NK/T-cell lymphoma (Fig 2). A positron emission tomography/CT scan showed an increased standard uptake value in bilateral adrenal masses, retroperitoneal adenopathy, and in the pituitary gland. Chemotherapy was initiated with cyclophosphamide, doxorubicin, vincristine, and prednisone. Two months after the initiation of chemotherapy, the