SUN-492 - When Bilateral Adrenal Masses Signal More: A Rare Case of NK/T-Cell Lymphoma

Primary adrenal lymphoma is a rare adrenal malignancy within the category of extra-nodal Non-Hodgkin lymphoma (ENNHL) that is typically of B-cell origin. However, ENNHL of NK/T-cell lymphoma is extremely rare, associated with EBV and carries a very poor prognosis. Of all reported cases, the longest surviving patient was 26 months from initial presentation. We present a case of rapidly progressive bilateral adrenal NK/T-cell lymphoma resulting in massive adrenal hemorrhage and adrenal insufficiency.

Case presentation:
Our patient is a 47-year-old male with known bilateral adrenal masses who presented with abdominal pain, fatigue, weight loss and hypotension (93/70 mmHg). Adrenal masses were initially found in August 2024 and demonstrated rapid growth over 18 months (increasing from 2 cm to 7.5 cm on the left and 1.3 cm to 8.7 cm on the right) and were complicated by massive bilateral adrenal hemorrhage (11 cm on the left and 24 cm on the right).

Cosyntropin stimulation demonstrated an inadequate response (baseline cortisol 14 µg/dL; 60 min: 15.2 µg/dL) consistent with adrenal insufficiency. Additional workup revealed normal aldosterone, plasma renin activity, and chromogranin A. Plasma metanephrine levels were not consistent with pheochromocytoma.

FDG-PET showed large bilateral heterogeneous adrenal masses with central necrosis and hypermetabolic peripheral activity (SUVmax 13.2 left, 18.3 right). CT guided biopsy confirmed pathology of peripheral NK/T-cell lymphoma. Cytogenetics was significant for c.58C>T, nonsense, truncating, loss of function mutation in SOCS1 gene.

His course was further complicated by IVC thrombosis, pleural effusion, tumor lysis syndrome, renal failure, and hemodynamic instability requiring stress-dose hydrocortisone and ICU level of care. Due to advanced disease and poor overall prognosis, he was not considered a candidate for systemic chemotherapy. Palliative care was consulted, and the patient ultimately died.

Conclusion:
Adrenal lymphoma accounts for less than 1% of all extranodal lymphomas and while most of these are of B-cell origin, we report a rare adrenal lymphoma of NK/T cell origin. Often presenting as abdominal pain or even as adrenal insufficiency if there is bilateral involvement, this cancer portends a poor prognosis. Therefore, when faced with bilateral adrenal masses, it is important to consider adrenal lymphoma in the differential and pursue prompt tissue diagnosis. The role of surgery is limited and mainstay treatment is systemic therapies, such as SMILE or CHOP.

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