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Primary Bilateral Adrenal Lymphoma Revealed by Adrenal Insufficiency: From Constitutional Symptoms to Crisis
Keiko Carter1, Abanoub Khalil2, Mohamad Abufaied3
1Internal Medicine, University Hospital of Wales, Cardiff, GBR.
Abstract:
Primary adrenal lymphoma (PAL) is an uncommon extranodal non‑Hodgkin lymphoma that typically presents with bilateral adrenal masses and carries a poor prognosis, especially when complicated by adrenal insufficiency; primary bilateral adrenal lymphoma is a rare but important cause of adrenal insufficiency and adrenal crisis, particularly when large bilateral adrenal masses are detected on imaging. A 50‑year‑old man presented with several months of constitutional symptoms, including night sweats, vomiting, weight loss, diarrhoea and myalgia, and was initially treated conservatively with symptom resolution, but four months later he was readmitted with anorexia, further weight loss, fever and left upper quadrant pain; imaging revealed bilateral adrenal masses (6.7 cm right, 4.5 cm left) with high non‑contrast attenuation (>20 Hounsfield units) and splenomegaly, and MRI confirmed progression of the right adrenal lesion to 8.5 cm. Biopsy demonstrated diffuse large B‑cell lymphoma consistent with PAL, and he was commenced on hydrocortisone and fludrocortisone replacement together with eight cycles of R‑CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisolone) and intrathecal methotrexate, achieving complete radiological remission sustained to 2016, with remission maintained until his death from sepsis and multi‑organ failure in 2023. This case illustrates the broad symptom spectrum of PAL, from B-symptoms to adrenal crisis, and underscores the importance of considering PAL in the differential diagnosis of bilateral adrenal masses with high attenuation. Early recognition of adrenal insufficiency and adrenal biopsy are critical to guide timely initiation of life-saving glucocorticoid replacement and lymphoma-directed therapy.
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