Background: Adrenal lymphomas are rare and often present with hypoadrenalism in the context of bilateral adrenal masses. We report a patient with unilateral adrenal mass and hypoadrenalism at presentation before evolving rapidly to bilateral masses proven to be a large B cell lymphoma. We discuss mechanisms of hypoadrenalism in adrenal lymphoma.
Case history: A 79 year gentleman with no signifanct past medical history admitted with a 6 week history of being generally unwell, dizzy and fatigued. Physical examination revealed low Blood pressure with postural drop and investigations revealed mild hyponatremia (131), hyperkalemia (6.3) and hypercalcemia (2.73). Random cortisol returned low at 117 mmol/l and failed to respond to synacthen 250 mcg with peak cortisol of 136 mmol/l. CT scan showed a Right sided large 12×10×8 cm suprarenal mass with central necrosis suspicious for primary adrenal cancer and the opposite adrenal looked normal. He was started on replacement hydrocortisone and his blood pressure improved. A subsequent FDG/PET showed disseminated uptake including in both adrenals (with the previously normal left adrenal now grown to × cm) and widespread lymphadenopathy. With normal plasma metanephrines, a CT guided biopsy of right adrenal was organised and showed diffuse large B cell lymphoma. He was started on RCHOP chemotherapy. A repeat CT scan after the 4th cycle of chemotherapy showed complete resolution of lympdenopathy and left adrenal mass and shrinkage of right adrenal to 11×7×3 cm. He remains well on replacement hydrocortisone and fludrocortisone.
Discussion: Hypoadrenalism in the context of adrenal masses is often related to near total (>90%) destruction of adrenal cortex. Our patients presentation with hypoadrenalism and unilateral mass is unusual although the opposite adrenal rapidly grew subsequently and responded to chemotherapy for B cell lymphoma. We discuss other possible mechanisms that may explain this unusual presentation.