(8.06) Adrenal Lymphoma: A Rare Yet Clinically Significant Entity

Adrenal gland involvement as the primary site for non-Hodgkin lymphoma is extremely rare. Here, we present a challenging case of primary adrenal lymphoma (PAL) from diffuse large B cell lymphoma with elevated normetanephrines. 


Case(s) Description : A 78-year-old male with a history of hypothyroidism presented with 2-3 months of night sweats, abdominal pain, 20 pound weight loss and an episode of transient altered mental status. Medications consisted of levothyroxine.  The patient had normal blood pressures. CT abdomen revealed bilateral heterogeneous adrenal masses measuring 8cm on the right and 4.3 cm on the left. PET CT revealed large FDG-positive bilateral adrenal masses and retroperitoneal lymph nodes. Blood work revealed serum sodium 129mmol/L(136-145), potassium 5.9 mmol/L (3.4-4.3) with low aldosterone 4.1 ng/dL(0.0-31.0) and elevated plasma renin activity 21.6ng/mL/hr(0.2-1.6). Plasma normetanephrine was mildly elevated 1.99 nmol/L(0.00-0.89). HPA axis was elevated with plasma ACTH 475 pg/mL (less than 47), mildly elevated midnight salivary cortisol 0.375 mcg/dL ( < 0.228) and Urine free cortisol 61 mcg/24hr (3.5-45). Although plasma normetanephrine was mildly elevated, the patient was normotensive. We decided to pursue adrenal biopsy to rule out malignancy, because the adrenal lesion was new and not seen in previous images, and the progression of his symptoms were rapid. Adrenal gland biopsy revealed diffuse large B cell lymphoma. The patient has been started chemotherapy. Fludrocortisone 0.1mg was started for the mineralocorticoid deficiency. Repeat hormonal workup in the outpatient setting revealed normal plasma normetanephrines and normal salivary cortisol levels and normalized plasma ACTH. 

Discussion :

PAL is rare and has been reported in less than 200 cases. It is male dominant occurrence and usually presents with bilateral  and large lesion. There are no specific imaging characteristics to differentiate between adrenal lymphoma with other tumors from adrenal origin other than biopsy. Cases of mildly elevated metanephrines in PAL were reported in the past, but there is no guideline for decision making of the adrenal biopsy. Our case presented rapid onset of adrenal insufficiency in mineralocorticoid but not in glucocorticoid. We speculate that rapid mineralocorticoid disturbance occurred because lymphoma originating from the peripheral lymphoid tissue predominantly effected the glomerulosa layer causing mineralocorticoid deficiency. Elevated plasma ACTH was probably due to acute illness and as a positive feedback to mineralocorticoid deficiency. In conclusion, clear guideline from endocrine aspect for the management of PAL is necessary for this rare condition.