Session: LBT 064-076-Late-Breaking Adrenal/HPA Axis I
Poster Board LBT-067
Adrenal lymphoma is a rare entity, and is usually fatal; we report a case of bilateral adrenal lymphoma, presenting with adrenal insufficiency and bilateral adrenal masses, with response to chemotherapy.
Patient is a 72 year-old male, with hypertension and COPD (not on chronic oral or inhaled steroids) who presented with recurrent syncope, hypotension and 30 lbs. weight loss. Initial work up revealed hyponatremia (Na 124mg/dl), a WBC of 4.72 k/cmm with lymphocytocis (69.9% neutrophils, and 17.2% lymphocytes). ACTH stimulation test was consistent with adrenal insufficiency. Baseline cortisol was 6.73 ug/dl, ACTH 45 pg/ml (n 6-50). After Cosyntropin 250 mcg IV, cortisol was 6.41 ug/dl and 6.52 ug/dl at 30 and 60 minutes respectively. Thyroid levels were normal: TSH 2.6 (n 0.27-42 uIU/ml), free T4 1.19 (n 0.93-1.7 ng/dl). Aldosterone was undetectable (< 1 ng/dl) with elevated plasma renin activity of 10.6 (n 0.25-5.8 ng/ml/hr). MRI of the abdomen showed bilateral adrenal masses measuring, 3.3 x 2.7 cm on the right and 3.3 x 2.5 cm on the left. Biopsy of the adrenal mass was advised but patient refused. Patient was initiated on Hydrocortisone 20 mg q am and 10 mg q pm and Fludrocortisone 0.1 mg daily. He was discharged home after initial improvement.
In follow up a PET CT (performed three months after initial abdominal imaging) showed bilateral enlarged adrenal masses measuring 4.2 x 3.9 cm on the right and 7.3 x 5.8 cm on the left, with diffuse lymphadenopathy and no involvement of CNS. Left inguinal lymph node excisional biopsy was histologically consistent with large B-cell lymphoma, intermediate grade, negative cyclin-D1, negative CD138 and Ki-67 > 60% (high). Patient was diagnosed with stage IVB diffuse large B-cell lymphoma and was started on chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). After 6 cycles of chemotherapy the right adrenal mass was reduced in size to 1 cm and left adrenal mass to 1.5 cm. Patient is currently on maintenance chemotherapy with Rituximab. His hyponatremia has corrected. Despite almost complete resolution of adrenal masses, adrenal insufficiency is persistent as evidenced by a low morning cortisol of 1.30 ug/dl (n 6.2-23) after holding pm and am dose of hydrocortisone. He is continued on hydrocortisone and fludrocortisone.
There are two types of adrenal involvement in lymphoma: primary adrenal lymphoma, defined as originating from and confined to the adrenal glands solely, and non-Hodgkin lymphoma with adrenal involvement. Primary adrenal lymphoma is rare with less than 100 cases reported and carries a poor prognosis. R-CHOP had been used with encouraging results in a series of patients with adrenal lymphoma (Kim YR, 2012). Our patient has shown good response to chemotherapy but with persistent adrenal insufficiency suggesting the destructive nature of the adrenal involvement.
Nothing to Disclose: AM
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