Background: Primary adrenal Non Hodgkin Lymphoma (NHL) accounts for less than 1% of all NHL cases. Among them, Diffuse Large B Cell Lymphomas (DLBCL) are the most commonly identified. Seventy percent of cases are bilateral and adrenal insufficiency usually occurs. Here we describe a case of a DLBCL with bilateral adrenal involvement and adrenal insufficiency.
Clinical case: A 68 years male with a personal history of malaria, asthma, hypertension and cholecystectomy. He was admitted in a primary level hospital for a consumptive condition with weight lost, fever, nausea, vomiting, muscular and abdominal pain. The initial complementary study showed hyponatremia (135mmol/L) and a positive blood culture for Streptococcus. Antibiotic therapy was implemented. A thoracic-abdominal-pelvic computed tomography (CT) revealed bilateral adrenal masses with intermediate density, measuring 76 mm in the right adrenal gland and 55 mm in the left. He requested medical care a week later due to worsening of his general condition, presenting with hypotension and skin and mucosa hyperpigmentation. Laboratorial analysis showed hyponatremia (121 mmol/L) and hyperkaliemia (5 mmolL), low serum cortisol (1.4 ug/dL), and high ACTH (327 pg/mL), high plasma renin activity (4.1 ng/mL/h), low aldosterone (0.9 ng/mL) and high ß2 microglobulin (13.9 mg/L). The leukocyte blood count was normal. At this time, the diagnosis of primary adrenal insufficiency was made. He was treated with intravenous hydrocortisone and saline fluids, followed by daily oral hydrocortisone and fludrocortisone, and his health status has progressively improved. A F18-Positron Emission Tomography (PET) was performed and showed two adrenal masses suggestive of malignancy (SUV 21.4 in right adrenal gland and 20.5 in left adrenal gland). It also demonstrated intra-abdominal and testicular lymph nodes. Considering the high suspicion of adrenal malignancy, he was transferred to our Oncology Center where an adrenal biopsy was performed. A large diffuse large B cell Lymphoma, non-germinal center, Ki67=70%, was diagnosed, showing positive staining for CD20, BCL6, BCL2, MUM1 and negative for CD3, CD5, CD10, MYC. He was then treated with chemotherapy combining rituximab, cyclophosphamide, doxorrubicin, vincristine and prednisolone. One year later the 18FDG-PET showed no evidence of disease.
Conclusion: This case describes a rare primary adrenal diffuse large B-cell lymphoma, one of the most uncommon causes of adrenocortical insufficiency. Although it is a rare entity, it should be considered in the differential diagnosis of bilateral nodular adrenal lesions, particularly in the setting of exclusive bilateral adrenal 18FDG-PET uptake and adrenal insufficiency.
18 - 21 May 2019
European Society of Endocrinology