SAT-233 Primary Adrenal Diffuse Large B-Cell Lymphoma in HIV Patient: A Rare Case Report

• Published in: Journal of the Endocrine Society Vol. 4; no. Supplement_1
• Main Authors: Ibrahim, Safa, Thibodeaux, Rachel, Mathew, Mini
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 08-05-2020
• Subjects: Adrenal
• ISSN: 2472-1972; 2472-1972
• DOI: 10.1210/jendso/bvaa046.687

Abstract
Introduction: Primary adrenal lymphoma is remarkably rare type of extra-nodal lymphomas, accounting for <1% of non-Hodgkin lymphomas. For unclear reasons, HIV population appear to have an increased risk of developing non-Hodgkin lymphoma. We are presenting a rare case of bilateral primary adrenal non-Hodgkin lymphoma in HIV patient.
Case Presentation: A 65-year-old man with history of HIV, presents with nausea and generalized weakness for the past month. Other symptoms include, fever and unintentional weight loss of 10 kg also reported.
On admission, patient was found to have low sodium value of 124 mmol/L. He was worked up for hyponatremia; Labs were suggestive of primary adrenal insufficiency. Other endocrinology labs including; TSH, metanephrines, renin, and aldosterone were within normal values.
CT of the abdomen showed bilateral adrenal masses. The right lesion measured 10.6 cm. The left lesion measured 10.1 cm. Fungal work up was negative. Epstein Barr virus (EBV) panel was suggestive of convalescent versus reactivated disease.
CT guided biopsy of the adrenal mass confirmed Diffuse Large B-cell Lymphoma, activated B Cell Phenotype. Bone marrow biopsy showed no evidence of lymphoma.
Following oncology recommendations, patient was started on R-CHOP therapy. Currently, patient received two cycles of chemotherapy which he is tolerating well.
Discussion: Primary adrenal lymphoma is a rare type of non-Hodgkin lymphomas (NHL). Diffuse large B cell lymphoma (DLBCL) is the most common histologic subtype of NHL that present in the adrenal glands. Approximately one hundred cases reported of primary adrenal DLBCL in the literature with only two previously reported cases in HIV patients. It is considered an AIDS defining malignancy. HIV population are at higher risk of developing NHL than general population. The pathogenesis of the development of NHL in adrenal glands, which lack lymphoid tissue, particularly in HIV patients is yet to be determined, but impaired cellular immunity and chronic B cell stimulation and T cell immunodeficiency leading to loss of control of transforming viruses, particularly EBV are thought to play essential roles.
Because of the rarity of this tumor, diagnosis can be challenging. As exemplified in our patient, most cases have nonspecific presentation, including; fever, weight loss, night sweats, nausea, vomiting, adrenal insufficiency, and hyprecalcemia.
The recommended treatment for primary adrenal DLBCL is chemotherapy, with R-CHOP being the typical treatment. This disease appears to have a poor prognosis, and the median survival is approximately 1 year. Young age, good performance status, and early diagnosis and treatment can improve the outcome.
Conclusion: Primary adrenal DLBCL are rare tumors that has been associated in the literature with impaired cellular immunity. Our patient is the third case reported of primary adrenal DLBCL in HIV positive patient.