Persistent adrenocortical insufficiency before and after treatment of lymphoma with marked adrenal enlargement: A case series

Yuri Takiyama¹Takashi Nanbu¹Tasuku Sato¹Fumika Maruyama¹Yuki Shukuda¹Takao Takiyama¹Chihiro Sumi²Takeshi Saito²Hiroya Kitsunai¹Shuichiro Takahashi²Yumi Takiyama¹Hiroshi Nomoto^1*

• ¹Division of Endocrinology, Metabolism, and Rheumatology, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan
• ²Division of Hematology, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Hokkaido, Japan

Although lymphoma involving the adrenal gland is uncommon, it is associated with a high incidence of adrenal insufficiency, which may lead to adrenal crisis. The changes in adrenocortical function over the course of lymphoma treatment are not well described. We report three cases with lymphoma with bilateral adrenal enlargement who presented with adrenal insufficiency and had their adrenocortical function monitored during treatment. Case 1 was a 72-year-old man who presented with non-specific symptoms and was diagnosed with lymphoma involving the adrenal glands. Case 2 was a 71-year-old woman who was diagnosed with adrenal lesion of intravascular large B-cell lymphoma. Case 3 was an 84-year-old man diagnosed with primary adrenal lymphoma, presenting rapidly progressing bilateral adrenal tumors. All three were diagnosed with adrenal insufficiency at presentation. Rapid ACTH stimulation test was performed before and after chemotherapy and/or glucocorticoid replacement therapy, and adrenal insufficiency remained in all. Therefore, each required persistent glucocorticoid supplementation despite marked reduction in adrenal lesion. It is important to evaluate adrenocortical function and consider continuing glucocorticoid replacement therapy even after a significant treatment response in adrenal lymphomas.