Primary Adrenal Lymphoma with Adrenal Insufficiency: Report of Three Cases and Review of Literature

Presentation Number: SUN 414
Date of Presentation: April 2nd, 2017

Masumi Ogawa*1, Naoki Edo1, Takuji Matsuo1, Tadashi Yamamoto1, Satoshi Takahashi1, Yamato Mashimo1, Koji Morita1, Yuko Sasajima2, Hiroshi Uozaki1, Fukuo Kondo2, Yasunori Ota1, Naoki Shirafuji1, Hiroko Okinaga1, Kazuhisa Tsukamoto1 and Toshio Ishikawa1
1Teikyo University School of Medicine, Tokyo, Japan, 2Teikyo University Hospital, Tokyo, Japan

Abstract

[Introduction] Primary adrenal lymphoma (PAL) is an extremely rare disease with a high incidence of bilateral adrenal involvement whose prognosis is usually poor even with aggressive chemotherapy such as R-CHOP. Here we report three patients with PAL. All of them initially presented with adrenal insufficiency, which later turned out to be due to destruction of both adrenal cortices by diffuse large B-cell lymphoma (DLBCL).

[Case Presentation] The three cases were referred to our hospital. In all of them, basal plasma ACTH concentrations were high, and serum cortisol levels were relatively low and inadequately responsive to exogenous ACTH administration, confirming the biochemical diagnosis of primary adrenal insufficiency. Together with the bilateral adrenal masses observed on their abdominal CT scans, it was suggested that malignant cells were infiltrating in their adrenal glands. Histological diagnosis of DLBCL was confirmed by percutaneous adrenal needle biopsy (Cases 1 and 3) or laparoscopic adrenalectomy (Case 2). In Case 1, despite initial shrinkage of the adrenal masses after R-CHOP, his lymphoma became aggressive, chemotherapy-refractory, and eventually fatal. In Case 2, while the adrenal lesions responded to R-CHOP, para-aortic lymph node swelling indicative of lymphoma relapse was found. He is now being treated with salvage chemotherapy. In Case 3, the adrenal masses are at present shrinking in response to R-CHOP.

[Discussion] PAL is so rare, with fewer than 200 cases reported in the English literature so far, that its pathological characteristics, clinical course, and optimal treatment regimen have not been clearly determined. Approximately 60% of PAL patients develop adrenal insufficiency, which in itself can be life-threatening if untreated. Therefore, adrenocortical function should be promptly evaluated, and, if necessary, immediate glucocorticoid replacement should be started in an appropriate amount, which is sufficient for the body in illness but should not interfere with histological diagnosis of lymphoma.

 

Nothing to Disclose: MO, NE, TM, TY, ST, YM, KM, YS, HU, FK, YO, NS, HO, KT, TI