Successful treatment of primary adrenal insufficiency due to malignant non-Hodgkin's lymphoma

Abstract

Although the adrenal glands are frequently the site of tumor metastases, adrenal insufficiency is exceedingly rare. We report on a patient with high-grade B-cell centroblastic lymphoma who initially presented with right axillary lymphadenopathy and bilateral adrenal masses. Four months after axillary lymphadenectomy the patient developed overt signs of Addison's disease. He recovered promptly after initiation of hormone replacement therapy and bilateral adrenalectomy. At present, 16 months after additional chemo- and radiation therapy the patient is considered free of tumor. To our knowledge this is the first report on a patient who presented with adrenal insufficiency in the course of non-Hodgkin's lymphoma and who was successfully treated. Demonstrating this case, we would also like to stress that the development of adrenal insufficiency does not necessarily indicate widespread tumor manifestation in patients with non-Hodgkin's lymphoma.

Non-Hodgkin's Lymphoma Presenting as Thyroid and Adrenal Gland Involvement.

“…Adrenal involvement in our patient was bilateral. Although adrenal insufficiency due to NHL involvement has been reported by some authors [20,[22][23][24][25][26][27], no clinical or laboratory evidence of such process was present in our patient.…”

“…Nodular enlargement is the most frequent pattern but diffuse goiter can also be seen [16]. Adrenal involvement in NHL can either be unilateral or bilateral [17][18][19][20][21][22][23][24][25][26][27][28][29][30]. The frequency of bilateral and unilateral presentations are similar in secondary lymphomatous adrenal involvement.…”

Metastatic Carcinomas of the Adrenal Glands: From Diagnosis to Treatment

“…Two of these studies that have been conducted at the Memorial Sloan-Kettering Cancer Center of New York (81) and Mayo Clinic (73), respectively, summarized the data. The remaining nine studies (1,21,22,61,62,82,(85)(86)(87)(88)(89) contained sufficient data but number of cases was small or had insufficient data. The larger of these two studies, conducted by Branum and associates, have compared surgical with non-surgical treatment of 24 patients with melanoma metastatic to the adrenals.…”

Adrenal insufficiency caused by primary aggressive non-Hodgkin's lymphoma of bilateral adrenal glands: report of a case and literature review

“…Only three of the 29 patients achieved a good response, with either complete remission (case 16) or recovery of adrenal function and regression of the tumor (cases 4, 19) after treatment. In case 16, the patient was successfully treated with bilateral adrenalectomy and subsequent chemotherapy as well as hormone support [26]. All of the others died within 9 months because of rapid disease progression and infection.…”

“…Concerning management, immediate replacement therapy to avoid the catastrophic consequence of addisonian crisis and then intensive chemotherapy play an important role in reducing the risk of mortality [8,13,14,21,25,26]. Some investigators suggested that surgical debulking with subsequent chemotherapy for large masses could provide a better complete response rate [13].…”