Lymphoma presenting with Addison's disease

Abstract

The commonest cause of primary adrenal failure and shrunken adrenals is autoimmune adrenalitis. The same clinical picture with adrenal masses is nowadays most commonly due to carcinomatous spread. Our case emphasizes the importance of obtaining tissue from patients such as these, where no primary lesion is apparent, as occasionally a treatable lymphoma will be found.

Clinically silent primary adrenal lymphoma: A case report and review of the literature

“…Primary malignant lymphoma of the adrenal gland is extremely rare and so far only 30 cases have been reported in the Western countries including France [3,[26][27][28][29], Germany [30], Italy [24,31], The Netherlands [23], Spain [13,17,18,20,25], United Kingdom [10,11,19], and the United States [4][5][6][7][8][9]12,[14][15][16]21,22]. There is one reported case from Taiwan [32], another from Hong Kong [33], and 33 other cases [34][35][36][37][38][39][40][41][42][43][44][45] from Japan over the past 49 years.…”

“…The pertinent clinicopathologic features of the previously reported 55 cases, which include those in the English literature as well as those non-English reports containing enough information in the English abstracts, are summarized in Table I [3][4][5][6][7][8][9][10][11][12][13][14][15][16][18][19][20][21][22][23]28,30,[32][33][34][35]37,39,[41][42][43][44][45]. A more detailed account of each of the 30 isolated cases reported in the English literature is illustrated in Table II.…”

“…Angiogram [5,6,7,23,39] and intravenous pyelogram [8,12,26] are useful to distinguish renal lesions from adrenal lesions. Nevertheless there is no pathognomonic appearance on CT, MRI, or US to indicate lymphomatous involvement of adrenal glands and one cannot distinguish primary from metastatic lesions [19]. The diagnosis of PALs in other cases is made by surgeries [3,5,6,7,12,16,22,23,33,39] and postmortem examinations [8,15,34,41].…”

“…Of those reports that provided follow-up, most patients died of tumor, intercurrent diseases, or infections within 1 year (Table II, [43]). However, 7 and 13 of these 55 patients achieved a complete [13,18,31,35,37,41,43] or partial [3,18,19,22,23,32,33,39,41,42,43] remission, respectively (Table II). One of the patients was in remission 4 years after treatment (Table II, case 28).…”

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

“…Some investigators suggested that surgical debulking with subsequent chemotherapy for large masses could provide a better complete response rate [13]. Alternatively, radiotherapy could be given to a bulky mass lesion in order to prevent local recurrence [12,21]. Our patient was treated with a less intensive chemotherapeutic regimen of cyclophosphamide, vincristine, and oral prednisolone because she had a very poor performance status.…”