Primary adrenal lymphoma: three case reports and review of Japanese cases

Abstract

Abstract Although primary adrenal lymphoma (PAL) is thought to be extremely rare, its prognosis is much worse than that of other extranodal malignant lymphomas. There are so far about 130 reported cases in the English literature, while 186 cases have been reported in the Japanese literature. Thus, the incidence of PAL in the Japanese population may be higher than in other races. The typical characteristics of PAL in Japanese patients are similar to those previously reported in Western literature: it predominantly occurs in male and elderly patients and involves bilateral involvement of the adrenal glands, which frequently leads to adrenal insufficiency. Here, we review three recent cases of PAL at our hospital, and analyze data from our institution regarding patients with PAL from 2002 to 2014. On biochemical analysis, median levels of sIL2R (5027.5 U/mL) and LDH (1111.46 U/L) were elevated in Japanese PAL patients compared to other adrenal tumors. It is critical that clinicians be familiar with the traits of PAL, especially for its differential diagnosis from adrenal large tumors.