A Case of Primary Bilateral Adrenal Diffuse Large B-cell Lymphoma

Abstract

A 78-year-old woman was admitted to our hospital because of pancytopenia. Biochemical analysis revealed high lactate dehydrogenase and soluble interleukin-2 receptor levels. Plasma adrenocorticotropic hormone, plasma noradrenaline, and urinary normetanephrine levels were elevated. Plasma adrenaline and urinary metanephrine levels were low. The plasma cortisol level was within normal ranges. Abdominal CT revealed 2 masses, both measuring 7 cm in diameter, in the adrenal gland, with comparable and homogeneous density. An FDG-PET scan showed intense FDG accumulation in both adrenal glands without abnormal FDG uptake in the extraadrenal regions. The bilateral adrenal tumors were highly suggestive of malignant lymphoma. To make a definite diagnosis, operative procedures such as laparoscopic biopsy of the right adrenal gland were performed. Pathological examination showed diffuse infiltration of large atypical lymphocytes. Immunohistochemical analysis showed that staining for B-lymphocyte-specific markers such as CD20 was exclusively positive in large atypical lymphocytes. Diffuse large B-cell lymphoma, which originated in both adrenal glands was diagnosed, and R-CHOP therapy was administered. Involvement of the central nervous system was noted 5 months after surgery, and the clinical course deteriorated rapidly thereafter. The patient died 8 months after surgery. The prognosis of patients with primary adrenal lymphoma seems to be dependent on the ability to make a definite diagnosis on the basis of laparoscopic biopsy and start accurate treatment without any delay.