Aggressive transformation of an indolent myeloma with abdominal extramedullary plasmacytoma after 6-year period of observation]

62-year-old female, who had been under observation for an indolent myeloma for six years without therapy, was admitted because of left flank pain. Various imaging modalities disclosed left pleural effusion, and a huge abdominal tumor involving the left crus of the diaphragm, spleen, stomach, pancreatic tail, left adrenal gland, left kidney and left posterior abdominal wall. Morphological and immunological examinations revealed extensive proliferation of more anaplastic myeloma cells within the tumor and in the pleural effusion than those in bone marrow on admission or at the beginning of the period of observation. Chemotherapy reduced serum M-protein, but LDH, the volume of the pleural effusion, and the size of the tumor increased. She died of pancreatitis associated with massive bleeding from the gastric tumor. Renal function was well preserved until her terminal phase. The clinical course showed the progression of indolent myeloma into an aggressive one without chemotherapeutic intervention, implying a rare subtype in terms of the natural history of multiple myeloma.