고립성 골수외 형질세포종은 드문 형질세포질환으로 일반적으로 상기도 점막에서 발생하고, 서서히 진행하는 것으로 알려져 있다. 하지만 본 환자는 그와 달리 거대하고 진행이 매우 빠른 후복막 고립성 골수외 형질세포종이었기에 문헌고찰과 함께 보고하는 바이다.

Extramedullary plasmacytoma (EMP) represent 3% of plasma cell neoplasms. Approximately 80-90% of EMPs involve the mucosa- associated lymphoid tissue of the upper airways and 75% of these involve the nasal and paranasal regions, while renal or retroperitoneal infiltration is very rare. EMPs are highly radiosensitive, with excellent results. The local control rate of radiotherapy can reach 90-97% and the 5-year overall survival rate can be 57-61%. EMP has an indolent course and the prognosis is generally favorable, with 70% of the patients remaining disease free for 10 years. However, about 30% may progress to plasma cell myeloma or relapse. Here, we report a case of a huge retroperitoneal solitary extramedullary plasmacytoma that grew rapidly. (Korean J Med 2016;90:253-257)