소아의 흉벽에서 진단된 평활근육종 1례

Leiomyosarcoma is an uncommon soft tissue sarcoma of mesenchymal cell origin, which shows smooth muscle differentiation. Leiomyosarcoma is seldom found in the pediatric population, and accounts for fewer than 2% of all soft tissue sarcomas. Leiomyosarcoma of the chest wall is extremely rare in children. We report here a case of an 8-year-old boy with a primary leiomyosarcoma that was incidentally found as a rib mass. The patient underwent a complete resection for a suspected osteochondroma diagnosed by a three-dimensional chest computed tomography examination. Pathological findings of the mass revealed intersecting fascicles of spindle cells showing cigar-shaped nuclei, inconspicuous nuclear pleomorphism and occasional mitotic figures in the background of a suspected osteochondroma of the rib. This report documents the first description of a leiomyosarcoma possibly arising in an osteochondroma of the rib in a child. 평활근육종은 중간엽세포 기원의 평활근 분화를 보이는 연부조직육종으로 소아에서는 전체 연부조직육종의 2% 이하의 발생률을 보이는 매우 드문 질환이다. 특히 흉벽의 평활근육종은 더욱 드물다. 저자들은 우연히 발견된 흉벽 종양을 방사선학적 검사로 골연골종으로 추측하여 완전절제술을 실시하였고, 조직검사 결과 저등급 평활근육종으로 진단된 1례가 있어 보고하는 바이다.