양측 흉막에 발생한 결합조직형성 소원형세포종양의 증례 보고

Abstract

Desmoplastic small round cell tumor (DSRCT) is a highly aggressive malignant small cell neoplasm occurring mainly in the abdominal cavity, but it is extremely rare in the pleura. In this case, a 15-year-old male presented with a 1-month history of left chest pain. Chest radiographs revealed pleural thickening in the left hemithorax and chest computed tomography showed multifocal pleural thickening with enhancement in both hemithoraces. A needle biopsy of the left pleural lesion was performed and the final diagnosis was DSRCT of the pleura. We report this unusual case arising from the pleura bilaterally. The pleural involvement of this tumor supports the hypothesis that it typically occurs in mesothelial-lined surfaces.