Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Intrathoracic Desmoid Tumor: A Case Report and Radioligical Evaluation

흉강내 발생한 데스모이드종양의 증례보고와 영상소견에 관한 고찰

Kim, Min-Ji;Park, Kyung-Joo;Sun, Joo-Sung;Kim, Jang-Hee;Choi, Ho
김민지;박경주;선주성;김장희;최호

  • Published : 2007.07.01
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Abstract

Desmoid tumors are rare soft tissue tumors arising from the fascia or from musculoaponeurotic structures. They are commonly seen in the extremities, but are rarely found in the thorax. Thoracic desmoid tumors commonly arise from the chest wall and rarely in the thoracic cavity. Imaging diagnosis of an intrathoracic desmoid tumor is difficult because there are no specific imaging findings for a desmoid tumor that can be differentiated from the various tumors of the chest wall, including a solitary fibrous tumor of the pleura. All desmoid tumor cells show negative immunohistochemical staining for CD34 in pathological specimen, a feature that makes it possible to differentiate a desmoid tumor from a solitary fibrous tumor of the pleura. Desmoid tumors are locally aggressive and the rate of local recurrence is very high. Consequently, wide radical resection is required and a preoperative accurate diagnosis of desmoid tumors is warranted. We describe the radiological findings of various imaging studies for an intrathoracic desmoid tumor. Our findings should facilitate a proper diagnosis of desmoid tumors.

데스모이드종양(desmoid tumor)는 근막 혹은 근육 및 건막에서 기원하는 드문 연조직 종양이다. 데스모이드종양은 주로 사지에 자주 생기고 흉부에는 드물게 발견되며 흉부에서 데스모이드종양이 발생할 경우 대개 흉벽에서 발생한다. 흉강 내 종괴로 발현하는 데스모이드종양은 더욱 드물며 흉막에서 발생하는 국한성 섬유종양을 비롯한 다른 종양들과 비교하여 영상 소견에 특이한 감별점이 없어 진단이 어렵다. 병리검사에서 데스모이드종양의 모든 종양세포는 특징적으로 CD 34에 대한 조직면역화학염색에 음성을 보여, 흉막에서 발생하는 국한성 섬유종양과의 감별점이 된다. 데스모이드종양은 매우 국소적으로 침습적이며 국소 재발률이 높다. 따라서 확장근치절제술이 필요하고 이를 위해서는 수술 전 정확한 진단이 필수적이다. 따라서 저자들이 경험한 흉강 내 데스모이드종양의 다양한 영상검사 소견을 문헌 고찰과 함께 보고하고자 한다.

Keywords

References

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