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Cancer of Unknown Primary Site Mimicking Retroperitoneal Fibrosis

후복막강섬유화증으로 오인된 원발 부위 불명암 1예

  • Park, Chan Keol (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital) ;
  • Yoo, Su-Jin (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital) ;
  • Yoo, In Seol (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital) ;
  • Kim, Jinhyun (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital) ;
  • Shim, Seung Cheol (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital) ;
  • Yeo, Min-kyung (Department of Pathology, Chungnam National University Hospital) ;
  • Kang, Seong Wook (Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital)
  • 박찬걸 (충남대학교병원 류마티스내과) ;
  • 유수진 (충남대학교병원 류마티스내과) ;
  • 유인설 (충남대학교병원 류마티스내과) ;
  • 김진현 (충남대학교병원 류마티스내과) ;
  • 심승철 (충남대학교병원 류마티스내과) ;
  • 여민경 (충남대학교병원 병리과) ;
  • 강성욱 (충남대학교병원 류마티스내과)
  • Received : 2017.12.05
  • Accepted : 2018.03.27
  • Published : 2018.12.01

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterized by marked fibro-inflammatory tissue in the retroperitoneum. Approximately 70% of cases of RPF are idiopathic, while the rest can be secondary to several other causes. The diagnosis is mainly obtained by imaging modalities such as computed tomography (CT). However, histological examination should be considered when the clinical manifestations and imaging studies suggest malignancy. In particular, in malignant diseases with retroperitoneal metastases, abnormal collagen plaques are formed from an exuberant desmoplastic response, which may not be distinguishable from RPF on CT scans. Therefore, even if CT suggests RPF, biopsy is essential to identify malignant disease because it typically results in a fatal prognosis. Here, we report a case of metastatic adenocarcinoma of unknown primary site that developed only in the retroperitoneum and was initially diagnosed as RPF based on CT findings.

저자들은 최초 복부 CT 소견상 후복막강섬유화증으로 오인되었으나 적극적인 조직 검사를 통하여 후복막강에만 국한된 원발 부위 불명 전이암으로 진단된 증례를 경험하였다. 최근 후복막강섬유화증의 진단에 있어서 PET-CT 등 영상 검사가 기술적으로 많은 발전을 이루었지만 후복막강섬유화증의 진단시 악성종양의 배제는 필수적이기 때문에 후복막강섬유화증의 확진은 반드시 조직학적인 검사에 기초하여 이루어져야 함을 강조하는 바이다.

Keywords

References

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