신장외 악성 횡문양 종양 - 1예 보고 -

Extrarenal Malignant Rhabdoid Tumor - A Case Report -

  • 이상용 (동아대학교 의과대학 병리학교실) ;
  • 김대철 (동아대학교 의과대학 병리학교실) ;
  • 나서희 (동아대학교 의과대학 병리학교실) ;
  • 홍숙희 (동아대학교 의과대학 병리학교실) ;
  • 강태훈 (동아대학교 의과대학 소아과학교실) ;
  • 이영호 (동아대학교 의과대학 소아과학교실) ;
  • 남경진 (동아대학교 의과대학 방사선과학교실) ;
  • 정진숙 (동아대학교 의과대학 병리학교실)
  • Lee, Sang-Yong (Departments of Pathology, Dong-A University College of Medicine) ;
  • Kim, Dae-Cheol (Departments of Pathology, Dong-A University College of Medicine) ;
  • Rha, Seo-Hee (Departments of Pathology, Dong-A University College of Medicine) ;
  • Hong, Sook-Hee (Departments of Pathology, Dong-A University College of Medicine) ;
  • Kang, Tae-Hun (Departments of Pediatrics, Dong-A University College of Medicine) ;
  • Lee, Young-Ho (Departments of Pediatrics, Dong-A University College of Medicine) ;
  • Nam, Kyoung-Jin (Departments of Radiology, Dong-A University College of Medicine) ;
  • Jeong, Jin-Sook (Departments of Pathology, Dong-A University College of Medicine)
  • 발행 : 1996.06.29

초록

Malignant rhabdold tumor is a distinct renal tumor in the pediatric age group. It was originally described as a rhabdomyosarcomatold variant of Wilms' tumor. However, subsequent studies fatted to confirm myogenous differentiation, so it is now considered to be a distinct and unique type of highly malignant tumor, histogenetically unrelated. Although extrarenal forms of this tumor are rare, several examples have been described in other sites, especially the liver, prostate, paravertebral area, urinary bladder and soft tissue. We experienced a case of malignant rhabdiod tumor located in the intraabdominal cavity in a 10 month-old boy. Smear of peritoneal fluid showed round, polygonal and irregular shaped cells with large nuclei, ample cytoplasm containing light pink to purple cytoplasmic inclusions, and one or a few prominent nucleoli. Immunocytochemistry revealed positivity to cytokeratin, epithelial membrane antigen and vimentin, and negativity to desmin and neuron-specific enolase. These distinct cytologic appearance and immunophenotypes were most consistent with a diagnosis of extrarenal malignant rhabdoid tumor. The cytoplasmic inclusions were correlated with eosinophilic inclusions seen in histologic section and electron microscopy confirmed this interpretation, showing filamentous aggregations in the cytoplasms of the tumor cells.

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