Congenital Hemagiopericytoma in the Flexor Digitorium Profundus Muscle of the Distal Forearm - A Case Report -

전완 원위부 심 수지 굴근에 발생한 선천성 혈관 외피세포종 - 증례 보고 -

  • Kim, Jung-Ryul (Department of Orthopedic Surgery, Chonbuk National University, Medical School, Research Institute of Clinical Medicine) ;
  • Jang, Kyu-Yun (Department of Pathology, Chonbuk National University, Medical School, Research Institute of Clinical Medicine) ;
  • Lee, Sang-Yong (Department of Radiology, Chonbuk National University, Medical School, Research Institute of Clinical Medicine)
  • 김정렬 (전북대학교 의학전문대학원 정형외과학교실) ;
  • 장규윤 (전북대학교 의학전문대학원 병리학교실) ;
  • 이상용 (전북대학교 의학전문대학원 영상의학교실)
  • Published : 2007.12.30

Abstract

Hemangiopericytoma is a rare malignant vascular tumor that usually occurs in adults. The occurrence of these tumors in infants, known as congenital or infantile hemangiopericytoma, is even rare and their behavior may be more benign than the adult type. Despite of the generally good prognosis associated with this neoplasm, a complete surgical excision has so far been recommended to avoid recurrence, because no definite criteria for determining whether or not the tumor will regress spontaneously have been established to date. We describe a 1-day-old male neonate with congenital hemangiopericytoma, presenting with a left forearm mass at birth. Wide resection was performed at 65 days of age and hemangiopericytoma was diagnosed by histology. There was no tumor recurrence during 32 months of follow-up.

혈관 외피 세포종은 드문 악성 혈관 종양으로 대부분 성인에서 발생한다. 영아에서 발생하는 경우는 선천성 또는 영아형 혈관 주변 세포종이라 불리우며, 아주 드물게 발생하고 성인형에 비해 양호한 예후를 보이지만 재발을 막기 위해서는 충분한 절제연을 가지고 절제 되어져야 한다. 본 증례는 출생후 1일째 내원하여, 65일째 광범위 절제술을 시행한 좌측 전완부의 혈관 외피 세포종의 증례를 보고하고자 한다.

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