지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2010.11
- 수록면
- 399 - 404 (6page)
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Purpose: Hepatic hemangioendothelioma (HET) is a rare benign vascular tumor in infants. The clinical manifestations are variable from asymptomatic to life-threatening heart failure or Kasabach-Merritt syndrome (KMSD). We report our non-surgical treatment experience for infantile HET.
Methods: We analyzed 15 infants (5 males, 10 females) of HET retrospectively, from January 1989 to December 2007.
Results: All except 2 were full-term babies and median birth weight was 3,140 g. The median age at diagnosis was 15 days of life (0∼157 days). Three of 5 patients diagnosed prenatally presented heart failure symptoms and the remaining 2 showed KM SD. Among 6 neonates, 3 showed cyanosis due to congestive heart failure. Nine patients showed multiple nodules at both liver lobes. Three asymptomatic patients were followed with radiologic examinations, thus HETs regressed spontaneously in two and decreased in one. Four cases that showed hepatomegaly or increase in size of subcutaneous hemangioma, were treated with prednisolone. All patients improved from tumor related symptoms and tumor size decreased. Four patients (2 patients of heart failure, 1 with rapid progressive hepatomegaly and 1 with congenital heart disease) received interferon-α. Among them, 3 showed regression of tumor and the remaining 1 showed decrease of tumor size. Two patients who suffered from severe heart failure received prednisolone and interferon-α, and then recovered. In the 2 patients with KM SD, we tried hepatic artery embolization with medical treatment. One survived but the other is not followed after failure of embolization.
Conclusion: We experienced that many patients who had clinical symptoms associated with HET very early in their life treated with an aggressive nonsurgical treatment in symptomatic patients showed favorable outcome.
Methods: We analyzed 15 infants (5 males, 10 females) of HET retrospectively, from January 1989 to December 2007.
Results: All except 2 were full-term babies and median birth weight was 3,140 g. The median age at diagnosis was 15 days of life (0∼157 days). Three of 5 patients diagnosed prenatally presented heart failure symptoms and the remaining 2 showed KM SD. Among 6 neonates, 3 showed cyanosis due to congestive heart failure. Nine patients showed multiple nodules at both liver lobes. Three asymptomatic patients were followed with radiologic examinations, thus HETs regressed spontaneously in two and decreased in one. Four cases that showed hepatomegaly or increase in size of subcutaneous hemangioma, were treated with prednisolone. All patients improved from tumor related symptoms and tumor size decreased. Four patients (2 patients of heart failure, 1 with rapid progressive hepatomegaly and 1 with congenital heart disease) received interferon-α. Among them, 3 showed regression of tumor and the remaining 1 showed decrease of tumor size. Two patients who suffered from severe heart failure received prednisolone and interferon-α, and then recovered. In the 2 patients with KM SD, we tried hepatic artery embolization with medical treatment. One survived but the other is not followed after failure of embolization.
Conclusion: We experienced that many patients who had clinical symptoms associated with HET very early in their life treated with an aggressive nonsurgical treatment in symptomatic patients showed favorable outcome.
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REFERENCES
참고문헌 (30)
참고문헌 신청
1. [학술지(정기간행물)] - Dehner LP / 1971 / Vascular tumors of the liver in infants and children. A study of 30 cases and review of the literature / Arch Pathol 92 : 101 ~ 111
2. [학술지(정기간행물)] - Stanley P / 1977 / Hepatic cavernous hemangiomas and hemangioendotheliomas in infancy / AJR Am J Roentgenol 129 : 317 ~ 321
3. [학술지(정기간행물)] - Dachman AH / 1983 / Infantile hemangioendothelioma of the liver: a radiologic- pathologic-clinical correlation / AJR Am J Roentgenol 140 : 1091 ~ 1096
4. [학술지(정기간행물)] - Kwon HJ / 2008 / Infantile hepatic hemangioendothelioma: seventeen years of experience at a single center / J Korean Assoc Pediatr Surg 14 : 134 ~ 143
5. [학술지(정기간행물)] - Kim JH / 1968 / A case report of hemangioendothelioma of the liver in an infant / J Korean Surg Soc 10 : 519 ~ 522
2. [학술지(정기간행물)] - Stanley P / 1977 / Hepatic cavernous hemangiomas and hemangioendotheliomas in infancy / AJR Am J Roentgenol 129 : 317 ~ 321
3. [학술지(정기간행물)] - Dachman AH / 1983 / Infantile hemangioendothelioma of the liver: a radiologic- pathologic-clinical correlation / AJR Am J Roentgenol 140 : 1091 ~ 1096
4. [학술지(정기간행물)] - Kwon HJ / 2008 / Infantile hepatic hemangioendothelioma: seventeen years of experience at a single center / J Korean Assoc Pediatr Surg 14 : 134 ~ 143
5. [학술지(정기간행물)] - Kim JH / 1968 / A case report of hemangioendothelioma of the liver in an infant / J Korean Surg Soc 10 : 519 ~ 522
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