J Korean Surg Soc.  2010 Nov;79(5):399-404. 10.4174/jkss.2010.79.5.399.

The Experience of Non-surgical Treatment for Infantile Hepatic Hemangioendothelioma

• Nam SH
• Park JI
• Kim DY
• Kim SC
• Kim IK

• KMID: 2212051
• DOI: http://doi.org/10.4174/jkss.2010.79.5.399

Abstract

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 (KM SD). 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-alpha. 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-alpha, 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.

Keyword

Infantile hepatic hemangioendothelioma; Neonate liver tumor; Kasabach-Merritt syndrome

MeSH Terms

Birth Weight
Cyanosis
Heart
Heart Failure
Hemangioendothelioma
Hemangioma
Hepatic Artery
Hepatomegaly
Humans
Infant
Infant, Newborn
Interferon-alpha
Kasabach-Merritt Syndrome
Liver
Male
Prednisolone
Retrospective Studies
Interferon-alpha
Prednisolone

Reference

1. Dehner LP, Ishak KG. Vascular tumors of the liver in infants and children. A study of 30 cases and review of the literature. Arch Pathol. 1971. 92:101–111.

2. Stanley P, Gates GF, Eto RT, Miller SW. Hepatic cavernous hemangiomas and hemangioendotheliomas in infancy. AJR Am J Roentgenol. 1977. 129:317–321.

3. Dachman AH, Lichtenstein JE, Friedman AC, Hartman DS. Infantile hemangioendothelioma of the liver: a radiologic-pathologic-clinical correlation. AJR Am J Roentgenol. 1983. 140:1091–1096.

4. Kwon HJ, Moon SB, Park KW, Jung SE. Infantile hepatic hemangioendothelioma: seventeen years of experience at a single center. J Korean Assoc Pediatr Surg. 2008. 14:134–143.

5. Kim JH, Park YK, Choi CR, Lee C. A case report of hemangioendothelioma of the liver in an infant. J Korean Surg Soc. 1968. 10:519–522.

6. Daller JA, Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS, et al. Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg. 1999. 34:98–105.

7. Jackson C, Greene HL, O'Neill J, Kirchner S. Hepatic hemangioendothelioma. Angiographic appearance and apparent prednisone responsiveness. Am J Dis Child. 1977. 131:74–77.

8. Jung HJ, Yu JE, Pai KS, Ahn MS, Jung WC, Park MS, et al. Giant neonatal hemangioendothelioma of the liver diagnosed by 99mTc-RBC scintigraphy. J Korean Soc Neonatol. 2005. 12:105–111.

9. Roos JE, Pfiffner R, Stallmach T, Stuckmann G, Marincek B, Willi U. Infantile hemangioendothelioma. Radiographics. 2003. 23:1649–1655.

10. Zenge JP, Fenton L, Lovell MA, Grover TR. Case report: infantile hemangioendothelioma. Curr Opin Pediatr. 2002. 14:99–102.

11. Nguyen L, Shandling B, Ein S, Stephens C. Hepatic hemangioma in childhood: medical management or surgical management? J Pediatr Surg. 1982. 17:576–579.

12. Becker JM, Heitler MS. Hepatic hemangioendotheliomas in infancy. Surg Gynecol Obstet. 1989. 168:189–200.

13. Selby DM, Stocker JT, Waclawiw MA, Hitchcock CL, Ishak KG. Infantile hemangioendothelioma of the liver. Hepatology. 1994. 20:39–45.

14. Kim KU, Yoo SY, Cho MY. Two cases of infantile hemangioendothelioma of the liver. Korean J Gastroenterol. 1993. 25:1356–1361.

15. Holcomb GW 3rd, O'Neill JA Jr, Mahboubi S, Bishop HC. Experience with hepatic hemangioendothelioma in infancy and childhood. J Pediatr Surg. 1988. 23:661–666.

16. Goo JM, Kim WS, Kim IO, Yoon CH, Yeon KM, Choi CG. Infantile hemangioendothelioma of liver. J Korean Radiol Soc. 1993. 29:547–552.

17. Lucaya J, Enriquez G, Amat L, Gonzalez-Rivero MA. Computed tomography of infantile hepatic hemangioendothelioma. AJR Am J Roentgenol. 1985. 144:821–826.

18. Kim JS, Moon SK, Yoon HS, Lee TS. A case of infantile hemangioendothelioma of the liver treated with hepatic embolization and lobectomy. Korean J Pediatr. 2005. 48:660–664.

19. Weinreb JC, Cohen JM, Armstrong E, Smith T. Imaging the pediatric liver: MRI and CT. AJR Am J Roentgenol. 1986. 147:785–790.

20. Woltering MC, Robben S, Egeler RM. Hepatic hemangioendothelioma of infancy: treatment with interferon alpha. J Pediatr Gastroenterol Nutr. 1997. 24:348–351.

21. Park EA, Seo JW, Lee SW, Choi HY, Lee SJ. Infantile hemangioendothelioma treated with high dose methylprednisolone pulse therapy. J Korean Med Sci. 2001. 16:127–129.

22. van der Meijs BB, Merks JH, de Haan TR, Tabbers MM, van Rijn RR. Neonatal hepatic haemangioendothelioma: treatment options and dilemmas. Pediatr Radiol. 2009. 39:277–281.

23. Touloukian RJ. Hepatic hemangioendothelioma during infancy: pathology, diagnosis and treatment with prednisone. Pediatrics. 1970. 45:71–76.

24. Zweifach BW, Shorr E, Black MM. The influence of the adrenal cortex on behavior of terminal vascular bed. Ann N Y Acad Sci. 1953. 56:626–633.

25. Rocchini AP, Rosenthal A, Issenberg HJ, Nadas AS. Hepatic hemangioendothelioma: hemodynamic observations and treatment. Pediatrics. 1976. 57:131–135.

26. Yoon HS, Lee JH, Moon HN, Seo JJ, Im HJ, Goo HW. Successful treatment of retroperitoneal infantile hemangioendothelioma with Kasabach-Merritt syndrome using steroid, alpha-interferon, and vincristine. J Pediatr Hematol Oncol. 2009. 31:952–954.

27. Fok TF, Chan MS, Metreweli C, Ng PC, Yeung CK, Li AK. Hepatic haemangioendothelioma presenting with early heart failure in a newborn: treatment with hepatic artery embolization and interferon. Acta Paediatr. 1996. 85:1373–1375.

28. Friesel R, Komoriya A, Maciag T. Inhibition of endothelial cell proliferation by gamma-interferon. J Cell Biol. 1987. 104:689–696.

29. Folkman J. Successful treatment of an angiogenic disease. N Engl J Med. 1989. 320:1211–1212.

30. Fellows KE, Hoffer FA, Markowitz RI, O'Neill JA Jr. Multiple collaterals to hepatic infantile hemangioendotheliomas and arteriovenous malformations: effect on embolization. Radiology. 1991. 181:813–818.