Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 113
About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Advertise Login 

 Table of Contents  
Year : 2016  |  Volume : 5  |  Issue : 2  |  Page : 140-141

Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol

1 Pediatric Hematology and Oncology Division, Diyarbakir Children's Hospital, Diyarbakir, Turkey
2 Radiology Division, Dicle University, Diyarbakir, Turkey

Date of Web Publication8-Apr-2016

Correspondence Address:
Gulen Tuysuz
Pediatric Hematology and Oncology Division, Diyarbakir Children's Hospital, Diyarbakir
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4847.179939

Rights and Permissions

How to cite this article:
Tuysuz G, Tayfun F, Goya C. Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol. J Clin Neonatol 2016;5:140-1

How to cite this URL:
Tuysuz G, Tayfun F, Goya C. Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol. J Clin Neonatol [serial online] 2016 [cited 2021 Apr 15];5:140-1. Available from: https://www.jcnonweb.com/text.asp?2016/5/2/140/179939


Dasgupta et al. [1] reported a case of infantile hepatic hemangioendothelioma (IHH) presenting with congestive cardiac failure who responded well to oral steroid treatment. Some new important treatment options should be discussed relating to this case.

We report an infant with hepatic hemangioendothelioma who presented with high output cardiac failure. She was resistant to, first line, steroid treatment but responded well to oral propranolol. The literature on treatment of hepatic hemangioendothelioma is scarce, and this case emphasizes the importance of propranolol treatment in IHH.

A 15-day-old girl was referred to our clinic because of feeding intolerance and abdominal distention. On physical examination, she had mild tachycardia, systolic cardiac murmur II/VI on apex, and edema on both legs. The liver was seven cm palpable below the right costal margin, and she had three cutaneous hemangiomas, 2-15 mm width on trunk [Figure 1]. Laboratory analysis including blood count, biochemistry, and coagulation tests was normal. Chest X-ray demonstrated cardiomegaly. Echocardiogram showed right ventricular dilatation and minimal tricuspid failure. Doppler ultrasonography (USG) and dynamic computed tomography of the abdomen revealed the diffuse involvement of the liver with hemangiomas [Figure 2]. Clinical and radiologic findings were consistent with IHH complicated with mild compensated high output cardiac failure. She was started on intravenous dopamine (10 μg/kg/min) to improve cardiac function. The patient had gone under Tru-Cut biopsy and histopathological examination of the specimen confirmed the diagnosis of hemangioendothelioma. The patient was put on oral prednisolone treatment (4 mg/kg/day; in two divided doses). Despite 14 days of treatment, the patient did not show any improvement; liver size, and focal lesions were stable on USG and the abdominal circumference was the same. The patient was evaluated as resistant to steroid and treatment was switched to oral propranolol. Propranolol was initially administered with 0.5 mg/kg/dose bid, gradually increased within two days and reached the target dose of 1 mg/kg/dose bid. At the 5 th day of treatment with propranolol, her symptoms improved rapidly (edema on legs resolved and liver size diminished on physical examination as well as on USG) allowing tapering and discontinuation of the inotropic drug. The patient is now on propranolol treatment for nine months. The latest USG showed involution of the lesions and also an overall decrease in the liver size (vertical size: 75 mm).
Figure 1: Excessive abdominal distention and dilated superficial abdominal wall veins due to enlarged liver are shown in the picture. Three cutaneous hemangiomas 0.2-1.5 cm width on trunk are also notable

Click here to view
Figure 2: Dynamic abdominal computed tomography: Pre-contrast scans shows diffuse liver enlargement with heterogeneous parenchyma and hyper dense septa. On arterial phase numerous hyper vascular nodular lesions nearly totally cover all the liver

Click here to view

Treatment of hepatic IHH previously was medical treatment with steroid, vincristine, interferon, hepatic arterial embolization or surgical resection. [2] Recent case studies showed the usage of Propranolol treatment in IHH as a first line therapy. [3],[4] Propranolol was reported to achieve a rapid response in severe IHH. [5] Partial or complete resolution of hepatic lesions was reported in all of the patients treated with Propranolol. [3],[4],[5] The drug is well tolerated even in infancy and severe side effects of long-term steroid, interferon alfa, or vincristine were not reported with propranolol treatment. [4] The response to propranolol treatment was prompt and efficient in our patient. Propranolol should be kept in mind in the treatment of patients with IHH.


Authors are thankful to all coworkers in Hematology-Oncology Division of Diyarbakir Children's Hospital for their cooperation and collaboration.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Dasgupta MK, Das S, Patra C, Sarkar S. Symptomatic infantile hepatic hemangioendothelioma successfully treated with steroid. J Clin Neonatol 2013;2:187-9.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Kim EH, Koh KN, Park M, Kim BE, Im HJ, Seo JJ. Clinical features of infantile hepatic hemangioendothelioma. Korean J Pediatr 2011;54:260-6.  Back to cited text no. 2
Sciveres M, Marrone G, Pipitone S, Riva S, Spada M, Gridelli B, et al. Successful first-line treatment with propranolol of multifocal infantile hepatic hemangioma with high-flow cardiac overload. J Pediatr Gastroenterol Nutr 2011;53:693-5.  Back to cited text no. 3
Mazereeuw-Hautier J, Hoeger PH, Benlahrech S, Ammour A, Broue P, Vial J, et al. Efficacy of propranolol in hepatic infantile hemangiomas with diffuse neonatal hemangiomatosis. J Pediatr 2010;157:340-2.  Back to cited text no. 4
Marsciani A, Pericoli R, Alaggio R, Brisigotti M, Vergine G. Massive response of severe infantile hepatic hemangioma to propanolol. Pediatr Blood Cancer 2010;54:176.  Back to cited text no. 5


  [Figure 1], [Figure 2]


Similar in PUBMED
  Search Pubmed for
  Search in Google Scholar for
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Article Figures

 Article Access Statistics
    PDF Downloaded173    
    Comments [Add]    

Recommend this journal