Journal of Clinical Neonatology

LETTER TO EDITOR
Year
: 2016  |  Volume : 5  |  Issue : 2  |  Page : 140--141

Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol


Gulen Tuysuz1, Funda Tayfun1, Cemil Goya2,  
1 Pediatric Hematology and Oncology Division, Diyarbakir Children's Hospital, Diyarbakir, Turkey
2 Radiology Division, Dicle University, Diyarbakir, Turkey

Correspondence Address:
Gulen Tuysuz
Pediatric Hematology and Oncology Division, Diyarbakir Children«SQ»s Hospital, Diyarbakir
Turkey




How to cite this article:
Tuysuz G, Tayfun F, Goya C. Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol.J Clin Neonatol 2016;5:140-141


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 Mar 3 ];5:140-141
Available from: https://www.jcnonweb.com/text.asp?2016/5/2/140/179939


Full Text

Sir,

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}{Figure 2}

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.

Acknowledgment

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Dasgupta MK, Das S, Patra C, Sarkar S. Symptomatic infantile hepatic hemangioendothelioma successfully treated with steroid. J Clin Neonatol 2013;2:187-9.
2Kim EH, Koh KN, Park M, Kim BE, Im HJ, Seo JJ. Clinical features of infantile hepatic hemangioendothelioma. Korean J Pediatr 2011;54:260-6.
3Sciveres 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.
4Mazereeuw-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.
5Marsciani A, Pericoli R, Alaggio R, Brisigotti M, Vergine G. Massive response of severe infantile hepatic hemangioma to propanolol. Pediatr Blood Cancer 2010;54:176.