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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 14  |  Issue : 3  |  Page : 144-147

Infantile hemangioendothelioma of the liver in a Nigerian newborn: A case report


1 Department of Pediatrics, College of Medicine, University of Lagos/Lagos University Teaching Hospital, Lagos, Nigeria
2 Department of Pediatrics, Lagos University Teaching Hospital, Lagos, Nigeria

Date of Web Publication17-Aug-2017

Correspondence Address:
Beatrice Nkolika Ezenwa
Department of Pediatrics, College of Medicine, University of Lagos/Lagos University Teaching Hospital, Lagos
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2468-6859.213084

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  Abstract 


Hepatic hemangioendothelioma is the most common benign vascular tumor of the liver in infancy and it is the most likely of all hepatic tumors to present with symptoms early in life, presenting in the first 6 months of life. It may present with life-threatening complications such as congestive cardiac failure and thrombocytopenia. As a way of creating awareness to the existence of this rare condition in our environment, we report a case of symptomatic hemangioendothelioma in a newborn and challenges that can be encountered during management in a resource poor-setting. Baby OA is a 22-day-old female neonate who presented with an abdominal mass noted at birth. Diagnosis was made based on clinical findings and results of abdominal ultrasound scan and computerized tomography scan which were in keeping with a hepatic hemangioendothelioma. She was treated with oral prednisolone, propranolol, and aggressive supportive treatment with blood and platelet concentrate transfusions. Her management was hampered by challenges in obtaining a histologic diagnosis, financial constraints, and unavailability of wider therapeutic options. She has been discharged and is being followed-up at the out-patient clinics. Hemangioendothelioma should be considered in the differential diagnosis of infants presenting with an abdominal mass in the newborn period as it could be associated with significant morbidity and mortality requiring aggressive treatment and meticulous supportive care.

Keywords: Hemangioendothelioma, newborn, tumor


How to cite this article:
Ezenwa BN, Nwaogazie U, Akintan P, Fajolu I, Ezeaka C, Adeniyi O. Infantile hemangioendothelioma of the liver in a Nigerian newborn: A case report . J Clin Sci 2017;14:144-7

How to cite this URL:
Ezenwa BN, Nwaogazie U, Akintan P, Fajolu I, Ezeaka C, Adeniyi O. Infantile hemangioendothelioma of the liver in a Nigerian newborn: A case report . J Clin Sci [serial online] 2017 [cited 2019 Oct 15];14:144-7. Available from: http://www.jcsjournal.org/text.asp?2017/14/3/144/213084




  Introduction Top


Hepatic hemangioendothelioma although rare, is the most common benign vascular tumor of the liver in infancy and the most common symptomatic hepatic tumor presenting in the first 6 months of life.[1],[2],[3],[4] Its propensity for presenting with life-threatening complications such as congestive cardiac failure and thrombocytopenia should heighten clinical suspicion of this condition in newborns presenting with an abdominal mass.

Hemangioendothelioma is a benign vascular tumor that arises from mesenchymal tissues of the liver. It is reported to occur in 1/20,000 infants [5] with a female preponderance of 2:1.[6] Unlike other hemangiomas which are more common in whites,[7] no racial predilection is known for hemangioendothelioma, and genetic transmission has not been described.[6]

Due to the rarity of the condition, there is no structured research into options for therapy, but a small number of cases have been reported to respond well to treatment with steroid therapy. In a bid to create awareness of the existence of this rare condition in our environment, we report a case of symptomatic hemangioendothelioma in a newborn and challenges that can be encountered during management in our environment.


  Case Report Top


Baby OA a 22-day-old female neonate presented to the children emergency center of LUTH on the 4th day of life with a history of abdominal distention noticed at birth and bleeding from the nostril noted on the 2nd day of life. She was a 2.9 kg term female neonate delivered through emergency cesarean section on an account of cephalopelvic disproportion and polyhydramnios to a 30-year-old mother in a private hospital. Antenatal period was said to be essentially uneventful with a normal antenatal ultrasound and no history of exposure to known teratogens. She was referred on account of the abdominal distention which was noticed at birth.

Physical examination at presentation was remarkable for jaundice, pallor, abdominal distention, and a mass in the right hypochondrium. The complete blood count showed packed cell volume of 30%, white blood cell - 7100/mm3, and platelet count - 50 × 109/L. Abdominal ultrasound scan (USS) performed revealed a large, fairly well-defined, oval-shaped, heterogeneous mass (54 mm × 86 mm) with multiple cystic areas arising from the right lobe of the liver with moderate vascularity on Doppler USS. Computerized tomography (CT) scan showed gross hepatomegaly with a large spherical hypo-attenuated mass (71.3 mm × 69 mm × 88.9 mm) with a central area of hypodensity occupying the entire right lobe of the liver; administration of contrast medium showed peripheral enhancement with a centripetal filling defect [Figure 1] and [Figure 2].
Figure 1: Large liver mass

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Figure 2: Mass with peripheral enhancement

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Other investigations performed were as follows: E and U/creatinine: Na - 134 mmol/L, K - 5.4 mmol/L, Cl - 105 mmol/L, HCO3 21 mmol/L, urea - 6.4 mmol/L, and Cr - 29 umol/L. Serum alpha fetoprotein was high - 59632 ng/ml. Clotting profile – prothrombin time - 39 s (control - 16.5 s), international normalized ratio - 3.11. Liver function tests revealed serum glutamic oxaloacetic transaminase - 30 U/L, serum glutamic pyruvic transaminase - 4 U/L, alkaline phosphatase - 18.8 U/L, total protein - 66.7 g/L, albumin - 35 g/L, and total serum bilirubin - 11.3 mg/dl. Thyroid function test: Thyroid stimulating hormone - 1.01 mIU/L, free T3- 2.1 pmol/L, and free T4- 11.6 pmol/L.

Baby had repeated transfusions with packed red blood cell, platelet concentrate, and fresh frozen plasma. She received intravenous Vitamin K, oral prednisolone (1 mg/kg/day), and propranolol (2 mg/kg/day). Thrombocytopenia was persistent with minimal improvement with prednisolone. Patient was co-managed with the pediatric hematologists and gastroenterologist. Parents were extensively counseled on the patient's diagnosis and the proposed line of management.

A liver biopsy was done under ultrasound guidance after optimizing coagulation profile and platelet counts; however, minimal liver tissue could be obtained due to the massive size of the cyst-like mass within the liver. The liver tissue was quite rudimentary and the vascular mass which appeared cystic on the USS filled the whole of the liver and it was difficult to obtain a sizeable liver tissue. Sample obtained was insufficient for a histopathologic diagnosis. Attempts to repeat liver biopsy were unsuccessful as the clotting profile worsened. Abdominal distention became static and after 29 days on admission father requested for out-patient management of the patient and refused any other in-hospital treatment due to financial constraints. Baby has been seen severally in the out-patient clinics, was re-admitted once for blood transfusion due to symptomatic anemia and still has the huge abdominal mass. Parents are still being encouraged to give consent for a repeat liver biopsy and have been counseled on the need for a liver transplant.


  Discussion Top


Hepatic hemangioendothelioma is more prevalent in females with a 2:1 ratio and no racial predilection has been described.[1],[8]

The clinical spectrum in neonates with hemangioendothelioma ranges from being asymptomatic to having life-threatening symptoms of consumptive thrombocytopenia and coagulopathy and congestive cardiac failure.[9] Other clinical features include hypothyroidism from inhibition of thyroid hormone by deiodinase secreted by the tumor, jaundice, anemia, abnormal liver function, multiple cutaneous hemangiomas, with a palpable abdominal mass, hepatomegaly, or diffuse abdominal enlargement [10],[11] being the commonest presenting feature. Our patient had a myriad of these clinical features. She had no cutaneous hemangioma; although, some authors have reported 40%–50% of prevalence in other studies.[12]

Imaging modalities play a crucial role in establishing a diagnosis as liver biopsy for histopathologic diagnosis may be technically difficult. Feng et al.[8] in a study of CT and magnetic resonance imaging (MRI) examinations of infants with biopsy proven hemangioendotheliomas demonstrated that CT and MRI imaging display typical features (lesions are most often hypodense and show calcifications on unenhanced CT) that allow accurate diagnosis of this condition. Calcifications seen on imaging are often fine and lobular, in contrast, to the coarse calcifications suggestive of hepatoblastoma.[3] Lesions appear as a large heterogeneous mass with areas of mixed echogenicity on USS. Our patient's lesion demonstrated some of these features.

Alpha-fetoprotein, an important tumor marker for most malignant hepatic tumors such as hepatoblastoma should be included in the work up for any hepatic tumor. The level of this tumor marker is usually normal in hemangioendothelioma.[11] The high level noted in our patient is likely due to the age of our patient as serum levels of alpha-fetoprotein, which are usually high in-utero, do not return to normal until 8–12 months after birth.

Treatment of infantile hepatic hemangioendotheliomas has remained controversial. The natural history of hepatic hemangioendotheliomas varies, but up to two-thirds of symptomatic patients, especially those with heart failure may die.[13],[14] Although children with asymptomatic lesions may experience spontaneous regression within a year, those with symptomatic lesions require aggressive management due to the risk of death once symptoms commence.[11] While close follow up with USS monitoring of the mass is sufficient for asymptomatic lesions, supportive care is paramount for symptomatic cases with aggressive treatment with blood and platelet concentrate transfusions when indicated. Systemic steroids have been the main stay of therapy for symptomatic cases as it has been shown to shrink the tumor. Although, mechanism of its action is not known prednisolone is given in a dose of 2–3 mg/kg/day in divided doses and slowly tapered over 2–3 months.[15] More recently, studies have shown remarkable response of hepatic hemangioma to oral propanolol.[16],[17] In our patient treated with prednisolone and propranolol; although, the tumor did not regress appreciably, its further growth was arrested as its size did not increase again after 4 weeks of treatment. However, complications of recurrent severe anemia and thrombocytopenia persisted. Alpha interferon has also been tried in severe symptomatic cases unresponsive to steroids.[7],[16],[17] Other modalities of treatment include selective hepatic artery embolization and surgical resection of the tumor.[11]


  Conclusion Top


Hemangioendothelioma should be considered in the differential diagnosis of infants presenting with an abdominal mass in the newborn period as it could be associated with significant morbidity and mortality requiring aggressive treatment and meticulous supportive care.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Braun P, Ducharme JC, Riopelle JL, Davignon A. Hemangiomatosis of the liver in infants. J Pediatr Surg 1975;10:121-6.  Back to cited text no. 1
    
2.
Zenge JP, Fenton L, Lovell MA, Grover TR. Case report: Infantile hemangioendothelioma. Curr Opin Pediatr 2002;14:99-102.  Back to cited text no. 2
    
3.
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. 3
    
4.
Roos JE, Pfiffner R, Stallmach T, Stuckmann G, Marincek B, Willi U. Infantile hemangioendothelioma. Radiographics 2003;23:1649-55.  Back to cited text no. 4
    
5.
Selby DM, Stocker JT, Waclawiw MA, Hitchcock CL, Ishak KG. Infantile hemangioendothelioma of the liver. Hepatology 1994;20(1 Pt 1):39-45.  Back to cited text no. 5
    
6.
Dachman AH, Lichtenstein JE, Friedman AC, Hartman DS. Infantile hemangioendothelioma of the liver: A radiologic-pathologic-clinical correlation. AJR Am J Roentgenol 1983;140:1091-6.  Back to cited text no. 6
    
7.
Antaya RJ, James WD, Ortonne JP. Infantile Hemangioma. Available from: http://www.emedicine.medscape.com/article/1083849-overview#a2. [Last accessed on 2016 Apr 10].  Back to cited text no. 7
    
8.
Reddy KR, Kligerman S, Levi J, Livingstone A, Molina E, Franceschi D, et al. Benign and solid tumors of the liver: Relationship to sex, age, size of tumors, and outcome. Am Surg 2001;67:173-8.  Back to cited text no. 8
    
9.
Sari N, Yalçin B, Akyüz C, Haliloglu M, Büyükpamukçu M. Infantile hepatic hemangioendothelioma with elevated serum alpha-fetoprotein. Pediatr Hematol Oncol 2006;23:639-47.  Back to cited text no. 9
    
10.
Chirdan LB, Uba AF, Bode-Thomas F, Mandong BM. Infantile hepatic haemangioendothelioma: A case report. Niger J Surg Res 2004;6:69-70.  Back to cited text no. 10
    
11.
Feng ST, Chan T, Ching AS, Sun CH, Guo HY, Fan M, et al. CT and MR imaging characteristics of infantile hepatic hemangioendothelioma. Eur J Radiol 2010;76:e24-9. doi: 10.1016/j.ejrad.2010.05.027.  Back to cited text no. 11
    
12.
Lee TC, Barshes NR, Agee EE, O'Mahony CA, Karpen SJ, Carter BA, et al. Resolution of medically resistant hypothyroidism after liver transplantation for hepatic hemangioendothelioma. J Pediatr Surg 2006;41:1783-5.  Back to cited text no. 12
    
13.
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.  Back to cited text no. 13
    
14.
Sevinir B, Ozkan TB. Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turk J Gastroenterol 2007;18:182-7.  Back to cited text no. 14
    
15.
Sadan N, Wolach B. Treatment of hemangiomas of infants with high doses of prednisone. J Pediatr 1996;128:141-6.  Back to cited text no. 15
    
16.
Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taéeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358:2649-51.  Back to cited text no. 16
    
17.
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. 17
    


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