|Year : 2018 | Volume
| Issue : 4 | Page : 1479-1481
Hepatoblastoma misdiagnosed as infantile hemangioendothelioma in a child – a case report
Aditya P Singh, Arun Gupta, Vinay Mathur, Dinesh K Barolia
Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
|Date of Submission||10-Jun-2017|
|Date of Acceptance||18-Feb-2018|
|Date of Web Publication||14-Feb-2019|
Aditya P Singh
Near The Mali Hostel, Main Bali Road, Falna, Pali District, Rajasthan
Source of Support: None, Conflict of Interest: None
Infantile hemangioendothelioma (IHH) is the most common benign tumor of the liver in children, which accounts for 12% of all pediatric liver tumors, and hepatoblastoma (HBL) is the most common malignant liver tumor in children. Although they share some clinical manifestations, the treatment strategies and prognoses are quite different. The most important alternative diagnosis to IHH in children is HBL. Histopathologic confirmation of a radiologically suspected hemangioendothelioma is often obtained before medical treatment is initiated. We are presenting here a case of HBL in a 2-year-male child misdiagnosed as having IHH on imaging studies.
Keywords: hemangioendothelioma, hepatoblastoma, infantile, misdiagnosed
|How to cite this article:|
Singh AP, Gupta A, Mathur V, Barolia DK. Hepatoblastoma misdiagnosed as infantile hemangioendothelioma in a child – a case report. Menoufia Med J 2018;31:1479-81
|How to cite this URL:|
Singh AP, Gupta A, Mathur V, Barolia DK. Hepatoblastoma misdiagnosed as infantile hemangioendothelioma in a child – a case report. Menoufia Med J [serial online] 2018 [cited 2021 Jun 21];31:1479-81. Available from: http://www.mmj.eg.net/text.asp?2018/31/4/1479/252056
| Introduction|| |
Liver tumors are rare in children, and they account for ∼5–6% of all intra-abdominal masses in children; moreover, two-thirds of the pediatric primary liver masses are malignant. Infantile hepatic hemangioendothelioma (IHH) is the most common benign liver tumor in children with a peak presentation at 6 months of age. Hepatoblastoma (HBL) is the most common malignant pediatric liver tumors, and it is also the third most common fetal and neonatal liver tumor,,. The final diagnosis of pediatric liver tumors should be made in a stepwise approach and based on clinical features including age, sex and serum α-fetoprotein (AFP) level and imaging characteristics with histopathological confirmation.
| Case Report|| |
A 2-year-old male child was brought to us by his parents with the complaints of fever with pain in the abdomen for the past 2 weeks. On local examination, there was hepatomegaly with mild abdominal distension. Abdomen was soft and nontender. Routine blood investigation findings were within normal limits including complete blood counts, renal function tests, serum electrolytes, and liver function test, except Hb (5.8gm/dl), and serum lactate dehydrogenase was raised (1720 U/l). Bleeding time and clotting time were normal with raised prothrombin time. Thyroid profile and two-dimensional echo findings were normal. Serum AFP was raised (814 ng/dl). We transfused blood, and vitamin K was started. Ultrasonography of the abdomen showed multiple well-defined echogenic round solid lesions in the right lobe of liver, with approximate size of 73 × 103 mm, and no evidence of significant vascularity, calcification, and necrosis. MRI showed heterogeneously enhancing altered signal intensity multilobulated lesion in the right (fifth, sixth, seventh, and eighth segments) and caudate lobe of liver with multiple fibrinous strands and T2 hypointense areas. Lesion shows infiltration in hepatic parenchyma. Lesion is hyerintense on T2 and hypointense on T1, with well-defined lobulated margins. Fat planes were clear with portal vein and hepatic artery, likely IHH with hepatomegaly [Figure 1]. Triple-phase contrast-enhanced computed tomography (CT) showed hepatomegaly with large heterogeneously enhancing mass lesion with internal nonenhancing hypodense cystic-necrotic core and hyperdense (hemorrhagic) areas seen predominantly involving segments 6, 7, and 8 of right lobe of liver and segment 4 of left lobe of liver (measuring ∼10 × 12 × 11 cm) and caudate lobe of liver extending till porta hepatis, closely abutting the hepatic vein, inferior vena cava, and portal vein, with no significant enhancement of the mass lesion in arterial phase and showing heterogenous enhancement in portal venous phase, likely indicating mesenchymal hamartoma/IHH/HBL [Figure 2]. Tru-cut biopsy showed tumor cells in trabeculae, separated by sinusoid. Histopathological examination confirmed fetal epithelial type of HBL. Immunohistochemistry showed HBL with positive marker anti-human hepatocyte and AFP.
|Figure 2: (a and b) Triple-phase contrast-enhanced computed tomography images.|
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| Discussion|| |
The clinical manifestation of IHH is variable. The tumor may be asymptomatic and discovered incidentally. More often, however, the tumor is large and manifests as hepatomegaly, abdominal distention, or a palpable upper abdominal mass.
Hematologic abnormalities may be seen, including anemia and thrombocytopenia caused by consumptive coagulopathy (Kasabach–Merritt syndrome),. Serum AFP levels are usually normal or slightly elevated,,.
Patients with IHH usually have an excellent prognosis, especially with spontaneous regression after the first year of life. Nevertheless, children may die of associated complications such as severe heart failure. Treatment is determined based on the tumor size and the severity of symptoms (congestive heart failure, arteriovenous shunting, and coagulopathy).
HBL is classified by histopathology as epithelial type or mixed epithelial/mesenchymal type. Surgical resection is the mainstay of treatment for HBL, and with the use of neoadjuvant chemotherapy, up to 85% of HBL cases become resectable and can be cured. Therefore, the differential diagnosis is crucial to the clinicians for appropriate treatment selection.
The most important alternative diagnosis to IHH in this age group is HBL. It is more commonly located in the right hepatic lobe (>60% of cases). The serum AFP level is elevated in up to 90% of patients with HBL,. The lesions are usually large and solitary but may also be multifocal. However, HBL may have similar imaging features and clinical findings (congestive heart failure, Kasabach–Merritt syndrome) as IHH.
On US, IHH appears as a complex, mostly solid hepatic lesion with variable hypoechoic and hyperechoic echotexture. The lesions are often well demarcated from the surrounding liver parenchyma. On US, HBL most often demonstrates heterogeneous echogenicity owing to hemorrhage or necrosis.
On unenhanced CT, IHH usually manifests as a well-defined mass that is hypoattenuating relative to the normal liver parenchyma. On contrast-enhanced CT, the enhancement pattern may resemble that of an adult giant hemangioma,, with 'nodular' peripheral puddling of contrast material in the early phase, subsequent peripheral pooling, and central enhancement with variable delay,. On unenhanced CT, the HBL shows irregular hypoattenuation and calcifications in up to 50% of cases, appearing less fine and granular than hemangioendothelioma. Contrast-enhanced CT typically demonstrates inhomogeneous intratumoral uptake that is generally less intense than in the normal liver parenchyma, and hemangioendothelioma remains relatively hypoattenuating compared with IHH,.
On unenhanced MRI, the lesions have low-signal intensity on T1-weighted images and high-signal intensity on T2-weighted images. Because of the simultaneous presence of hemorrhage, necrosis, and fibrosis, the mass often appears heterogeneous on both T1-weighted and T2-weighted images. After intravenous administration of gadopentetate dimeglumine, the lesions usually show an enhancement pattern similar to that described on CT,.
Serum AFP is a useful laboratory marker for the differential diagnosis of pediatric liver tumors. AFP levels are rarely elevated above the normal reference range for age in patients with IHH, whereas nearly 90% of HBL tumors consist of hepatoblast-like cells that secrete large amounts of AFP,,,.
On histopathologic analysis, IHH manifests as a mesenchymal tumor composed of a connecting network of predominantly small-diameter vascular channels lined by endothelial cells. Areas of varying degrees of hemorrhage, necrosis, calcification, thrombosis, or fibrosis are often present in large tumors.
The IHH may often be diagnosed based on clinical, imaging, and laboratory findings. Nevertheless, depending on the proclivities of the treating physicians, histopathologic confirmation of a radiologically suspected hemangioendothelioma is often obtained before medical treatment is initiated.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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