Infantile hemangioendothelioma with elevated serum α fetoprotein: report of 2 cases with immunohistochemical analysis

Department of Hospital Pathology, College of Medicine, the Catholic University of Korea, 150-713 Seoul, Korea.
Human pathology (Impact Factor: 2.77). 02/2010; 41(5):763-7. DOI: 10.1016/j.humpath.2009.05.019
Source: PubMed


Infantile hemangioendothelioma is the most common benign mesenchymal tumor of the liver presenting during the first 6 months of life. Serum alpha fetoprotein is an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors. However, it is rarely elevated in patients with hepatic infantile hemangioendothelioma. In such cases, surgery may be done to rule out malignancies when alpha fetoprotein levels are high. The etiology of the elevated alpha fetoprotein level has not yet been elucidated. We report 2 cases of solitary hepatic infantile hemangioendothelioma and demonstrate immunohistochemically that hepatocytes near or entrapped within the tumor were the source of the increased serum levels of alpha fetoprotein explaining the unusual clinical presentation.

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Available from: Tae-Jung Kim, Sep 11, 2015
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    • "Serum AFP concentrations decrease rapidly after birth and reach adult levels by 8 months of age. Increased serum AFP concentrations are rarely observed in patients with IHHE, with one study reporting that clinical symptoms, age at presentation, tumor size and AFP concentration were not significantly related to time required for complete tumor regression21-23). Similarly, only three of our patients had increased concentrations of AFP, with all three having multiple tumors; despite receiving different treatment modalities, all three showed nearly complete tumor resolution. "
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    ABSTRACT: Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy. We conducted this study to review our clinical experience of patients with IHHE and to suggest management strategies. We retrospectively analyzed the medical records of 23 IHHE patients (10 males, 13 females) treated at the Asan Medical Center between 1996 and 2009. Median age at diagnosis was 38 days (range, 1 to 381 days). Seven patients (30%) were diagnosed with IHHE based on sonographically detected fetal liver masses, 5 (22%) were diagnosed incidentally in the absence of symptoms, 5 (22%) had congestive heart failure, 3 (13%) had skin hemangiomas, 2 (9%) had abnormal liver function tests, and 1 (4%) had hepatomegaly. All diagnoses were based on imaging results, and were confirmed in three patients by histopathology analysis. Six patients were observed without receiving any treatment, whereas 12 received corticosteroids and/or interferon-alpha. One patient with congestive heart failure and a resectable unilobar tumor underwent surgical resection. Three patients with congestive heart failure and unresectable tumors were managed by hepatic artery embolization with/without medical treatment. At a median follow-up of 29 months (range, 1 to 156 months), 21 (91%) patients showed complete tumor disappearance or >50% decrease in tumor size. One patient died due to tumor-related causes. IHHE generally has a benign clinical course with low morbidity and mortality rates. Clinical course and treatment outcome did not differ significantly between medically treated and non-treated groups. Surgically unresectable patients with significant symptoms may be treated medically or with hepatic artery embolization.
    Korean Journal of Pediatrics 06/2011; 54(6):260-6. DOI:10.3345/kjp.2011.54.6.260
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    The Korean Journal of Hepatology 06/2010; 16(2):192-6. DOI:10.3350/kjhep.2010.16.2.192
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    ABSTRACT: To investigate whether the clinicopathologic features of infantile hemangioendothelioma (IHE) of the liver in a Chinese population are similar to the features observed in other races. The clinical data, radiological findings, histopathological changes and outcome of 12 cases of IHE diagnosed by the Department of Pathology, West China Hospital over the last 10 years were analyzed retrospectively. Immunohistochemical studies were carried out using antibodies against CD31, CD34, Factor VIII, cytokeratin 8 and cytokeratin 18. The 12 patients were aged from fetal to 5 years (three males and nine females). The tumor was presented with different clinical manifestations, mainly as an asymptomatic, palpable, upper abdominal mass, except for the two fetuses who were detected antenatally by ultrasound. In one patient, this presentation was accompanied by an initial severe pneumothorax. No symptoms of congestive heart failure were present and neither congenital abnormalities nor vascular tumors in the skin or other organs were found. Laboratory abnormalities included leukocytosis (40%), anemia (60%), thrombocytosis (60%), hyperbilirubinemia (16.7%), abnormal liver function (50%) and increased α-fetoprotein (80%). Based on radiological findings and gross specimens, the tumor presented as a solitary lesion or a multifocal space-occupying lesion. The tumor size ranged from 5.0 cm × 3.5 cm × 2.0 cm to 13.8 cm × 9.0 cm × 7.7 cm, and the 0.2-1.1 cm nodules were diffusely distributed within the multifocal tumor. Seven cases were surgically resected, three cases underwent biopsy and the two fetuses were aborted. Histologically, nine cases were classified as type I and three as type II, presenting aggressive morphologic features, immature vessels, active mitosis and necrosis. An inflammatory component, predominantly eosinophilic granulocytes, sometimes obscured the nature of the tumor. Ten patients are alive after a follow-up of 1-9 years. Based on immunohistochemistry, the endothelial cells in all cases were positive for CD31, CD34 and polyclonal factor VIII antigen, whereas the scattered hyperplasia bile ducts were positive for cytokeratin 8 and cytokeratin 18. The clinical manifestations of IHE are non-specific. There is no significant correlation between histological type and prognosis. The clinicopathologic features of IHE in Chinese patients may provide a clue to further evidence-based studies.
    World Journal of Gastroenterology 09/2010; 16(36):4549-57. · 2.37 Impact Factor
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