Abstract
Infantile hepatic hemangioendothelioma (IHH) is a rare benign mesenchymal tumor. As the name implies, it is commonly seen in infancy and has variable clinical presentation and outcome. This tumor can spontaneously regress or can have expansile growth replacing whole of the liver. In very large tumors, there may be extensive arteriovenous shunting within the lesion, resulting congestive heart failure (CHF) in a significant number of patients with poor outcome. Since there is no tumor marker for this tumor, the diagnosis can only be suspected on the basis of clinical features and radiological investigations from among the various mass occupying lesions of the liver in this age group, which includes hepatoblastoma, mesenchymal hamartoma or hepatocellular carcinoma. As a result, the management strategy cannot be defined and in the literature also there are isolated reports with diverse treatment protocols. The medical management includes corticosteroid, interferon, cytotoxic agents and irradiation or even the radical interventions such as hepatic artery ligation, transcatheter hepatic artery embolization. The surgical options are biopsy, complete resection of the lesion or even liver transplantation. We are reporting a case of IHH who was managed with surgical resection and postoperative corticosteroid. However, the patient had recurrence of the tumor and developed CHF, which led to the demise of the patient. The idea of reporting this case is that this patient was 11-year-old child who very unusual for this tumor at this age, and the surgical resection of this lesion should be radical rather than just excision biopsy as was done in the present case.
Published Version
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