[Hemangioendothelioma of the pancreas and choledochus, as a cause of cholestatic neonatal and Kasabach-Merrit syndromes]

Abstract

Vascular congenital abnormalities are common in children and are often seen in skin and soft tissues. They are rarely observed in internal organs. They may be found singly or in a multiple fashion. The liver is the most frequently affected organ by this disease. The most common histological variety is the hemangioendothelioma. This is a rare neoplasm of the pancreas. The case of a six month old boy with a pancreatic head hemangioendothelioma, associated with thrombocytopenia and digestive track bleeding is presented. The case is clinically interesting due to the vascular malformation and its location. These in turn caused obstructive neonatal jaundice and very severe liver lesions which contributed to the child's death. Paradoxically, the neoplasm is not considered malignant and the secondarily produced liver lesions could have been prevented if the patient would have been diagnosed correctly and at an earlier stage. Hemangioendothelioma; Kasabach-Merrit syndrome; neonatal cholestatic syndrome.