Unusual presentation of a pancreatic mass in an infant: pancreatic haemangioendotheliomatosis

Abstract

ABSTRACT. Primary pancreatic tumours are extremely rare in children. We report a case of a 5-month-old male with a diffuse invasive tumour of the head of the pancreas. The tumour demonstrated peripancreatic extension into the porta hepatis, which occluded the portal vein and invaded the superior mesenteric artery. It was found to be haemangioendotheliomatosis of the pancreas. Imaging, pathological findings and a brief relevant classification of haemangioma are discussed.

Pancreatic kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon: A rare entity

“…On ultrasound, KHE appears as a homogeneous, ill-defined, soft tissue mass, which may be isoechoeic or hyperechoic compared to the pancreas, with associated mild to marked increased vascularity on colour Doppler. 2,6 All previously reported cases described a mass in the head of the pancreas, as in this case report. 6 Although the possibility of a vascular tumour could be suggested on US, a more specific diagnosis cannot be made because of the limitations in demonstrating the infiltrative portions and exact extent of KHE.…”

“…2,6 All previously reported cases described a mass in the head of the pancreas, as in this case report. 6 Although the possibility of a vascular tumour could be suggested on US, a more specific diagnosis cannot be made because of the limitations in demonstrating the infiltrative portions and exact extent of KHE. Computed tomography and MRI together with magnetic resonance cholangiopancreatography (MRCP) are of added value in localising the mass, assessing the tumour extent and giving information of any associated findings such as dilation of the pancreatic and biliary ducts, duodenal obstruction and ascites.…”

“…Haemangiomas may appear similar to KHE on imaging, except for the more aggressive and locally invasive nature of this lesion. 6 This together with laboratory evidence of KMP may assist in the differentiation between these two entities.…”

“…2 MRI findings typically show a lobulated, invasive mass with increased signal intensity on T2WI and avid enhancement following intravenous gadolinium. 6 Ryu et al reported 12 cases of pathologically proven KHE across the body, with lesions demonstrating heterogeneous regions of isointensity or mild hyperintensity on T2 weighted imaging (T2WI) and heterogeneous enhancement post-contrast administration. 2 Regions of low signal on T2WI are likely secondary to hemosiderin deposition and dense hyaline stromal responses.…”

Epithelioid hemangioendothelioma of the pancreas presented as massive hematemesis

“…The treatment of such tumors depend on the size of the lesion, the location, and the presentation. Some lesions are treated with enucleation, others are treated with local or wide excision, systemic use of steroids and interferon have been used with different responses, in our case we didn’t use these form of treatment because the patient had emergency presentation, we performed complete surgical excision as a primary form of treatment [9,10].…”