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428 Hepatic Epithelioid Hemangioendothelioma: A Rare Vascular Malignancy Mimicking as Intrahepatic Cholangiocarcinoma

Authors :
T M H Gall
Michal Kawka
Shengyang Qiu
S. Mak
Long R. Jiao
Source :
British Journal of Surgery. 108
Publication Year :
2021
Publisher :
Oxford University Press (OUP), 2021.

Abstract

Hepatic epithelioid hemangio-endothelioma (EHE) is a rare, often misdiagnosed malignancy of vascular origin. We describe a case of a 36-year-old, who presented with a burning sensation in his chest on exercise and was believed to have bilobar intrahepatic cholangiocarcinoma. After receiving chemotherapy with partial response, the patient underwent staged resection modified ALPPS (associating liver partition with portal vein ligation for staged hepatectomy) procedure – laparoscopic radiofrequency assisted ALPPS (RALPPS). Histological examination of the stage 1 specimen revealed EHE deposits in the left lobe of the liver. The patient proceeded to have stage 2 open right hepatectomy with concurring histology. 90-days follow-up CT scan showed almost complete resolution of EHE lesions. Low incidence, non-specific clinical and radiological characteristics all contribute to high EHE misdiagnosis rate. Histological analysis can be used for confirmation of the diagnosis; however, specialist staining is required, which is not routinely performed. Multiple treatments are available against EHE, but only liver resection and liver transplantation are potentially curative. ALPPS and its modifications (such as RALPPS) offer an alternative for conventional two-stage hepatectomy for patients with extensive bilobar EHE, who are at risk of developing post-hepatectomy liver failure or patients with anomalous hepatic vasculature anatomy, which makes PVE challenging.

Details

ISSN :
13652168 and 00071323
Volume :
108
Database :
OpenAIRE
Journal :
British Journal of Surgery
Accession number :
edsair.doi...........5efaefee0d16c1eb4433554ab1fc9b70