Hepatic epithelioid hemangioendothelioma (HEHE)-rare vascular malignancy mimicking cholangiocarcinoma: a case report

Hepatic epithelioid hemangioendothelioma (HEHE) 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 with modified associating liver partition with portal vein ligation for staged hepatectomy (ALPPS) procedure- laparoscopic radiofrequency-assisted ALPPS (RALPPS). Histological examination of the stage 1 specimen revealed HEHE deposits in the left lobe of the liver. The patient proceeded to have stage 2 open right hepatectomy with concurring histology. Ninety-day follow-up computed tomography scan showed almost complete resolution of HEHE lesions. Low incidence, non-specific clinical and radiological characteristics all contribute to high HEHE 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 HEHE, but only liver resection and liver transplantation are potentially curative. ALPPS and its modifications (such as RALPPS) offer an alternative from conventional two-stage hepatectomy for patients with extensive bilobar HEHE, who are at risk of developing post-hepatectomy liver failure or patients with anomalous hepatic vasculature anatomy which makes portal vein embolisation (PVE) challenging.