Challenging diagnosis of primary hepatic epithelioid hemangioendothelioma in a patient with familial Mediterranean fever and hypothyroidism: A rare case report

Background: Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular tumor that accounts for less than 1 % of all vascular tumors. The diagnosis of primary hepatic epithelioid hemangioendothelioma (HEHE) can be challenging because it may be mistaken for other liver lesions. This has led to misdiagnosis in many cases. This study aimed to present a rare case of HEHE that was diagnosed in a female patient with familial Mediterranean fever (FMF) and hypothyroidism who received pazopanib treatment and demonstrated a long-term response to it.

Case presentation: A 43-year-old Syrian woman with FMF who was treated with colchicine for 5 years and hypothyroidism treated with thyroxin was diagnosed with HEHE. She was treated with a tyrosine kinase inhibitor (pazopanib) for over two years and showed a durable response. Although the patient stopped taking colchicine, the episodes of abdominal pain improved after the initiation of pazopanib administration. Pazopanib was well tolerated, and the patient is still alive and doing well.

Conclusions: This study highlights a case of chronic abdominal pain in a patient with multiple comorbidities that resulted in the diagnosis of a rare case of HEHE and prolonged response to pazopanib. In addition, we discuss the effects of pazopanib on FMF symptoms.