Alarashi Leen, Hamodi Laila, Abdallah Lamis, Alali Mousa, Saifo Maher
Faculty of Medicine, Damascus University, Fayez Mansour Street, P. O. Box: 222, Damascus, Syrian Arab Republic.
Department of Oncology, Albairouni University Hospital, Faculty of Medicine, Damascus University, Harasta M5, Damascus, Syrian Arab Republic.
Heliyon. 2024 Sep 28;10(19):e38704. doi: 10.1016/j.heliyon.2024.e38704. eCollection 2024 Oct 15.
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.
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.
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.
上皮样血管内皮瘤（EHE）是一种极为罕见的血管肿瘤，占所有血管肿瘤的比例不到1%。原发性肝上皮样血管内皮瘤（HEHE）的诊断可能具有挑战性，因为它可能被误诊为其他肝脏病变。这导致了许多病例的误诊。本研究旨在介绍一例罕见的HEHE病例，该病例发生在一名患有家族性地中海热（FMF）和甲状腺功能减退症的女性患者中，该患者接受了帕唑帕尼治疗并显示出长期疗效。
一名43岁患有FMF的叙利亚女性，接受秋水仙碱治疗5年，甲状腺功能减退症接受甲状腺素治疗，被诊断为HEHE。她接受酪氨酸激酶抑制剂（帕唑帕尼）治疗超过两年，并显示出持久的疗效。尽管患者停止服用秋水仙碱，但在开始使用帕唑帕尼后腹痛发作有所改善。帕唑帕尼耐受性良好，患者仍然存活且情况良好。
本研究突出了一例患有多种合并症的患者出现慢性腹痛，最终诊断为罕见的HEHE病例并对帕唑帕尼产生长期反应。此外，我们讨论了帕唑帕尼对FMF症状的影响。
