Chaouki A, Mkhatri A, Ballage A, Zouhair N, Mahtar M
ENT Department, 20 August Hospital, CHU Ibn Rochd, Casablanca, Morocco.
ENT Department, 20 August Hospital, CHU Ibn Rochd, Casablanca, Morocco.
Int J Surg Case Rep. 2019;58:70-73. doi: 10.1016/j.ijscr.2019.04.013. Epub 2019 Apr 16.
Epitheliod hemandioendothelioma (EHE) is a rare vascular tumor which was first reported by Weiss and Enzinger in 1982. It can be seen in many locations whose paranasal sinus is extremly rare. Its main treatment is surgery. To our best knowledge, no report of EHE treated by radiation therapy and chemotherapy has been described.
A 18 years old man presented to our hospital with a 3 months history of right intermittent epistaxis, permnanent nasal obstruction and right hearing loss. The physical exam found a right exophtalmia, swelling deformatted right hemifacia and a bulky whitish tumor filling the right nasal cavity. The magnetic resonance imaging (MRI) showed an extensive hyperascularized nasopharyngeal process filling the right nasal cavity whose histopathological exam revealed an epitheloid hemangioendothelioma. Due to intracranial extension, surgery was contraindicated. The patient received 65 Gy of radiation therapy and chemotheray (cisplatin). After 18 months of follow up, the exopthalmous regressed and the MRI showed a 50% regression of the tumour size.
Extensive EHE can be treated by radiation therapy and chemotherapy when surgery can't be perfomed.
上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，于1982年由韦斯和恩津格首次报道。它可见于许多部位，累及鼻窦极为罕见。其主要治疗方法是手术。据我们所知，尚无关于EHE接受放疗和化疗的报道。
一名18岁男性因右侧间歇性鼻出血、持续性鼻塞和右耳听力减退3个月就诊于我院。体格检查发现右眼突出、右侧面部肿胀变形，右侧鼻腔内有一个巨大的灰白色肿瘤。磁共振成像（MRI）显示一个广泛血管化的鼻咽部肿物充满右侧鼻腔，组织病理学检查显示为上皮样血管内皮瘤。由于肿瘤侵犯颅内，手术禁忌。患者接受了65Gy的放疗和化疗（顺铂）。随访18个月后，眼球突出消退，MRI显示肿瘤大小缩小了50%。
当无法进行手术时，广泛的EHE可通过放疗和化疗进行治疗。
