A rare case of intravascular solitary fibrous tumor from the internal jugular vein to the sigmoid sinus

M. Masuda, Y. Honda, Daizo Murakami, Y. Orita
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引用次数: 1

Abstract

We report a rare case of intravascular solitary fibrous tumor (SFT) from the internal jugular vein to the sigmoid sinus, which has been followed over nine years. A 51-year-old woman was referred to our hospital, pre-senting with fullness of the right ear and swelling of the right neck. On imaging study, a tumor occupying from the right supraclavicular to the skull base, through the jugular foramen was found. The biopsy specimen of the intravascular tumor of the right jugular vein was initially diagnosed as epithelioid hemangioendothelioma (EHE). The tumor increased in size at first, and after two courses of S-1 therapy (Tegafur, a prodrug of 5-fluorouracil, and 5-chloro-2-4-dihydroxypyridine), the intravascular tumor in the right jugular vein had disappeared, whereas the lesion at the skull base remained stationary. This remaining lesion had gradually enlarged over the next 3—4 years, causing Ⅶ—Ⅻ cranial nerve paralysis. Radiation therapy was administered, and arytenoid adduc-tion and nerve-muscle pedicle flap implantation were performed for laryngeal paralysis. Over 5-year follow-up observation after irradiation, the remaining lesion at the skull base gradually diminished each year. Afterward, the histopathological findings of the biopsy specimen were reviewed and a revised diagnosis of intravascular SFT was made. Intravascular SFT is extremely rare, and the effects of chemotherapy or radiotherapy for a malignant soft tissue tumor are controversial, however, S-1 therapy and radiation therapy were successful in this case.
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一例罕见的从颈内静脉到乙状窦的血管内孤立性纤维性肿瘤
我们报告一例罕见的从颈内静脉到乙状窦的血管内孤立性纤维性肿瘤(SFT),该病例已被随访超过9年。一位51岁的女性被转介到我们医院,表现为右耳丰满和右颈部肿胀。影像学检查发现肿瘤从右锁骨上沿颈静脉孔向颅底占位。右颈静脉血管内肿瘤活检标本初步诊断为上皮样血管内皮瘤(EHE)。肿瘤开始增大,经过两个疗程的S-1治疗(替加富,5-氟尿嘧啶的前药,5-氯-2-4-二羟基吡啶)后,右颈静脉血管内肿瘤消失,颅底病变保持不变。在接下来的3-4年里,剩余的病变逐渐扩大,导致Ⅶ-Ⅻ脑神经麻痹。给予放射治疗,并行杓状内收及神经肌蒂皮瓣植入术治疗喉麻痹。放疗后5年随访观察,颅底残余病变逐年逐渐减少。之后,对活检标本的组织病理学结果进行了回顾,并对血管内SFT进行了修订诊断。血管内SFT极为罕见,恶性软组织肿瘤的化疗或放疗效果存在争议,然而,S-1治疗和放射治疗在本病例中是成功的。
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来源期刊
Japanese Journal of Head and Neck Cancer
Japanese Journal of Head and Neck Cancer Medicine-Otorhinolaryngology
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0.10
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7
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