颈静脉孔软骨肉瘤1例。

Josip Prnjak, Andro Košec, Alan Pegan, Tomislav Greguric, Jakov Ajduk
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摘要

绝大多数颅骨软骨肉瘤(CS)位于颅底,是局部侵袭性恶性肿瘤,占颅内肿瘤的0.15%。广泛手术切缘的完全手术切除是目前主要的治疗策略,但由于头颈部复杂的解剖结构,很难实现。颈静脉孔位于后窝底和枕岩缝合线的后外侧,是一个非常罕见的CS位置。本文报告一例65岁的颈静脉孔原发CS患者,表现为耳痛、搏动性耳鸣、左耳轻度听力丧失,并伴有面神经麻痹。x线影像学显示左侧乳突、中耳和颈静脉窝肿块,颈静脉窝和乳突骨破坏,而磁共振成像显示岩石斜裂、舌下管、颈静脉球和乙状窦的额外累及。肿瘤经Fisch颞下窝a型入路切除宽切缘,随后行放疗。免疫表型和组织学特征与中度分化软骨肉瘤基本吻合。治疗后6个月无复发。以面瘫为首发表现的中耳分泌物至今未见报道。独特的定位、治疗细节和组织病理学数据与扩大当前对该主题的知识水平有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Chondrosarcoma of the Jugular Foramen: A Case Report.

The vast majority of chondrosarcomas of the skull (CS) are located at the skull base and represent locally aggressive malignant tumors that account for 0.15% of all intracranial neoplasms. Complete surgical resection with wide surgical margins is currently the main treatment strategy, but can be hard to achieve due to the complex anatomy of the head and neck. The jugular foramen, situated in the floor of the posterior fossa and posterolaterally to the petro-occipital suture, is a remarkably rare location for CS. A case of primary CS of the jugular foramen in a 65-year-old patient is reported, presenting with otalgia, pulsatile tinnitus, and mild hearing loss in the left ear, accompanied by peripheral facial nerve paresis. Radiographic imaging showed a mass in the left mastoid, middle ear and jugular fossa with bone destruction of the jugular fossa and mastoid, while magnetic resonance imaging showed additional involvement of the petroclival fissure, hypoglossal canal, jugular bulb, and sigmoid sinus. The tumor was resected with wide margins through a Fisch infratemporal fossa approach type A, followed by radiotherapy. Results of the immunophenotyping along with histological features primarily matched moderately differentiated chondrosarcoma. The patient is recurrence free at 6 months after treatment. Middle ear discharge with facial palsy as a first manifestation of the CS has not been described to date. The unique localization, treatment details and histopathologic data are relevant in expanding the current level of knowledge on the subject.

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