Prnjak Josip, Košec Andro, Pegan Alan, Greguric Tomislav, Ajduk Jakov
University of Zagreb, School of Medicine, Zagreb, Croatia.
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Center Sestre Milosrdnice,Zagreb, Croatia.
J Int Adv Otol. 2025 Mar 25;21(2):1-5. doi: 10.5152/iao.2025.231362.
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.
绝大多数颅骨软骨肉瘤(CS)位于颅底,是具有局部侵袭性的恶性肿瘤,占所有颅内肿瘤的0.15%。目前,采用宽手术切缘的完整手术切除是主要治疗策略,但由于头颈部解剖结构复杂,难以实现。颈静脉孔位于后颅窝底部、岩枕缝后外侧,是CS极为罕见的发病部位。本文报告了一例65岁患者的颈静脉孔原发性CS,表现为耳痛、搏动性耳鸣和左耳轻度听力损失,并伴有周围性面神经麻痹。影像学检查显示左侧乳突、中耳和颈静脉窝有肿块,颈静脉窝和乳突骨质破坏,而磁共振成像显示岩斜裂、舌下神经管、颈静脉球和乙状窦也有受累。通过Fisch A型颞下窝入路对肿瘤进行了宽切缘切除,并进行了放疗。免疫表型分析结果与组织学特征主要符合中度分化软骨肉瘤。患者在治疗后6个月无复发。以中耳流脓和面神经麻痹作为CS首发表现的情况迄今尚未见报道。该独特的发病部位、治疗细节和组织病理学数据对于扩展当前对该疾病的认识水平具有重要意义。