■鼻窦神经内分泌癌是一种罕见的头颈部肿瘤,仅占鼻窦肿瘤的5%。该病变有很高的侵入邻近结构的风险,例如轨道,颅底,还有软组织,症状通常是非特异性的。大多数病例是晚期诊断的,不治疗降低总生存率。迄今为止,鉴于其患病率较低,在管理上没有达成共识;然而,研究表明,多模式疗法,以正确的手术方式为主,提供更好的无病预后。
一名46岁女性,有1年的鼻部症状史,以阻塞和鼻出血为特征。影像学检查显示轴外肿块导致颅底侵蚀和右额眶区移位,不侵入脑实质或脑膜。进行了活检,并诊断出不可切除的低分化鼻腔鼻窦神经内分泌癌。开始用放疗和化疗治疗,随着肿瘤体积的减少,她被转诊为神经外科介入治疗;进行了鼻内镜入路.完全切除,患者出院,无术后并发症,影像学上无残留病灶。
■我们描述了一种罕见的晚期肿瘤的演变过程。它强调了尽管接受了不可切除的肿块的初步诊断,多模式疗法,和适当的手术方法认为整个病变被切除。尽管临床进展良好,神经内分泌癌的随访优先考虑复发和转移率高的肿瘤。
UNASSIGNED: Sinonasal neuroendocrine carcinoma is a rare head and neck tumor that represents only 5% of
sinonasal neoplasms. This lesion has a high risk of invasion to adjacent structures such as the orbit, skull base, and soft tissues, with symptoms usually being nonspecific. Most cases are diagnosed in late stages, decreasing overall survival without treatment. To date, there is no consensus on management given its low prevalence; however, it has been shown that multimodal therapy, with the correct surgical approach as the mainstay, offers a better disease-free prognosis.
UNASSIGNED: A 46-year-old woman presented with a 1 year history of nasal symptoms, characterized by obstruction and epistaxis. Imaging studies showed an extra-axial mass causing skull base erosion and displacement of the right fronto-orbital region, without invasion of brain parenchyma or meninges. A biopsy was performed and an unresectable poorly differentiated
sinonasal neuroendocrine carcinoma was diagnosed. Treatment with radio and chemotherapy was initiated and, as the tumoral volume decreased, she was referred for neurosurgical intervention; an endonasal endoscopic approach was performed. Gross total resection was achieved and the patient was discharged without postoperative complications and no residual lesion on imaging.
UNASSIGNED: We describe the evolution of a rare advanced-stage neoplasm. It highlights that despite receiving an initial diagnosis of an unresectable mass, multimodal therapy, and an adequate surgical approach deemed the entire lesion to be resected. Despite the favorable clinical evolution, the follow-up of neuroendocrine carcinoma is prioritized as a neoplasm with a high rate of recurrence and metastasis.