Abstract：
Tumors located in the nasal cavity, paranasal sinuses and the skull base comprise a wide range of histologic subtypes. Among them, neuroendocrine and undifferentiated tumors are rare but noteworthy, because of their distinctive features, aggressive nature, and diagnostic complexities. A literature search was conducted in the PubMed/MEDLINE and the Scopus databases from 2019 until inception. The keywords \"neuroendocrine\", \"undifferentiated\", \"nose\", \"sinonasal\", \"paranasal\", \"skull base\" were used. Thirty-eight articles referring to neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base were finally included and analyzed. Neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base are infrequent malignancies, most commonly affecting middle-aged men. They usually present with non-specific symptoms, even though ocular or neurologic manifestations may occur. Prognosis is generally poor; however, novel targeted and immunological therapies have shown promising results. Sinonasal Neuroendocrine Carcinomas (SNECs) carry distinct histological and immunohistochemical features. Management consists of surgical resection coupled with systematic therapy. Sinonasal Undifferentiated Carcinomas (SNUCs) lack specific squamous or glandular features. They typically stain positive for pancytokeratin and INI1 antibody. Treatment includes induction chemotherapy, followed by a combination of chemotherapy and radiotherapy. Olfactory neuroblastomas (ONBs) have neuroepithelial or neuroblastic features. They show diffuse positivity for various markers, including synaptophysin, chromogranin, and neuron-specific enolase (NSE). Surgical resection plus radiotherapy is considered the treatment of choice. In conclusion, neuroendocrine and undifferentiated tumors arising from the nose, paranasal sinuses and the skull base represent a unique group of malignancies. A thorough understanding of their clinical features, molecular changes, diagnostic approaches, treatment modalities, and prognostic factors is critical for providing optimal patient care. Still, continued research efforts and multidisciplinary collaboration are warranted, in order to improve outcomes for patients diagnosed with these rare and aggressive tumors.
摘要：
位于鼻腔的肿瘤，鼻旁窦和颅底包括广泛的组织学亚型。其中,神经内分泌和未分化肿瘤罕见但值得注意，由于它们的独特特征，侵略性的性质，和诊断的复杂性。从2019年到成立，在PubMed/MEDLINE和Scopus数据库中进行了文献检索。关键词\"神经内分泌\",\"未分化\",“鼻子”，“鼻窦”，“鼻旁”，使用了“颅底”。38篇文章提到神经内分泌和鼻子未分化肿瘤，最终纳入鼻旁窦和颅底并进行分析。鼻子的神经内分泌和未分化肿瘤，鼻旁窦和颅底是罕见的恶性肿瘤，最常影响中年男子。他们通常表现为非特异性症状，即使可能出现眼部或神经系统表现。预后通常较差；然而，新的靶向和免疫疗法已显示出有希望的结果。鼻窦神经内分泌癌（SNECs）具有独特的组织学和免疫组织化学特征。管理包括手术切除和系统治疗。鼻窦未分化癌（SNUCs）缺乏特定的鳞状或腺状特征。它们通常对全细胞角蛋白和INI1抗体染色呈阳性。治疗包括诱导化疗，然后联合化疗和放疗。嗅觉神经母细胞瘤（ONBs）具有神经上皮或神经母细胞特征。它们显示了各种标记的弥漫性阳性，包括突触素,嗜铬粒蛋白,和神经元特异性烯醇化酶（NSE）。手术切除加放疗被认为是治疗的选择。总之,由鼻子引起的神经内分泌和未分化肿瘤，鼻旁窦和颅底代表一组独特的恶性肿瘤。彻底了解他们的临床特征，分子变化,诊断方法，治疗方式，和预后因素对于提供最佳的患者护理至关重要。尽管如此，持续的研究努力和多学科的合作是必要的，为了改善被诊断患有这些罕见和侵袭性肿瘤的患者的预后。
