Chapurin Nikita, Totten Douglas J, Louis Peter C, Lewis James S, Chowdhury Naweed I, Turner Justin, Chandra Rakesh K
Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.
Vanderbilt University Medical School, Nashville, TN, USA.
Ear Nose Throat J. 2022 Jul;101(6):392-395. doi: 10.1177/0145561320964640. Epub 2020 Oct 22.
Sinonasal small cell neuroendocrine carcinoma (SNEC) is an extremely rare and aggressive neoplasm that can arise in the sinonasal region. These tumors are associated with high morbidity and mortality, are difficult to diagnose, and are hard to treat. We describe 2 cases of this poorly understood malignancy and review imaging, pathology, and treatment decisions. A 41-year-old male and a 64-year-old female presented to a tertiary center in 2019 after developing nasal obstruction and were found to have sinonasal masses on imaging. Both biopsies showed strong expression of pancytokeratin with dot-like reactivity and expression of neuroendocrine markers chromogranin and synaptophysin. The findings were diagnostic of SNEC. Staging positron emission tomography/computed tomography and brain MRI were performed, and patients were discussed at a multidisciplinary tumor board. Neither had distant metastatic disease at presentation. One patient had no intracranial or orbital disease and underwent a subtotal endoscopic resection with adjuvant chemoradiation. The other patient demonstrated middle cranial fossa, dural, and orbital involvement as well as cranial nerve V palsy. This patient was treated with induction chemotherapy, followed by concurrent chemoradiation. Both patients are presently alive at 4 months follow-up, but one with persistent local disease and the other distant metastasis. Sinonasal small cell neuroendocrine carcinoma is a rare and poorly understood malignancy with an aggressive clinical course. Continued careful review of pathology and study of molecular features are needed for improved understanding of SNEC, and particularly for head and neck SNEC, to establish a staging system and better formulate treatment protocols.
鼻窦小细胞神经内分泌癌（SNEC）是一种极其罕见且侵袭性强的肿瘤，可发生于鼻窦区域。这些肿瘤与高发病率和高死亡率相关，诊断困难且治疗棘手。我们描述了2例这种了解甚少的恶性肿瘤病例，并回顾了影像学、病理学及治疗决策。一名41岁男性和一名64岁女性于2019年因出现鼻塞就诊于一家三级中心，影像学检查发现鼻窦有肿物。两次活检均显示全细胞角蛋白呈强表达且有斑点状反应，同时神经内分泌标志物嗜铬粒蛋白和突触素也有表达。这些发现确诊为SNEC。进行了分期正电子发射断层扫描/计算机断层扫描及脑部磁共振成像检查，并在多学科肿瘤讨论会上对患者进行了讨论。两名患者就诊时均无远处转移病灶。一名患者无颅内或眶内病变，接受了辅助放化疗的内镜下次全切除术。另一名患者表现为中颅窝、硬脑膜及眶内受累，同时伴有颅神经Ⅴ麻痹。该患者接受了诱导化疗，随后进行同步放化疗。两名患者在随访4个月时均存活，但一名有持续性局部病变，另一名有远处转移。鼻窦小细胞神经内分泌癌是一种罕见且了解甚少的恶性肿瘤，临床病程侵袭性强。需要持续仔细地回顾病理学并研究分子特征，以更好地了解SNEC，尤其是头颈部SNEC，从而建立分期系统并更好地制定治疗方案。
