Introduction: Neuroendocrine carcinoma is an aggressive neoplasm with a high recurrence potential. Its location in the Sino nasal tract is considered a rare disease. CT and MR imaging features of these tumors are nonspecific. Paranasal sinus neuroendocrine carcinomas showed expansion and destruction of the sinus. Endoscopic examination is complementary to imaging and mandatory in order to reveal early stage tumour and its extension. Treatment recommendations for this entity vary considerably due largely to a lack of consensus and variable pathological classification. Given the high incidence of distant failure and the chemosensitivity of NEC, neoadjuvant chemotherapy followed by either chemoradiation or surgery and postoperative radiation therapy is a promising strategy. Case report: We report the case of a 17-years-old patient, with a history of a non-resolving rhinological burning pain evolving since 1 year. She didn’t consult until a blistering lesion filled half of the oral cavity. Oral examination found an proliferative process of the posterior aspect of the oral cavity She received a CT scan and MRI that showed a destruction of the periodontium, with an extensive geographical osteolytic homogeneous mass destroying the left nasal cavity, as well as an infiltration of the ipsilateral orbit. The diagnosis of Sino nasal neuroendocrine carcinoma was suggested histologically after biopsy. After a regimen of etoposide and carboplatine supplemented by palliative radiotherapy no regression was noted. The evolution was characterized by the death of the patient. Conclusion: In conclusion, our case suggests that Sino nasal NEC is an exceedingly rare tumor that commonly presents as late-stage dis ease. Proper histopathologic diagnosis is paramount to dictate appropriate therapy. Poor prognostic factors affecting survival include TNM staging, tumor site, and bony or orbital invasion. Response to systemic therapy may play a greater role in defining the individualized treatment strategy for the management for these patients.
