Objective To investigate the clinicopathologic features of sinonasal teratocarcinosarcoma (SNTCS) in order to improve the understanding of clinician and pathologists.

Methods The clinicopathological data of 3 cases of SNTCS were collected and immunohistochemistry was performed by EnVision two-step method, and related literature was reviewed.

Results All the 3 cases were male, with the age of 35, 55, and 69 years old (range 35~69), and located in the nasal cavity and sinuses, all of them presented with nasal obstruction and rhinorrhea. Microscopically, benign and malignant epithelial, mesenchymal. Different components expressed corresponding immunohistochemical markers, two cases showed that all components were SMARCA4 deficient. One case received chemotherapy only, two cases underwent complete surgical resection, including one case combined with radiotherapy and one case combined with radiotherapy and chemotherapy. The follow-up was 4~20 months, 2 cases were lost to follow-up at 4 months and 16 months after diagnosis, respectively, and one case was still alive at 20 months after diagnosis.

Conclusions The histological morphology of SNTCS is complex, which needs to be differentiated from various tumors of nasal cavity and sinuses, and the diagnosis depends on sufficient sampling. The tumor is highly malignant and aggressive. Early surgical resection and postoperative combination of radiotherapy and chemotherapy can improve the prognosis.