RESUMEN
Sinonasal teratocarcinosarcomas (SNTCSs) are rare and aggressive malignant tumours with histological features of the three embryonic layers. They have an elevated local recurrence rate, risk of metastasis and mortality. Moreover, the therapeutic options are limited, and optimal management is not yet clear. As fewer than 150 cases have been reported, therapeutic strategies remain a clinical challenge. Here, we discuss a case of a large SNTCS successfully treated with surgical resection followed by concurrent chemotherapy and radiation. Despite the significant size of the tumour and the inferred high recurrence risk, the patient has had no recurrence over the past 45 months. Although the optimal treatment of SNTCS is not clearly outlined, the very limited data suggests that a multidisciplinary approach with surgery, radiation and chemotherapy is the best option for patients.