Rationale for Individualized Therapy in Sinonasal Teratocarcinosarcoma (SNTC): Case Report

BACKGROUND: Sinonasal Teratocarcinosarcoma (SNTC) is a very unusual and aggressive neoplasm characterized by the combination of malignant teratoma and carcinosarcoma features. We present the first case of malignant SNTC treated with individualized multimodal therapy including a histology-specific chemotherapy. CASE REPORT: A 31-year-old man presented with an obstruction of the right pansinus. Histology showed an SNTC with major parts of small cell, poorly differentiated carcinoma and a small proportion of highly differentiated embryonal rhabdomyosarcoma. An operation was performed followed by intraoperative application of a 5-FU ointment. Adjuvant chemotherapy with cisplatin, etoposid and ifosfamid were given in regard to the major components of this heterogeneous tumor. Radiotherapy up to 59.4 Gy was applied. CONCLUSION: 36 months after the end of therapy, there is no sign of tumor recurrence or metastasis in our patient. We suggest that surgery, radiotherapy and a histology-specific multidrug chemotherapy seems to be a therapeutic approach that is appropriate for this heterogeneous tumor.