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.