Prognostic factors for the malignant triton tumor of the head and neck.
BACKGROUND
Malignant triton tumors are rare neoplasias consisting of a malignant peripheral nerve sheath tumor with additional rhabdomyoblastic differentiation. These tumors are highly aggressive and prognosis is poor. Our aim is to describe the outcome and to identify potential prognostic factors.


METHODS
From 1993 to 2005, 7 patients with a malignant triton tumor of the head and neck were treated at our institution. A literature search revealed another 46 published cases. All these cases were analyzed for outcome and prognostic factors.


RESULTS
Patients with primary tumors involving the nose and paranasal sinuses have better, patients involving the neck a poor prognosis. All other locations show an intermediate course. Complete surgical removal is of crucial importance. Additional radiation or chemotherapy show little effect.


CONCLUSIONS
Location of the primary tumor is a key factor for prognosis. Complete surgical removal is the only treatment associated with survival.
Terzic
Andrej
A
Department of Cranio-Maxillofacial Surgery, University Hospital Zurich, Zürich, Switzerland. andrej.terzic@hcuge.ch
Bode
Beata
B
Gratz
Klaus W
KW
Stoeckli
Sandro J
SJ
D000328:Adult; D000368:Aged; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D016019:Survival Analysis