Prognostic factors for the malignant triton tumor of the head and neck

• Published in: Head & neck 2009-05, Vol.31 (5), p.679-688
• Main Authors: Terzic, Andrej, Bode, Beata, Gratz, Klaus W., Stoeckli, Sandro J.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Biological and medical sciences; Female; Head and Neck Neoplasms - mortality; Head and Neck Neoplasms - pathology; Head and Neck Neoplasms - therapy; Humans; Male; malignant peripheral nerve sheath tumor; malignant triton tumor; Medical sciences; Middle Aged; Nerve Sheath Neoplasms - mortality; Nerve Sheath Neoplasms - pathology; Nerve Sheath Neoplasms - therapy; Neurology; Otorhinolaryngology. Stomatology; Prognosis; rhabdomyoblastic differentiation; Survival Analysis; treatment; Tumors of the nervous system. Phacomatoses
• ISSN: 1043-3074; 1097-0347
• DOI: 10.1002/hed.21051

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. Conclusion. Location of the primary tumor is a key factor for prognosis. Complete surgical removal is the only treatment associated with survival.© 2009 Wiley Periodicals, Inc. Head Neck, 2009