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.
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.
Authors: Shih-Chiang Huang; Ronald A Ghossein; Justin A Bishop; Lei Zhang; Tse-Ching Chen; Hsuan-Ying Huang; Cristina R Antonescu Journal: Am J Surg Pathol Date: 2016-01 Impact factor: 6.394
Authors: Simon Andreasen; Justin A Bishop; Henrik Hellquist; Jennifer Hunt; Katalin Kiss; Alessandra Rinaldo; Alena Skálová; Stefan M Willems; Michelle Williams; Alfio Ferlito Journal: Virchows Arch Date: 2018-08-14 Impact factor: 4.064
Authors: Justin A Bishop; Lester D R Thompson; Antonio Cardesa; Leon Barnes; James S Lewis; Asterios Triantafyllou; Henrik Hellquist; Goran Stenman; Jennifer L Hunt; Michelle D Williams; Pieter J Slootweg; Kenneth O Devaney; Douglas R Gnepp; Bruce M Wenig; Alessandra Rinaldo; Alfio Ferlito Journal: Head Neck Pathol Date: 2015-03-11
Authors: Ikram Chaudhry; Thabet Algazal; Ahsan Cheema; Aman Al Faraj; Noor Al Malki; Hadi Mutairi; Ahmed Abbas; Samir Amr Journal: Int J Surg Case Rep Date: 2019-08-20