Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells. It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis. Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival. However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor. The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors. We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.
From the *Department of Otorhinolaryngology, Hospital Universitario Central de Asturias, IUOPA; and †Department of Pathology, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain.
Received February 7, 2009.
Accepted for publication March 22, 2009.
Address correspondence and reprint requests to Fernando López Álvarez, MD, c/ Marcos Peña Royo 20-4A, 33013 Oviedo, Asturias, Spain; E-mail: firstname.lastname@example.org
This study was supported by grants RD0&/0020/0034 of Red Temática de Investigación Cooperativa en Cáncer (RTICC), Spain.