Malignant triton tumor of the oral cavity: a rare occurrence

Abstract

Malignant peripheral nerve sheath tumors (MPNST) arise from Schwann cells or within existing neurofibromas and, as a group, account for approximately 5–10% of all soft tissue sarcomas [1]. AlthoughMPNST may consist of tissues such as glandular epithelium, squamous cell, cartilage, or bones, those which possess only skeletal muscle differentiation are referred to as malignant triton tumor (MTT). The tumor consists of two cell lines, rabdomyoblasts and Schwann cells [2]. Rhabdomyosarcomatous elements were first described by Mason in patients with neurofibromatosis [3]. One third of these tumors arise in the head and neck region and at least one third are associated with neurofibromatosis type 1 [4]. The biological behavior of this tumor is more aggressive than the usual malignant schwannoma. It shows rapid growth, early metastases, and poor prognosis [5] and therefore should be regarded as a distinct clinical entity. We present a case with MTT of the oral cavity which is a rare localization for this tumor.