1 | INTRODUCTION
Malignant triton tumors (MTTs) are a rare rhabdomyosarcoma-like differentiated subtype in malignant peripheral nerve sheath tumors (MPNSTs), accounting for 5% of all MPNSTs.1 As many as 50% of MPNSTs occur in patients with neurofibromatosis type 1 (NF-1).2 MTTs are aggressive, often difficult to completely remove, and the prognosis are poor, which may be related to certain gene mutations.3 It is known that individuals with mutations in the NF1 tumor suppressor gene will develop into NF- I and MPNSTs. However, few cytogenetic studies specific to MTTs have been performed. At present, the relationship between the genetic test results of MTTs and clinical manifestations is not completely clear. Here, we report a rare case of neck MTTs with mutations in NF1 and FANCD2 genes, without clinical manifestations of NF- I. As far as we know, this is the first case of MTT with mutations in the NF1 and FANCD2 genes, without clinical manifestations of NF- 1.