Unusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differ

• 作 者: Smita Shete Saroj Bolde Gopal Pandit Pushkar Matkari Sachin B Ingle
• 机 构: Department of Pathology, Dr. Vaishampayan Memorial Government Medical College,Department of Patholog
• 出 处: World Journal of Clinical Cases
• 发表日期: 2015年04期
• 关键词: sheath histological recurrence excision aggressive herein spots prognosis report

Malignant peripheral nerve sheath tumor(MPNST)with rhabdomyoblastic differentiation is called as malignant triton tumor(MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis(NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.

Malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation is called as malignant triton tumor (MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an Enlarge mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.