MPNST is a very uncommon malignant type of neoplasm. It is often associated with neurofibromatosis type 1 (von Recklinghausen disease). It involves large anatomical regions, and thus takes on varied clinical presentat...MPNST is a very uncommon malignant type of neoplasm. It is often associated with neurofibromatosis type 1 (von Recklinghausen disease). It involves large anatomical regions, and thus takes on varied clinical presentations. However, bone location of MPNST, particularly in the spinal canal has been poorly described in the literature. We hereby report the case of a 29-year old young man with MPNST in the spinal canal. He presented a slow spinal cord compression confirmed by spinal MRI. MPNST was revealed through histologic and immune histochemical features after tumor resection.展开更多
Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromato...Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromatosis type 1 (NF1) who had a growing abdominal wall tumor diagnosed as MTT. Computed tomography (CT), magnetic resonance imaging (MRI) and 2-[F-18]-fluoro-2-deoxy-D-glucose positron emission tomography/CT (FDG-PET/CT) were performed. The MRI and FDG-PET/CT indicated that the lateral component of the tumor was composed of many proliferative cells, corresponding to the histopathological finding of a cellular proliferation of spindle-shaped cells. In light of this case and previous reports, it is apparent that FDG-PET/CT is a helpful tool for distinguishing MTT from benign peripheral nerve sheath tumor.展开更多
A malignant peripheral nerve sheath tumor(MPNST)is a rare neoplasm arising f rom peripheral nerve sheath.Here,we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastroint...A malignant peripheral nerve sheath tumor(MPNST)is a rare neoplasm arising f rom peripheral nerve sheath.Here,we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastrointestinal tract.The patient was a 2-day-old neonate with symptoms and signs of intestinal obstruc tion.The patient had no family history or stigmata of neurofibromatosis type 1.A computed tomographic scan revealed a 5-cm-sized mass in ascending colon cau sing intestinal obstruction,and emergent right hemicolectomy was performed.The microscopic examination showed atypical spindle cells with hyperchromatic nucle i and high mitotic activity.The results of immunohistochemical staining,which showed positivity for S-100 and vimentin as well as negativity for smooth muscl e actin,CD34,and c-Kit,supported the final diagnosis of MPNST.Genetic analy sis of the patient revealed no abnormalities.After surgery,the patient recover ed uneventfully and has been free of the disease for 17 months.展开更多
Dear editor,Malignant peripheral nerve sheath tumors(MPNSTs)are rare,aggressive sarcomas arising from peripheral nerves or pre-existing neurofibromas,accounting for approximately 5%10%of all soft tissue sarcomas[1].Th...Dear editor,Malignant peripheral nerve sheath tumors(MPNSTs)are rare,aggressive sarcomas arising from peripheral nerves or pre-existing neurofibromas,accounting for approximately 5%10%of all soft tissue sarcomas[1].These tumors are notoriously heterogeneous:approximately 50%associated with neurofibromatosis type 1(NF1),40%occurring sporadically,and 10%linked to prior radiation exposure[2].展开更多
文摘MPNST is a very uncommon malignant type of neoplasm. It is often associated with neurofibromatosis type 1 (von Recklinghausen disease). It involves large anatomical regions, and thus takes on varied clinical presentations. However, bone location of MPNST, particularly in the spinal canal has been poorly described in the literature. We hereby report the case of a 29-year old young man with MPNST in the spinal canal. He presented a slow spinal cord compression confirmed by spinal MRI. MPNST was revealed through histologic and immune histochemical features after tumor resection.
文摘Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromatosis type 1 (NF1) who had a growing abdominal wall tumor diagnosed as MTT. Computed tomography (CT), magnetic resonance imaging (MRI) and 2-[F-18]-fluoro-2-deoxy-D-glucose positron emission tomography/CT (FDG-PET/CT) were performed. The MRI and FDG-PET/CT indicated that the lateral component of the tumor was composed of many proliferative cells, corresponding to the histopathological finding of a cellular proliferation of spindle-shaped cells. In light of this case and previous reports, it is apparent that FDG-PET/CT is a helpful tool for distinguishing MTT from benign peripheral nerve sheath tumor.
文摘A malignant peripheral nerve sheath tumor(MPNST)is a rare neoplasm arising f rom peripheral nerve sheath.Here,we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastrointestinal tract.The patient was a 2-day-old neonate with symptoms and signs of intestinal obstruc tion.The patient had no family history or stigmata of neurofibromatosis type 1.A computed tomographic scan revealed a 5-cm-sized mass in ascending colon cau sing intestinal obstruction,and emergent right hemicolectomy was performed.The microscopic examination showed atypical spindle cells with hyperchromatic nucle i and high mitotic activity.The results of immunohistochemical staining,which showed positivity for S-100 and vimentin as well as negativity for smooth muscl e actin,CD34,and c-Kit,supported the final diagnosis of MPNST.Genetic analy sis of the patient revealed no abnormalities.After surgery,the patient recover ed uneventfully and has been free of the disease for 17 months.
基金supported by the grant:the National Key Research and Development Program of China(No.2023YFC2508500)the National Natural Science Foundation of China(Nos.82272951 and 82272953).
文摘Dear editor,Malignant peripheral nerve sheath tumors(MPNSTs)are rare,aggressive sarcomas arising from peripheral nerves or pre-existing neurofibromas,accounting for approximately 5%10%of all soft tissue sarcomas[1].These tumors are notoriously heterogeneous:approximately 50%associated with neurofibromatosis type 1(NF1),40%occurring sporadically,and 10%linked to prior radiation exposure[2].
