malignant peripheral nerve sheath tumors การใช้

• About 10 % of plexiform neurofibromas undergo transformation into a malignant peripheral nerve sheath tumor ( MPNST ).
• Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves.
• Features common in neurofibromatosis-Lisch nodules, bone abnormalities, neurofibromas, optic pathway gliomas and malignant peripheral nerve sheath tumors-are absent in this condition.
• There is a wide differential diagnosis, which includes spindle cell carcinoma, spindle cell melanoma, fibrosarcoma, malignant peripheral nerve sheath tumor and even biphenotypic sinonasal sarcoma.
• Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs & mdash; the lumbar plexuses & mdash; or from nerves as they arise from the trunk.
• In tumors, CD34 is found in alveolar soft part sarcoma, preB-ALL ( positive in 75 % ), AML ( 40 % ), AML-M7 ( most ), dermatofibrosarcoma protuberans, gastrointestinal stromal tumors, giant cell fibroblastoma, granulocytic sarcoma, Kaposi s sarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumors, mengingeal hemangiopericytomas, meningiomas, neurofibromas, schwannomas, and papillary thyroid carcinoma.