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中文Malignant Triton Tumor (MTT)
Alias:
Malignant Peripheral Nerve Sheath Tumor with Rhabdomyosarcomatous Differenciation
Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation
Malignant Peripheral Nerve Sheath Neoplasm with Rhabdomyosarcoma
Mpnst with Rhabdomyosarcomatous Differentiation
Mpnst with Rhabdomyoblastic Differentiation
Mpnst with Rhabdomyosarcoma
Malignant Triton Tumour
Mtt
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恶性特里顿瘤，又称为具有横纹肌样分化恶性周围神经鞘瘤，与神经纤维瘤病1型和恶性周围神经鞘瘤有关。与恶性特里顿瘤相关的重要基因是HTRA1（HtrA丝氨酸肽酶1），其相关通路/超级通路包括间充质干细胞和特异性标记物以及DUX4在FSHD1中的病理生理作用。在该疾病的背景下，已提到的药物有异环磷酰胺和多柔比星。附属组织包括小肠和肺，相关表型为增殖活力和肌肉。
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