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周围神经系统肿瘤。

Tumors of the peripheral nervous system.

作者信息

Ariel I M

出版信息

CA Cancer J Clin. 1983 Sep-Oct;33(5):282-99. doi: 10.3322/canjclin.33.5.282.

Abstract

Most tumors (benign and malignant) do not arise from the nerves per se, but from the supporting cells; tumors arising from the cells of Schwann are termed schwannoma or neurilemoma--benign or malignant. Surgical extirpation is the only way of treating these tumors. Radiation therapy can offer significant palliation and prolongation of life, but no cures have been observed. Other forms of therapy (chemotherapy, immunotherapy, etc.) have little to offer at this time. Benign tumors can be treated by local surgical extirpation; malignant tumors must be radically resected, including major amputations where indicated. Certain dermatomes have a proclivity to produce tumors, and new growths (benign and malignant) proximal to the one clinically apparent can be expected in certain patients. Neurofibromatosis (von Recklinghausen's disease) is a genetic error of metabolism with a proclivity to produce multiple neurofibrosarcomas, and in about 10 percent of the patients, malignant neurilemomas. Of 100 patients with malignant neurilemomas treated by the author, 74 were considered determinate; among them, the 10-year "cure" rate was 32 percent. Patients with von Recklinghausen's disease had almost as good a 10-year survival rate as those with solitary malignant schwannoma (30 percent vs 39 percent).

摘要

大多数肿瘤(良性和恶性)并非起源于神经本身,而是起源于支持细胞;起源于施万细胞的肿瘤被称为神经鞘瘤或神经膜瘤——有良性和恶性之分。手术切除是治疗这些肿瘤的唯一方法。放射治疗可提供显著的姑息治疗并延长生命,但尚未观察到治愈的情况。目前其他治疗形式(化疗、免疫治疗等)效果甚微。良性肿瘤可通过局部手术切除进行治疗;恶性肿瘤必须进行根治性切除,必要时包括大截肢。某些皮节易于产生肿瘤,在某些患者中,可预期在临床上明显的肿瘤近端出现新的生长物(良性和恶性)。神经纤维瘤病(冯·雷克林豪森病)是一种代谢遗传缺陷病,易于产生多发性神经纤维肉瘤,约10%的患者会发生恶性神经鞘瘤。在作者治疗的100例恶性神经鞘瘤患者中,74例被认为是确诊的;其中,10年“治愈”率为32%。患有冯·雷克林豪森病的患者10年生存率与患有孤立性恶性神经鞘瘤的患者几乎一样好(分别为30%和39%)。

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