SARCOMAS OF PERIPHERAL NERVES AND SOMATIC SOFT TISSUES ASSOCIATED WITH MULTIPLE NEUROFIBROMATOSIS (VON RECKLINGHAUSENS DISEASE)
SARCOMAS OF PERIPHERAL NERVES AND SOMATIC SOFT TISSUES ASSOCIATED WITH MULTIPLE NEUROFIBROMATOSIS (VON RECKLINGHAUSENS DISEASE)
复制标题
DOI：
10.1002/1097-0142(196308)16:8
复制
发表时间：
1963-01-01
期刊：
影响因子：
6.2
通讯作者：
SOULE, EH
中科院分区：
文献类型：
--
作者：
DAGOSTINO, AM;MILLER, RH;SOULE, EH
本文报告21例多发性神经纤维瘤病（vonRecklinghausen病）并发神经和躯体软组织恶性肿瘤的临床和病理表现。恶性肿瘤在组织学上分为两型，即12例均匀梭形细胞肉瘤（恶性神经鞘瘤）和9例多形性肉瘤。后者中3例为横纹肌肉瘤; 1例为脂肪肉瘤; 5例未分类。我们的研究并未证实良性神经纤维瘤手术后的恶变。多发性神经纤维瘤病患者的恶性转化没有准确的发生率。在12例中，神经纤维瘤和恶性肿瘤，但只有2例肿瘤的恶性和良性组织密切相关。手术切除后，已知2例患者存活，1例存活13 1/2年，1例存活3年。2例患者的病程我们尚不清楚。其余17例患者死亡，16例死于肿瘤，1例死于肉瘤切除后1年半的转移性肾上腺瘤。肉瘤的位置和组织学类型似乎对预后没有任何直接影响。在我们的研究中没有证据表明多发性神经纤维瘤病相关的肉瘤比其他类型的肉瘤恶性程度低或转移率低。疼痛的发作或预先存在的肿块的快速扩大或新肿块的形成，特别是在深层软组织中，应提示癌症的发作。迅速、彻底的消融手术似乎是病人唯一的救星。
The clinical and pathological findings are presented for 21 patients with multiple neurofibromatosis (von Recklinghausen''s disease) complicated by the development of malignant tumors of nerves and somatic soft tissue. The malignant tumors were divided into 2 types on a histological basis, namely 12 uniform spindle cell sarcomas (malignant neurilemomas) and 9 pleomorphic sarcomas. Three of the latter were rhabdomyosarcomas; 1, a liposarcoma; and 5 were unclassified. That malignant transformation follows surgical procedures on benign neurofibromas was not substantiated by our study. No accurate incidence could be established for malignant transformation in patients with multiple neurofibromatosis. In 12 instances, both neurofibromas and malignant tumors were present but in only 2 tumors were the malignant and benign tissues intimately associated. Two patients are known to be alive, 1 for 13 1/2 years and 1 for 3 years, following surgical excision. The course of 2 patients is unknown to us. The remaining 17 patients are dead, 16 as a result of the tumors and 1 from metastatic hypernephroma 1 1/2 years after excision of the sarcoma. The location and the histological type of the sarcoma did not seem to have any direct bearing on prognosis. There is no evidence in our study that sarcomas associated with multiple neurofibromatosis are less malignant or metastasize less frequently than do other types of sarcoma. The onset of pain or the rapid enlargement of a pre-existing mass or formation of a new mass, particularly in the deep soft tissues should suggest the onset of cancer. Prompt, radical, ablative surgery appears to offer the only salvation for the patient.