Shields T W, Reynolds M
Northwestern University Medical School, Chicago, Illinois.
Surg Clin North Am. 1988 Jun;68(3):645-68. doi: 10.1016/s0039-6109(16)44538-x.
Neurogenic tumors of the thorax are observed in all age groups, although they are rare in the elderly. They are more likely to be malignant in the child than in the adult. Tumors of the autonomic system are common in children, whereas the nerve sheath tumors are more likely to be found in adults. The malignant lesions are almost always symptomatic and the benign lesions asymptomatic, except in the child, in whom a benign lesion may result in symptoms because of its size relative to the volume of the child's thorax. Intraspinal canal extension, although relatively infrequent, should be sought for in all paravertebral tumors, for a significant percentage of these may be initially asymptomatic. Excision of such an hourglass tumor without foreknowledge of such extension may lead to serious spinal cord complications. Surgical excision of most of these tumors is sufficient except for the malignant lesions, especially in infancy and childhood, for which the addition of postoperative irradiation and chemotherapy may be beneficial. The prognosis after the removal of benign neurogenic tumors of the thorax is excellent. In the infant or child with Stage III or IV neuroblastoma, ganglioneuroblastoma, or an Askin tumor, the prognosis is poor. In the adult with a malignant neurogenic sarcoma or a malignant paraganglioma, the prognosis likewise is grave.
胸部神经源性肿瘤可见于所有年龄组,不过在老年人中较为罕见。儿童患者的肿瘤比成人患者的肿瘤更易恶变。自主神经系统肿瘤在儿童中较为常见,而神经鞘瘤则更常见于成人。恶性病变几乎总是有症状的,良性病变通常无症状,但在儿童中,由于良性病变相对于儿童胸腔体积的大小,可能会导致症状。椎管内扩展虽然相对少见,但对于所有椎旁肿瘤都应进行检查,因为其中相当一部分最初可能无症状。在未事先知晓这种扩展的情况下切除这种哑铃形肿瘤可能会导致严重的脊髓并发症。除恶性病变外,大多数此类肿瘤手术切除即可,尤其是在婴儿期和儿童期,术后加用放疗和化疗可能有益。切除胸部良性神经源性肿瘤后的预后极佳。患有III期或IV期神经母细胞瘤、神经节神经母细胞瘤或Askin肿瘤的婴儿或儿童,预后较差。患有恶性神经源性肉瘤或恶性副神经节瘤的成人,预后同样严重。