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嗅神经母细胞瘤:预后与治疗

Esthesioneuroblastoma: prognosis and management.

作者信息

Morita A, Ebersold M J, Olsen K D, Foote R L, Lewis J E, Quast L M

机构信息

Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota.

出版信息

Neurosurgery. 1993 May;32(5):706-14; discussion 714-5. doi: 10.1227/00006123-199305000-00002.

DOI:10.1227/00006123-199305000-00002
PMID:8492845
Abstract

Forty-nine patients with esthesioneuroblastoma were treated at the Mayo Clinic between 1951 and 1990. Their clinical manifestations and treatment results were reviewed to identify possible prognostic factors. The 5-year survival rate for all patients was 69%. Tumor progression occurred in 25 patients (51%; no local control in 6 and local recurrence in 19). Metastasis was found in 15 patients (31%; regional in 10 and distant in 9). Nineteen patients died directly from metastatic or intracranial tumor extension. The pathological grade of the tumor was the most significant prognostic factor identified. The 5-year survival rate was 80% for the low-grade tumors and 40% for the high-grade tumors (P = 0.0001). Surgical treatment alone is effective for low-grade tumors if tumor-free margins can be obtained. Radiation is used for low-grade tumors when margins are close, for residual or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Recurrent tumor and regional metastasis should be treated aggressively because this approach has been shown to be worthwhile. A craniofacial resection is now the surgical procedure performed in all cases. Because recurrence can occur after 5 or even 10 years, long-term follow-up is mandatory.

摘要

1951年至1990年间,梅奥诊所对49例嗅神经母细胞瘤患者进行了治疗。回顾了他们的临床表现和治疗结果,以确定可能的预后因素。所有患者的5年生存率为69%。25例患者(51%)出现肿瘤进展(6例无局部控制,19例局部复发)。15例患者(31%)发生转移(10例为区域转移,9例为远处转移)。19例患者直接死于转移性肿瘤或颅内肿瘤扩展。肿瘤的病理分级是确定的最显著的预后因素。低级别肿瘤的5年生存率为80%,高级别肿瘤为40%(P = 0.0001)。如果能获得无瘤切缘,单纯手术治疗对低级别肿瘤有效。当切缘接近时,低级别肿瘤、残留或复发性疾病以及所有高级别癌症均采用放疗。与高级别肿瘤相关的预后不良可能也需要加用化疗。复发性肿瘤和区域转移应积极治疗,因为这种方法已被证明是值得的。目前所有病例均采用颅面切除术。由于复发可能在5年甚至10年后发生,因此必须进行长期随访。

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