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儿童和青少年头颈部软组织肉瘤

Soft tissue sarcoma of the head and neck in children and adolescents.

作者信息

Lyos A T, Goepfert H, Luna M A, Jaffe N, Malpica A

机构信息

Department of Head and Neck Surgery, University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.

出版信息

Cancer. 1996 Jan 1;77(1):193-200. doi: 10.1002/(SICI)1097-0142(19960101)77:1<193::AID-CNCR31>3.0.CO;2-Z.

Abstract

BACKGROUND

The experience of one institution in treating soft tissue sarcomas of the head and neck in a pediatric population is presented.

METHODS

Case materials of 134 patients younger than 20 years who were referred to the University of Texas M. D. Anderson Cancer Center between 1970 and 1989 for treatment of sarcoma of the head and neck were retrospectively reviewed. Patients with rhabdomyosarcoma underwent multimodality treatment consisting of surgery, irradiation, and chemotherapy. Wide resection was the treatment used for patients with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). Adjuvant chemotherapy and irradiation were used to treat high grade neoplasms and residual disease. The clinical response to therapy was measured in terms of the disease-specific survival rate.

RESULTS

Seventy-nine of 134 patients presented with untreated or biopsy-proven disease. Fifty-six had rhabdomyosarcoma and 23 had

NRSTS

At 2 and 5 years, the disease-specific survival rates for patients with rhabdomyosarcoma were 74% and 63%, respectively, and patients with NRSTS had 80% and 75% disease-specific survival rates at 2 and 5 years, respectively.

CONCLUSIONS

Rhabdomyosarcoma of the head and neck in children is effectively treated with multimodality therapy. Prognostic indicators for rhabdomyosarcoma include completeness of tumor resection and the development of recurrent disease. Aggressive surgical resection is the treatment of choice for patients with

NRSTS

Prognostic indicators for NRSTS include completeness of tumor resection and the development of recurrent disease.

摘要

背景

本文介绍了一家机构治疗儿童头颈部软组织肉瘤的经验。

方法

回顾性分析了1970年至1989年间转诊至德克萨斯大学MD安德森癌症中心治疗头颈部肉瘤的134例20岁以下患者的病例资料。横纹肌肉瘤患者接受了包括手术、放疗和化疗在内的多模式治疗。广泛切除是用于非横纹肌肉瘤性软组织肉瘤(NRSTS)患者的治疗方法。辅助化疗和放疗用于治疗高级别肿瘤和残留疾病。根据疾病特异性生存率来衡量治疗的临床反应。

结果

134例患者中有79例表现为未经治疗或活检证实的疾病。56例患有横纹肌肉瘤,23例患有NRSTS。横纹肌肉瘤患者在2年和5年时的疾病特异性生存率分别为74%和63%,而NRSTS患者在2年和5年时的疾病特异性生存率分别为80%和75%。

结论

儿童头颈部横纹肌肉瘤采用多模式治疗可得到有效治疗。横纹肌肉瘤的预后指标包括肿瘤切除的完整性和复发性疾病的发生。积极的手术切除是NRSTS患者的首选治疗方法。NRSTS的预后指标包括肿瘤切除的完整性和复发性疾病的发生。

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