头颈部嵴索瘤及软骨肉瘤治疗一文知道

编译:肿瘤资讯来源:肿瘤资讯专家介绍:

Ying J. Hitchcock教授

犹他州立大学亨斯迈癌症医院放射肿瘤科主任医师和教授

Hitchcock教授擅长头颈部,肺,皮肤癌和成人软骨肉瘤,担任美国NCCN头颈组成员。今天跟我们分享的是头颈部嵴索瘤及软骨肉瘤的诊疗。

1. 嵴索瘤是一种罕见的恶性肿瘤,起源于原始嵴索的残余部分,占原发性骨肿瘤的1%至4%,在美国每年每10万人中有0.08例。嵴索瘤是生长缓慢但局部具有破坏性的肿瘤。其具有低转移风险,但10%-40%的患者可能发生转移。

2.

嵴索瘤的治疗:嵴索瘤的主要失败模式是局部复发,局部复发后的挽救治疗可以在一段时间内实现疾病控制,但最终预后很差。因此,首次规范性治疗尤为重要。如果可行的话,手术是嵴索瘤的首要治疗手段。据报导,切缘阴性的完整切除后,可获得70-80%的局部控制率。然而,如果手术边缘呈阳性,则局部失败率为高达70%。目前嵴索瘤治疗没有有效的化疗药物。放疗联合手术切除是颅底嵴索瘤的最佳治疗方法。据报导,颅底嵴索瘤的治疗会产生严重的远期毒性,如垂体机能减退、记忆障碍、颅神经损伤、感音神经性耳聋、中枢神经系统坏死等。

3. 软骨肉瘤可能在任何年龄出现,但通常发生在中年和老年人,恶性转化风险约为5%。远处转移和软骨肉瘤恶性程度有关: 低度恶性: <10%;中度恶性: 10-50%;高度恶性: 50-70%。

4. 软骨肉瘤的治疗:依赖于肿瘤的组织学分级、肿瘤的位置及其范围。对于低度恶性肿瘤,仅手术切除就足以达到较高的疾病控制率,其允许经瘤切除或刮除法。对于中高度恶性肿瘤,广泛的整体切除是最理想的治疗手段。对于未能整体切除的高度恶性肿瘤,放射治疗可以提高局部控制率。对于软骨肉瘤,推荐术前放疗剂量50 Gy; 阳性切缘剂量60-66 Gy;

不可切除的或残留肿瘤70Gy。PMH报告了手术联合放疗治疗高危颅外软骨肉瘤的临床结果,经过75个月随访,R0、R1和R2切除的患者的局部控制率分别为100%、94%和42%,10年的总生存率为86%。MGH等研究显示对200例颅底软骨肉瘤的患者进行光子质子联合放疗治疗(平均剂量72 CGE), 10年局部控制率可达到98%。

英文原文

Management of Head Neck Chordoma and Chondrosarcoma

Ying J. Hitchcock

Department of radiation oncology in Huntsman cancer institution, university of Utah

Professor Hitchcock is the medical director and a professor in the department of radiation oncology in Huntsman cancer institution, university of Utah, specializing in head and neck, lung, and

skin cancers and in adult soft tissue sarcomas. She is the National Comprehensive Cancer Network Head Neck Cancer Panel Member. Today, Dr. Ying Hitchcock will share us about 「Management of Head Neck

Chordoma and Chondrosarcoma」.

1. Chordoma is a rare, malignant neoplasm arising from the remnant of the primitive notochord, accounts for 1% to 4% of primary bone tumors, annual 0.08 cases per 100,000 in the United States.

Chordomas are slow-growing but locally destructive tumors. Chordomas have a low metastatic potential, but metastases may occur in as many as 10% to 40% of patients.

2. Treatment of chordoma. The dominant failure pattern of the chordoma is local recurrence. Salvage after local recurrence can achieve disease control for a period of time, but the ultimate

outcomes tends to be poor. Thus, aggressive upfront treatment affords the best potential for cure. Surgery has been the primary approach for these tumors if feasible. It has been reported 70-80%

local control if complete en bloc resection with negative margins is achieved. However, local failure is >70% if surgical margins are positive. Chemotherapy is believed to be inactive in chordoma,

so it does not play a role in primary definitive management of these tumors. Radiotherapy combined with maximal surgical resection is considered as optimal therapy for skull base chordoma. Serious

long-term side effects from treating base of skull chordoma have been reported for hypopituitarism, memory impairment, cranial nerve injury, sensory neural hearing loss, and central nervous system

necrosis, etc..

3. Chondrosarcoma may arise at any age but typically occurs in middle-aged and older adults, malignant transformation rate is about 5%. Distant metastases depend on tumor grade: < 10% in

low-grade tumor, 10-50% in intermediate-grade lesions, and 50-70% in high-grade lesions.

4. Treatment approach depends on tumor histologic grade, tumor location and its extension. For low-grade tumors, surgical resection alone is sufficient to achieve a high rate of disease control;

it is oaky to undergo intralesional excision or curettage procedure. For the less common intermediate- and high-grade tumors, wide en bloc excision is the optimal surgical management. Radiation

therapy is indicated to improve on high local control rates after incomplete resection of high-risk tumors. For soft tissue chondrosarcoma, preoperative RT is indicated; 50 Gy preop RT; 60-66 Gy for

close or positive margins; 70 Gy or high for gross residual or unresectable disease. PMH reported their experience of combined surgery and radiation therapy for high-risk extracranial chondrosarcoma.

With median follow-up of 75 months, local control rates of 100%, 94%, and 42% for R0, R1, and R2 resected patients, respectively. Ten-year overall survival was 86%. MGH reported 200 patients with

base-of-skull chondrosarcoma treated using a combination of photon and proton radiation therapy. With median dose of 72 cobalt-gray-equivalent (CGE), a 10-year local control rate of 98%.

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