Characterization of Malignant Head and Neck Paragangliomas at a Single Institution Across Multiple Decades

医学 SDHB系统 恶性肿瘤 颈淋巴结清扫术 头颈部癌 转移 内科学 回顾性队列研究 头颈部 副神经节瘤 肿瘤科 队列 种系突变 癌症 病理 突变 基因 外科 生物 遗传学
作者
Hilary C. McCrary,Eric Babajanian,Matias Calquin,Patrick Carpenter,Geoffrey C. Casazza,Anne Naumer,Samantha Greenberg,Wendy Kohlmann,Richard B. Cannon,Marcus M. Monroe,Jason P. Hunt,Luke Buchmann
出处
期刊:JAMA otolaryngology-- head & neck surgery [American Medical Association]
卷期号:145 (7): 641-641 被引量:29
标识
DOI:10.1001/jamaoto.2019.1110
摘要

Importance

Malignant head and neck paragangliomas (HNPGLs) are rare entities, and there are limited data regarding optimal treatment recommendations to improve clinical outcomes.

Objective

To classify succinate dehydrogenase (SDH) germline mutations associated with malignant HNPGLs, evaluate time from diagnosis to identification of malignant tumor, describe locations of metastases and the functional status of malignant HNPGLs, and determine the role of selective neck dissection at the time of initial surgical resection.

Design, Setting, and Participants

A retrospective cohort study was completed of patients diagnosed with paragangliomas on various sites on the body at an academic tertiary cancer hospital between the years 1963 and 2018. A subanalysis of HNPGLs was also completed. Data regarding diagnosis, gene and mutation, tumor characteristics and location, and treatments used were reviewed between February 2017 and March 2018.

Main Outcomes and Measures

Mutations ofSDHgenes associated with benign and malignant HNPGLs, treatments used, time to the discovery of malignancy, and location of metastasis.

Results

Of the 70 patients included in the study, 40 (57%) were male, and the mean (SD) age was 47 (21.1) years. Of patients with tumors isolated to the head and neck, 38 (54%) had benign HNPGLs, which were associated with mutations in the genesSDH subunit B (SDHB) (n = 18; 47%),SDHsubunit C (n = 2; 5%), andSDH subunit D (n = 18; 47%). Among those with malignant HNPGLs, all but 1 patient had mutations inSDHB(n = 5; 83%); 1 patient had no mutation associated with their disease. The average age at diagnosis for malignant HNPGLs was 35 years, while benign tumors were diagnosed at an average age at 36 years. All patients with malignant disease underwent surgery. Four patients were found to have metastasis at the time of selective neck dissection. Among patients with malignant HNPGLs, 5 (83%) were treated with adjuvant radiation, and 1 (17%) was treated with adjuvant chemotherapy.

Conclusions and Relevance

Malignant HNPGLs are rare entities that are difficult to diagnose and are typically identified by the presence of regional or distant metastasis. The results of this study found the prevalence of malignant HNPGLs to be 9%. These data suggest that it is beneficial to perform a selective neck dissection at the time of tumor excision. All patients with malignant HNPGLs but 1 hadSDHBmutations.

科研通智能强力驱动
Strongly Powered by AbleSci AI
更新
大幅提高文件上传限制,最高150M (2024-4-1)

科研通是完全免费的文献互助平台,具备全网最快的应助速度,最高的求助完成率。 对每一个文献求助,科研通都将尽心尽力,给求助人一个满意的交代。
实时播报
科研小白完成签到,获得积分10
2秒前
我是老大应助Darknewnew采纳,获得10
5秒前
summer完成签到 ,获得积分10
11秒前
工藤新一完成签到 ,获得积分10
14秒前
15秒前
yanzu应助labordoc采纳,获得10
15秒前
科研小白发布了新的文献求助10
16秒前
电子屎壳郎完成签到,获得积分10
17秒前
初色完成签到,获得积分10
17秒前
marska完成签到,获得积分10
17秒前
xx完成签到 ,获得积分10
18秒前
unowhoiam完成签到 ,获得积分10
19秒前
Darknewnew发布了新的文献求助10
19秒前
可可西里完成签到 ,获得积分10
31秒前
33秒前
mike2012完成签到 ,获得积分10
36秒前
张沐泽发布了新的文献求助10
37秒前
Darknewnew发布了新的文献求助10
38秒前
ZD飞完成签到,获得积分10
41秒前
41秒前
labordoc完成签到,获得积分10
45秒前
小何发布了新的文献求助10
45秒前
45秒前
Darknewnew完成签到,获得积分10
46秒前
陈大西罗完成签到 ,获得积分10
50秒前
hhan完成签到,获得积分10
54秒前
54秒前
xiaohong完成签到 ,获得积分0
55秒前
嘻哈学习完成签到,获得积分10
1分钟前
1分钟前
Agnesma完成签到,获得积分10
1分钟前
慧慧完成签到 ,获得积分10
1分钟前
111完成签到,获得积分10
1分钟前
fanssw完成签到 ,获得积分10
1分钟前
大鹏完成签到,获得积分10
1分钟前
1分钟前
喵咪西西完成签到 ,获得积分10
1分钟前
合适的寄灵完成签到 ,获得积分10
1分钟前
InfoNinja应助追寻的白秋采纳,获得30
1分钟前
科研小萌新完成签到 ,获得积分10
1分钟前
高分求助中
LNG地下式貯槽指針(JGA Guideline-107)(LNG underground storage tank guidelines) 1000
Generalized Linear Mixed Models 第二版 1000
rhetoric, logic and argumentation: a guide to student writers 1000
QMS18Ed2 | process management. 2nd ed 1000
Asymptotically optimum binary codes with correction for losses of one or two adjacent bits 800
Preparation and Characterization of Five Amino-Modified Hyper-Crosslinked Polymers and Performance Evaluation for Aged Transformer Oil Reclamation 700
Operative Techniques in Pediatric Orthopaedic Surgery 510
热门求助领域 (近24小时)
化学 医学 材料科学 生物 工程类 有机化学 生物化学 物理 内科学 纳米技术 计算机科学 化学工程 复合材料 基因 遗传学 物理化学 催化作用 免疫学 细胞生物学 电极
热门帖子
关注 科研通微信公众号,转发送积分 2924562
求助须知:如何正确求助?哪些是违规求助? 2571038
关于积分的说明 6944537
捐赠科研通 2224536
什么是DOI,文献DOI怎么找? 1182444
版权声明 589054
科研通“疑难数据库(出版商)”最低求助积分说明 578628