Milad Darrat, Louis Lau, Colin Leonard, Stephen Cooke, Muhammad A. Shahzad, Claire McHenry, David R. McCance, Steven J. Hunter, Karen Mullan, John R. Lindsay, Una Graham, Neil Bailie, Susie Hampton, Simon Rajendran, Fionnuala Houghton, David Conkey, Patrick J. Morrison, Philip C. Johnston
{"title":"头颈部副神经节瘤（HNPGLs）的临床管理和疗效：单中心回顾性研究","authors":"Milad Darrat, Louis Lau, Colin Leonard, Stephen Cooke, Muhammad A. Shahzad, Claire McHenry, David R. McCance, Steven J. Hunter, Karen Mullan, John R. Lindsay, Una Graham, Neil Bailie, Susie Hampton, Simon Rajendran, Fionnuala Houghton, David Conkey, Patrick J. Morrison, Philip C. Johnston","doi":"10.1111/cen.15070","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Context</h3>\n \n <p>Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined.</p>\n </section>\n \n <section>\n \n <h3> Objective</h3>\n \n <p>To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17–91 years old). 58.6% (<i>n</i> = 51) of patients had carotid body tumours, 25.2% (<i>n</i> = 22) glomus vagal tumours, 6.8% (<i>n</i> = 6) tumours in the middle ear, 2.2% (<i>n</i> = 2) in the parapharyngeal space and 1.1% (<i>n</i> = 1) in the sphenoid sinus. 5.7% (<i>n</i> = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5–6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (<i>n</i> = 36), SDHB in 12.6% (<i>n</i> = 11), SDHC in 2.2% (<i>n</i> = 2) and SDHA in 1.1% (<i>n</i> = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (<i>n</i> = 45) of patients, radiotherapy in 14.9% (<i>n</i> = 13), observation in 28.7% (<i>n</i> = 25), and somatostatin analogue therapy with octreotide in 4.5% (<i>n</i> = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (<i>p</i> = .04), tumour size exceeding 2 cm (<i>p</i> = .03), positive SDHx variants (<i>p</i> = .01), and vagal and jugular tumours (<i>p</i> = .04).</p>\n </section>\n \n <section>\n \n <h3> Conclusion</h3>\n \n <p>The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.</p>\n </section>\n </div>","PeriodicalId":10346,"journal":{"name":"Clinical Endocrinology","volume":"101 3","pages":"243-248"},"PeriodicalIF":3.0000,"publicationDate":"2024-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study\",\"authors\":\"Milad Darrat, Louis Lau, Colin Leonard, Stephen Cooke, Muhammad A. Shahzad, Claire McHenry, David R. McCance, Steven J. Hunter, Karen Mullan, John R. Lindsay, Una Graham, Neil Bailie, Susie Hampton, Simon Rajendran, Fionnuala Houghton, David Conkey, Patrick J. Morrison, Philip C. Johnston\",\"doi\":\"10.1111/cen.15070\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Context</h3>\\n \\n <p>Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Objective</h3>\\n \\n <p>To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Methods</h3>\\n \\n <p>Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Results</h3>\\n \\n <p>There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17–91 years old). 58.6% (<i>n</i> = 51) of patients had carotid body tumours, 25.2% (<i>n</i> = 22) glomus vagal tumours, 6.8% (<i>n</i> = 6) tumours in the middle ear, 2.2% (<i>n</i> = 2) in the parapharyngeal space and 1.1% (<i>n</i> = 1) in the sphenoid sinus. 5.7% (<i>n</i> = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5–6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (<i>n</i> = 36), SDHB in 12.6% (<i>n</i> = 11), SDHC in 2.2% (<i>n</i> = 2) and SDHA in 1.1% (<i>n</i> = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (<i>n</i> = 45) of patients, radiotherapy in 14.9% (<i>n</i> = 13), observation in 28.7% (<i>n</i> = 25), and somatostatin analogue therapy with octreotide in 4.5% (<i>n</i> = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (<i>p</i> = .04), tumour size exceeding 2 cm (<i>p</i> = .03), positive SDHx variants (<i>p</i> = .01), and vagal and jugular tumours (<i>p</i> = .04).</p>\\n </section>\\n \\n <section>\\n \\n <h3> Conclusion</h3>\\n \\n <p>The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.</p>\\n </section>\\n </div>\",\"PeriodicalId\":10346,\"journal\":{\"name\":\"Clinical Endocrinology\",\"volume\":\"101 3\",\"pages\":\"243-248\"},\"PeriodicalIF\":3.0000,\"publicationDate\":\"2024-05-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Endocrinology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1111/cen.15070\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"ENDOCRINOLOGY & METABOLISM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Endocrinology","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/cen.15070","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"ENDOCRINOLOGY & METABOLISM","Score":null,"Total":0}
Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study
Context
Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined.
Objective
To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities.
Methods
Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023.
Results
There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17–91 years old). 58.6% (n = 51) of patients had carotid body tumours, 25.2% (n = 22) glomus vagal tumours, 6.8% (n = 6) tumours in the middle ear, 2.2% (n = 2) in the parapharyngeal space and 1.1% (n = 1) in the sphenoid sinus. 5.7% (n = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5–6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (n = 36), SDHB in 12.6% (n = 11), SDHC in 2.2% (n = 2) and SDHA in 1.1% (n = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (n = 45) of patients, radiotherapy in 14.9% (n = 13), observation in 28.7% (n = 25), and somatostatin analogue therapy with octreotide in 4.5% (n = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (p = .04), tumour size exceeding 2 cm (p = .03), positive SDHx variants (p = .01), and vagal and jugular tumours (p = .04).
Conclusion
The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.
期刊介绍：
Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.
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