Management of head and neck paragangliomas: Review of 120 patients

Papaspyrou, Konstantinos; Mann, Wolf J.; Amedee, Ronald G.

doi:10.1002/hed.20967

Cited by 70 publications

(70 citation statements)

References 44 publications

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“…Glomus Tympanicum (GT) and Glomus Jugulare (GJ) are the next most common, each representing approximately 15% [16]. Within this cohort GT was the most common tumour.…”

Section: Discussionmentioning

confidence: 99%

Head and Neck Paraganglioma: Medical Assessment, Management, and Literature Update

Hayward¹,

Cousins

2017

JOHBM

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Head and neck paraganglioma (HNPGL) are rare, highly vascular; typically slow growing and mostly benign neoplasms arising from paraganglia cells. HNPGL cause morbidity via mass effect on adjacent structures (particularly the cranial nerves), invasion of the skull base and, rarely, catecholamine secretion with associated systemic effects. The last decade has seen significant progress in the understanding of HNPGL genetics, with pertinent implications for diagnostic assessment and management of patients and their relatives. The implicated genes code for three of the five subunits of mitochondrial enzyme succinate dehydrogenase (SDH); recent literature reports that approximately one third of all HNPGL are associated with SDH mutations-a prevalence significantly greater than traditionally thought. There are distinct phenotypical syndromes associated with mutations in each individual SDH subunit (SDHD, SDHB, SDHC, and SDHAF2). This article focuses on the clinical features of HNPGL, the implications of HNPGL genetics, and the current evidence relating to optimal identification, investigation, and management options in HNPGL, which are supported by reference to a personal series of 60 cases. HNPGL require a systematic and thorough assessment to appropriately guide management decisions, and a suggested algorithm is presented in this article. Recent developments are particularly pertinent to surgeons of multiple disciplines, including otolaryngology, neurosurgery, vascular, and general surgery.

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“…Glomus Tympanicum (GT) and Glomus Jugulare (GJ) are the next most common, each representing approximately 15% [16]. Within this cohort GT was the most common tumour.…”

Section: Discussionmentioning

confidence: 99%

Head and Neck Paraganglioma: Medical Assessment, Management, and Literature Update

Hayward¹,

Cousins

2017

JOHBM

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“…However, the low incidence (8%) of malignant PGL in Dutch SDHD variant carriers probably means that it has little influence on overall mortality rates. 4 An expected increase in mortality due to the serious morbidity that may result from (treatment of) HNPGLs and the potentially fatal course of (untreated) PCC 6,8,29,30 was not seen, which could be partly due to the LUMC follow-up policy. First, although 90% of SDHD variant carriers in our cohort developed a HNPGL, only 35% of these patients were treated.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4] Although the majority of HNPGLs are benign and indolent tumors, 5 their location in close proximity to important neurovascular structures may lead to serious morbidity. 6 Neurovascular complications occur in up to 60% of cases following surgical treatment, for example, cranial nerve injury and lesions to the carotid artery. 7,8 It is therefore of great importance to carefully consider whether HNPGL should be treated, and a 'wait and scan' policy is often the best option.…”

Section: Introductionmentioning

confidence: 99%

No evidence for increased mortality in SDHD variant carriers compared with the general population

et al. 2015

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Germline variants in subunit D of the succinate dehydrogenase gene (SDHD variants) are associated with an increased risk of developing paragangliomas. The aim of this study was to compare mortality rates and survival in a Dutch cohort of SDHD variant carriers with those in the general population. The study was conducted at the Leiden University Medical Center, a tertiary referral center for patients with paragangliomas. Included subjects all tested positive for SDHD variants before 1 July 2012 and visited the departments of Otorhinolaryngology or Endocrinology at least once or had a diagnosed paraganglioma and a SDHD variant-positive family history. Clinical data were retrieved from medical records, information on mortality was obtained from the Municipal Personal Records Database, and mortality rates for the Dutch population were obtained from the Dutch Central Bureau of Statistics, stratified by sex, age and date. SDHD variant carriers were followed from the date of first SDHD variant-related contact until death, emigration or 12 December 2012 and the standardized mortality ratio (SMR) was calculated. Two-hundred and seventy-five SDHD variant carriers were included in the study, of which 80% carried the c.274G4T, p. (Asp92Tyr) variant, had a mean duration of follow-up of 7.6 years, yielding 2242 person-years of observation for analysis. There were 18 deaths in the SDHD variant carrier group; two were paraganglioma related. The SMR for the whole cohort was 1.07 (95% confidence interval 0.67-1.73). In conclusion, mortality in SDHD variant carriers is not substantially increased. Additional studies are required to confirm these findings.

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“…При больших опухолях (IV тип) данное вмешательство сопряжено с высоким риском возникновения эмболических осложнений [12,14,19,20,22,25,28]. Ряд авторов [13,17,29,31,32,34,35] считают, что после эмболизации происходит асептическое воспаление адвентиции, в результате чего во время операции невозможно субадвентици-ально выделить артерии от опухоли и сохранить целостность артерии, поэтому они отказываются от проведения эмболиза-ции. В связи с этим в приведенных наблюдениях мы не выпол-няли предварительную эмболизацию сосудов, питающих опу-холь.…”

Section: Discussionunclassified

“…Параганглионарная система широко представлена в различных органах и тканях человека в виде рас-сеянных и собранных в клубочки клеток, связанных с ганглиями [1,2,5,6,16,18,30]. Термин параганглиома был впервые исполь-зован Kohn в начале ХХ века и является наиболее подходящим наименованием с точки зрения эмбриологии [1,2,18,23,27,30,31,35].…”

Section: кардиология и сердечно-сосудистая хирургия 1 2015unclassified