Is Routine Genetic Testing Warranted in Head and Neck Paragangliomas?

All individuals presenting with an extratympanic paraganglioma of the head and neck should undergo genetic testing.
Source: ENTtoday - TRIO Best Practices - Category: ENT & OMF Authors: Tags: Head and Neck Practice Focus TRIO Best Practices clinical best practices genetic testing head and neck cancer paragangliomas Source Type: research

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This article describes our institutional experience with head and neck paragangliomas, with an emphasis on evaluating clinical outcomes among malignant head and neck paragangliomas. All of these patients with malignant head and neck paragangliomas were considered surgical candidates without known metastatic disease at presentation. Furthermore, all patients were presented at our institutional head and neck treatment planning conference where surgical oncologists, medical oncologist s, and radiation oncologists recommend the best treatment option that is catered for each patient. Surgical resection was only offered when the...
Source: JAMA Otolaryngology - Head and Neck Surgery - Category: ENT & OMF Source Type: research
To the Editor As academic physicians with an interest in pheochromocytoma/paraganglioma, we read with great interest the article by McCrary et al characterizing malignant head and neck paragangliomas (HNPGLs). The authors used the paraganglioma cohort from an academic tertiary cancer center diagnosed between 1963 and 2018. They found that the prevalence of malignant HNPGLs was 6 of 70 (9%), with 5 of 6 patients carrying the succinate dehydrogenase subunit B (SDHB) mutation. They suggested that patients with paragangliomas should undergo genetic testing, and owing to the difficulty in diagnosing malignant HNPGL prior to sur...
Source: JAMA Otolaryngology - Head and Neck Surgery - Category: ENT & OMF Source Type: research
Renato Mariani-Costantini In this review, we propose that paraganglioma is a fundamentally organized, albeit aberrant, tissue composed of neoplastic vascular and neural cell types that share a common origin from a multipotent mesenchymal-like stem/progenitor cell. This view is consistent with the pseudohypoxic footprint implicated in the molecular pathogenesis of the disease, is in harmony with the neural crest origin of the paraganglia, and is strongly supported by the physiological model of carotid body hyperplasia. Our immunomorphological and molecular studies of head and neck paragangliomas demonstrate in all ca...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
acute; R, Faggiano A, Schlumberger M, Borson-Chazot F, Mannelli M, Gimenez-Roqueplo AP, Caron P, Timmers HJLM, Fassnacht M, Robledo M, Borget I, Baudin E, European Network for the Study of Adrenal Tumors (ENS@T) Abstract Background: Malignant pheochromocytoma and paraganglioma (MPP) are characterized by prognostic heterogeneity. Our objective was to look for prognostic parameters of overall survival in MPP patients. Patients and Methods: Retrospective multicentric study of MPP characterized by a neck-thoraco-abdomino-pelvic CT or MRI at the time of malignancy diagnosis in European centers between 1998 and 201...
Source: Clinical Lung Cancer - Category: Cancer & Oncology Authors: Tags: J Clin Endocrinol Metab Source Type: research
Bajcsay A, Polgár C Abstract The treatment of squamous cell carcinoma of the head and neck is multimodal, including surgery, chemotherapy, and radiotherapy, or the combination of those. Though aggressive treatment results in complete tumor remission in many patients even in locally advanced stages, unfortunately local relapse is not uncommon. For patients not candidate for salvage surgery, chemotherapy and conventional fractionated external beam irradiation can be applied. However, for patients previously treated with full-dose radiotherapy, the deliverable reirradiation dose is limited, considering the el...
Source: Magyar Onkologia - Category: Cancer & Oncology Authors: Tags: Magy Onkol Source Type: research
CONCLUSION: Proton beam therapy is an effective and well-tolerated treatment modality of skull base paragangliomas, with documented functional benefit. A longer follow-up is planned in order to assess local control and long-term toxicities. PMID: 29269165 [PubMed - as supplied by publisher]
Source: Cancer Radiotherapie - Category: Cancer & Oncology Authors: Tags: Cancer Radiother Source Type: research
Is first-line treatment for carotid body tumors the same as that for jugular and vagal paragangliomas?Cancer Control: Journal of the Moffitt Cancer Center
Source: Medscape Today Headlines - Category: Consumer Health News Tags: Hematology-Oncology Journal Article Source Type: news
AbstractThe fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as “Tumours of the adrenal cortex” and “Tumours of the adrenal medulla and extra-adrenal paraganglia.” Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma) , haematological tumours, and secondary tumours. Amongst them, schwannoma and haematological tumou...
Source: Endocrine Pathology - Category: Pathology Source Type: research
ConclusionsOur results suggested that epigenetic inactivation on multiple TSGs may serve as a key mechanism for the progressive behaviors of SDH-mutated HNPGLs. Thus, an interplay between genetic status, epigenetic alterations, and clinical features might be established in the disease.
Source: Journal of Cancer Research and Clinical Oncology - Category: Cancer & Oncology Source Type: research
Thyroid nodule is extremely common in clinical practice. An evaluation of thyroid nodule is often straightforward. However, pathologic findings can occasionally be surprising. A 42-year-old previously healthy man referred to endocrinology clinic with asymptomatic neck enlargement noted during evaluation of acid reflux symptom. He had no previous radiation exposure to head and neck. He did not take medication on regular basis, denied smoking cigarette or drinking alcohol in excess. Family history was unremarkable for thyroid disease or cancer.
Source: The American Journal of Medicine - Category: Journals (General) Authors: Tags: Diagnostic Dilemma Source Type: research
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