Neck paragangliomas are orphan diseases with incidence 1:30 000-1:100 000. Life expectancy is poor in patients with distant metastasis (5-year overall survival 11.8%), whereas 5-year overall survival in patients with regional metastasis is 76.8-82.4%. Meanwhile, there is still no any reliable tool for prediction of malignant potential of paraganglioma. Above-mentioned data indicate an importance of early diagnosis and timely treatment of neck paragangliomas. Total resection of tumor in ablastic conditions is a gold standard of treatment. However, surgery is associated with a high risk of neurovascular complications and requires multidisciplinary approach. Nevertheless, new knowledge dedicated to different aspects of pathogenesis of neck paraganglioma, diagnosis and treatment arise every year. This review is devoted to modern data on neck paragangliomas.
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http://dx.doi.org/10.17116/hirurgia202207164 | DOI Listing |
Clin Case Rep
January 2025
Ear, Nose and Throat Centre, Xinjiang Uygur Autonomous Region People's Hospital Urumqi Xinjiang Uygur Autonomous Region China.
This report describes a rare case of a paraganglioma occurring beneath the vocal folds. During the preoperative biopsy, we encountered significant hemorrhage, forcing us to stop the procedure and preventing us from obtaining a definitive diagnosis. Despite these challenges, the eventual surgery had a good outcome.
View Article and Find Full Text PDFJNCI Cancer Spectr
January 2025
Section on Medical Neuroendocrinology National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, 20892, MD, USA.
Head and neck paragangliomas (HNPGLs) are typically slow-growing, hormonally inactive tumors of parasympathetic paraganglia. Inactivation of prolyl-hydroxylase domain-containing 2 protein causing indirect gain-of-function of hypoxia-inducible factor-2α (HIF-2α), encoded by EPAS1, was recently shown to cause carotid body hyperplasia. We previously described a syndrome with multiple sympathetic paragangliomas caused by direct gain-of-function variants in EPAS1 (Pacak-Zhuang syndrome, PZS) and developed a corresponding mouse model.
View Article and Find Full Text PDFLaryngoscope Investig Otolaryngol
February 2025
Division of Otolaryngology - Head and Neck Surgery, Department of Surgery Dalhousie University Halifax Nova Scotia Canada.
Objective: Carotid body tumors (CBTs) are rare neoplasms of the paraganglia at the carotid bifurcation. While typically benign, CBTs occasionally exhibit malignancy, metastasizing to nearby lymph nodes. Histopathologic analysis alone is insufficient to confirm malignancy, requiring metastases to non-neuroendocrine tissue for a definitive diagnosis.
View Article and Find Full Text PDFEur J Endocrinol
January 2025
Department of Internal Medicine IV, LMU University Hospital, LMU Munich, 80336 Munich, Germany.
Objective: The effects of sex hormones remain largely unexplored in pheochromocytomas and paragangliomas (PPGLs) and gastroenteropancreatic neuroendocrine tumors (GEP-NETs).
Methods: We evaluated the effects of estradiol, progesterone, Dehydroepiandrosterone sulfate (DHEAS), and testosterone on human patient-derived PPGL/GEP-NET primary culture cell viability (n = 38/n = 12), performed next-generation sequencing and immunohistochemical hormone receptor analysis in patient-derived PPGL tumor tissues (n = 36).
Results: In PPGLs, estradiol and progesterone (1 µm) demonstrated overall significant antitumor effects with the strongest efficacy in PPGLs with NF1 (cluster 2) pathogenic variants.
Int J Surg Case Rep
January 2025
Neurosurgery Section, Department of Surgery, The Aga Khan Hospital, P. O Box 2289, Dar Es Salaam, Tanzania.
Introduction And Importance: Paragangliomas are rare neuroendocrine tumors, typically arising from extra-adrenal chromaffin cells. Primary intra-spinal paragangliomas are uncommon, and metastatic spinal paragangliomas without paraneoplastic symptoms are even rarer. This case highlights the diagnostic challenges posed by such rare tumors.
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