Paragangliomas, tumors derived from the extra-adrenal paraganglion system, have commonly been found in the retroperitoneum, abdomen, mediastinum, skull base, and neck. Rare intraspinal cases have included involvement of the cauda equina and filum terminale, and a recent case has been reported of thoracic cord paraganglioma with metastasis to the cauda equina. The authors present the case of a patient with a cervical paraganglioma who underwent subtotal resection followed by postoperative irradiation; she subsequently developed multiple intracranial and intraspinal metastases 7 months following the first decompression procedure.
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http://dx.doi.org/10.3171/jns.1991.75.2.0320 | DOI Listing |
J Neurol Surg B Skull Base
February 2025
Department of Otorhinolaryngology, Leiden University Medical Centre, Leiden, The Netherlands.
SDHx variant carriers have an increased risk of developing head and neck paraganglioma. The Dutch guidelines state that these patients require lifelong follow-up, but no clear recommendation is made about the frequency of screening. To determine the annual risk of developing head and neck paraganglioma in SDHx variant carriers after a negative initial screening.
View Article and Find Full Text PDFPheochromocytoma (PHEO) currently is considered to be malignant due to metastatic potential. One of the most common familial forms of PHEO is multiple endocrine neoplasia syndrome (MEN) type 2. The penetrance of PHEO in MEN2 syndrome is up to 50% of cases.
View Article and Find Full Text PDFEndocrine
January 2025
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, Florence, Italy.
Purpose: To compare functional deficits associated to surgery with those caused by the growth of the head and neck paragangliomas (HNPGLs).
Methods: 72 patients with HNPGLs were included. Patients were divided in group A (49 patients undergoing surgery) and group B (23 patients following a wait and see approach).
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.
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