Extra-adrenal paragangliomata are uncommon entities. They can be classified into four basic groups according to their anatomical sites, i.e. branchiomeric, intravagal, aorticosympathetic and visceral autonomic. Similar tumours may arise in sites away from the usual distribution of the sympathetic and parasympathetic ganglia, e.g. orbit, nose, small intestine and even in the pancreas. We report three instructive cases of extra-adrenal paraganglioma which were found in unusual sites such as urinary bladder, thyroid gland and on the wall of the inferior vena cava.
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Int J Surg Case Rep
January 2025
Department of Thoracic surgery, National University Hospital, Damascus University, Damascus, Syria.
Introduction: Mediastinal paragangliomas are rare neoplasms arising from extra-adrenal neural crest cells, presenting as either functional or nonfunctional tumors. Clinical manifestations range from catecholamine-related symptoms to physical compression effects. Accurate recognition of these tumors is crucial for diagnosis and management due to their rarity and association with vital mediastinal structures.
View Article and Find Full Text PDFJ Nippon Med Sch
January 2025
Department of Surgery, Nippon Medical School.
Paraganglioma, a catecholamine-producing tumor originating in extra-adrenal paraganglion cells, is rare in children. Although diagnosis of paraganglioma is based on the presence of catecholamine symptoms, some patients lack such symptoms. Delayed diagnosis is associated with tumor growth and invasion of surrounding vessels.
View Article and Find Full Text PDFJ Cardiothorac Surg
January 2025
Department of Thoracic Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, P. R. China.
Background: Paragangliomas are rare neoplasms arising from extra-adrenal chromaffin cells, with mediastinal paragangliomas representing an exceptionally rare subset. This report details the surgical management of a complex mediastinal paraganglioma case, presenting with refractory hypertension and invasion of critical surrounding structures. A comprehensive review of the current literature is included to underscore existing cases, enhance clinical awareness, and share our insights and experience in the diagnosis and treatment of this challenging condition.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
One Health Research Group, Universidad de las Americas, Quito, Ecuador.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.
View Article and Find Full Text PDFPak J Med Sci
December 2024
Asif Shabbir Associate Professor, Department of Neurosurgery, Neurosurgery Unit-I, Punjab Institutes of Neurosciences Lahore, Pakistan.
Paragangliomas are slow-growing, extra-adrenal neuroendocrine tumors with rare intracranial presentation. Although benign, they can be locally aggressive tumors causing bone destruction and compression related symptoms. We report the case of a 19 years old, normotensive female who presented with headache and vertigo for the past six months.
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