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Extra-adrenal Pheochromocytoma of the Urinary Bladder in an Adolescent Female Patient.
Cureus
December 2024
Pediatric Radiology, Detroit Medical Center, Wayne State University, Detroit, USA.
Pheochromocytoma is a catecholamine-secreting tumor that arises from the medullary chromaffin cells but can rarely be extra-adrenal in origin. We present a case of a 16-year-old female patient with uncontrolled hypertension, despite being on lisinopril and metoprolol, and associated left-sided chest pain, recurrent headaches, and an unintentional weight loss of 10 pounds in one month. Laboratory work-up showed a markedly elevated plasma metanephrine level of 4463.
View Article and Find Full Text PDFHead and Neck Paraganglioma in Pacak-Zhuang Syndrome.
JNCI 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 PDFRole of neck dissections in the management of carotid body tumors.
Laryngoscope 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 PDFA rare case of bladder paraganglioma treated successfully with robotic partial cystectomy.
Endocr Oncol
January 2025
Department of Diabetes and Endocrinology, University College London Hospital NHS Foundation Trust, London, UK.
Unlabelled: Bladder paragangliomas are rare extra-adrenal urological tumors that account for around 0.05% of bladder cancers. Their diagnosis is often delayed because of the rarity of these tumors.
View Article and Find Full Text PDFSpinal impostor: Metastatic cervical paraganglioma presenting with paraparesis, a case report.
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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