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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.

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New Developments in VHL-Associated Neuroendocrine Neoplasms.

Curr Oncol Rep

January 2025

Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece.

Purpose Of Review: The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.

Recent Findings: Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option.

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Long-term persistence of glycemic dysregulation in patients with a history of pheochromocytoma/paraganglioma.

J Clin Endocrinol Metab

January 2025

Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH), CH-8091 Zurich, Switzerland.

Context: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.

Objective: To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.

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Epigenomic Alterations of the Human Gene in Adrenal Zonation.

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November 2024

Department of Health Promotion and Medicine of Future, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa 920-8641, Japan.

Article Synopsis
  • * It finds that certain genes are unmethylated in the zona fasciculata (ZF) but heavily methylated in the zona glomerulosa (ZG) and reticularis (ZR), affecting the expression of CYP11B enzymes.
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Mediastinal paragangliomas, though rare, present significant surgical challenges due to their proximity to critical vascular structures within the mediastinum. This case report discusses the management of a patient with an incidentally discovered non-functional mediastinal paraganglioma. The tumor's location necessitated meticulous preoperative planning and intraoperative navigation to prevent vascular injury.

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