Pheochromocytoma is a rare neuroendocrine tumor of childhood. We present a 14-year-old boy with bilateral pheochromocytoma, post nephrectomy in view of a non-functioning kidney presenting with severe hypertension and end organ damage. Diagnosis was confirmed with 24-hour urinary VMA, catechol amines, and CT scan. Preoperative blood pressure (BP) was controlled with prazosin, propranolol, nicardipine, and HCT-spironolactone. Anesthesia was given with general endotracheal anesthesia with epidural analgesia. Intraoperative BP rise was managed with infusion of NTG, MgSO4, esmolol, and dexmedetomidine which was especially challenging on account of bilateral tumor.
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http://dx.doi.org/10.4103/1658-354X.114051 | DOI Listing |
JCEM Case Rep
January 2025
Division of Diabetology and Metabolism, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Shinjuku-ku, Tokyo 162-8666, Japan.
A 37-year-old man presented with symptoms of polyuria and weight loss over the past year. Initial laboratory examination showed elevated blood glucose level (468 mg/dL [25.9 mmol/L]; normal reference range [RR], 75-109 mg/dL [4.
View Article and Find Full Text PDFCurr 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.
BMC Anesthesiol
December 2024
Department of Anaesthesiology, The Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, Zhejiang, 322000, China.
Background: Pheochromocytomas (PCCs) are rare neuroendocrine catecholamine (CA)-secreting tumours that originate from chromaffin tissue and can produce and store CAs. Unexpected PCCs pose a serious threat to the perioperative safety of patients and a considerable challenge to anaesthesiologists because of the risks of fatal hypertensive crises and other stresses.
Case Presentation: A 37-year-old woman who was scheduled for tonsillectomy and palatopharyngoplasty under general anaesthesia experienced a malignant cardiovascular event after induction, which was characterized mainly by a sharp increase in heart rate and blood pressure, ultimately leading to cardiac arrest and the occurrence of secondary long QT syndrome.
J Cell Mol Med
December 2024
"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours that originate from chromaffin cells and occur in the adrenal medulla and in the sympathetic or parasympathetic ganglia. Nearly 70% of PPGLs result from germline or somatic mutations in a single driver gene. The aim of this study was to characterize the genetic background and clinical characteristics related to genetic profile of patients with PPGLs from Romania.
View Article and Find Full Text PDFJ ASEAN Fed Endocr Soc
December 2024
Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, University of the Philippines Philippine General Hospital (UP-PGH).
Objective: This study aims to describe the epidemiologic profile and determine the clinical outcomes of patients with pheochromocytoma at the University of the Philippines Philippine General Hospital (UP-PGH).
Methodology: We reviewed the medical records of 30 patients with histopathology-proven, clinical, and biochemical diagnosis of pheochromocytoma. Demographic, clinical characteristics, and clinical outcomes were collected for each patient.
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