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Pheochromocytoma (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 PDFSynchronous transperitoneal robotic-assisted bilateral cortical-sparing adrenalectomy for pheochromocytomas in a patient with multiple endocrine neoplasia type 2a (MEN2A) syndrome: a case report.
Gland Surg
December 2024
Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Background: Pheochromocytoma is a rare neuroendocrine tumor, and bilateral pheochromocytomas is even less common. Due to the limited experience with such cases, this study aims to explore the optimal surgical strategy, assess the potential advantages of robotic surgery, and evaluate surgical outcomes for managing bilateral pheochromocytomas.
Case Description: This report presented a case of a 33-year-old woman with bilateral pheochromocytomas related to multiple endocrine neoplasia type 2a (MEN2A), who was successfully managed by synchronous transperitoneal robotic-assisted bilateral cortical-sparing adrenalectomy.
Remission of Insulin-Dependent Diabetes Mellitus in Multiple Endocrine Neoplasia Type 2A After Adrenalectomy.
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 PDFNew 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.
Unexpected pheochromocytoma leading to cardiac arrest during the perioperative period: a case report and literature review.
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.
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