A 64-year-old man presented to our hospital feeling ill with epigastralgia. Computed tomography (CT) showed right suprarenal cystic tumor. High urinary catecholamine level was noted. Based on metaiodobenzylguanidine (MIBG) scintigraphy, magnetic resonance imaging and blood tests, preoperative diagnosis was adrenal pheochromocytoma. En-bloc resection of the tumor and the right kidney was performed. The cyst contained yellowish serous fluid, which had a catecholamine level about 3,000 times that in the blood. The histological diagnosis was cystic pheochromocytoma. Pathogenesis of cystic adrenal tumor is discussed briefly.
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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.
Discov Oncol
December 2024
Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
Introduction: Although giant cystic pheochromocytoma and paraganglioma (PPGL) are uncommon, they can be life-threatening when it occurs. Unfortunately, prior case reports have shown that giant cystic PPGLs are highly susceptible to diagnostic errors. Therefore, this study aimed to explore giant cystic PPGLs by comparing them with non-cystic PPGLs, defining the clinical features of the affected patients, and analyzing the characteristics of misdiagnosis and mistreatment associated with PPGLs.
View Article and Find Full Text PDFCureus
September 2024
General Surgery, Lahore General Hospital, Lahore, PAK.
Radiol Case Rep
December 2024
Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India.
Pheochromocytoma is a rare neuroendocrine tumor arising from adrenal medulla. Patients usually show classic triad of headache, palpitations and diaphoresis along with persistent or paroxysmal hypertension. Majority of the tumors are solid or mixed solid and cystic.
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June 2024
Radiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune (Deemed to be University), Pune, IND.
Von Hippel-Lindau (VHL) syndrome is characterized by a range of tumors including phaeochromocytomas, pancreatic adenomas, cerebellar haemangioblastomas, and renal cell carcinomas. A 50-year-old male presented with a three-week history of headache. Additionally, the patient exhibited signs of hypertension.
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