Introduction: Pheochromocytoma is a tumour arising from the adrenal medulla, which secretes catecholamines. Approximately 20% of pheochromocytomas are cystic and more likely to be asymptomatic. They should be surgically resected as all have a malignant potential and pose cardiovascular risk. We report the case of a 61-year-old female patient admitted electively for laparoscopic adrenalectomy for a large cystic pheochromocytoma detected incidentally. Diagnosis was confirmed preoperatively by elevated 24-h urinary metanephrines. The patient was treated preoperatively with alpha and beta blockade. Surgery was without complication; she had an uneventful postoperative recovery and no evidence of recurrence at one-year follow-up.
Conclusion: This case highlights the necessity of investigating for biochemical function in all adrenal lesions by measuring metanephrines, even when entirely cystic on imaging. Given the surgical and anaesthetic risk in resection of pheochromocytoma, attaining a preoperative diagnosis allows for careful preoperative planning and safe surgery.
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http://dx.doi.org/10.1177/0036933019900339 | DOI Listing |
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.
View Article and Find Full Text PDFCureus
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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