ECE2023 Eposter Presentations Adrenal and Cardiovascular Endocrinology (124 abstracts)
Pheochromocytoma associated with von Hippel-lindau disease
Sevgul Faki 1 , Unzile Arifoglu 2 , Bilge Dibeklioğlu 3 , Altuğ Tuncel 4 , Müge Keskin 3 , Abbas Ali Tam 2 , Oya Topaloglu 2 , Reyhan Ersoy 2 & Bekir Cakir 2
1Ankara City Hospital, Departments of Endocrinology and Metabolism, Ankara, Turkey; 2Ankara Yıldırım Beyazıt University, Faculty of Medicine, Departments of Endocrinology and Metabolism, Ankrara, Turkey; 3Ankara City Hospital, Departments of Endocrinology and Metabolism, Ankara, Turkey; 4Ankara City Hospital, Department of Urology, Ankara, Turkey
Von Hippel-Lindau (VHL) syndrome is a pathological condition that causes various clinical symptoms and is difficult to diagnose. The most common pathological lesions are hemangioblastomas of the central nervous system, retinal angiomas, renal clear cell carcinomas, and pheochromocytomas. Here we reposrt a case of likely to be VHL due to his family history.
Case: A 33-year-old male suffering from hypertension and a history of hemangioblastoma operated in 2019. The patient had an endoscopic examination after developing nausea, vomiting and weight loss. His endoscopic biopsies include grade 2 neuroendocrine tumors NET). the patient refer to endocrinologist. The father of the patient died at age of 40 due to brain tumor and his brather was operated due to retinal and cerebral hemangioblastoma (in 2013) since that date the familyhad been followed up in the department of neurosurgery with suspicion of VHL. His plasma free metanephrines and urinary metanephrines was high and a computer tomography scan, MIBG and MRI scan of the abdomen showed a solid mass in the lower pole of the lenf kidney at 2.5×2 cm, pancreatic cysts and right adrenal mass at 36×40 laparascopic adrenalectomy and parsiel nefrectomy was performed. The Pathological examination revealed renal cell carcinoma and pheochromocytomas with a low PASS score. The contro plasma free metanephrines and urinary metanephrines were withen normal range. Conclution Since pheochromocytomas can have low activity, the classical symptoms may be missing. The absence of symptoms can make it difficult to diagnose pheochromocytoma and Even if we couldn’t perform the genetic examination there is a strong association between pheochromocytoma and VHL syndrome, and pheochromocytoma is an important feature in the clinical classification of VHL syndrome. The family history of retinal or central nervous system hemangioblastoma (Hb) exists, only one Hb or visceral lesion (renal tumours, pancreatic cysts or tumours, pheochromocytoma, papillary cystadenomas of the epididymis) is required to make the diagnosis of VHL and Due to the risk of pheochromocytoma, the radyologıc sxanning and biochemical tests shoud performed.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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