EYES2022 ESE Young Endocrinologists and Scientists (EYES) 2022 Adrenal and Cardiovascular (12 abstracts)
Recurrent paraganglioma in patient with aneurysmal disease
Ilic S 1 , Popovic B 2 , Ognjanovic S 2 , Gostiljac D 1 , Dimitrijevic Sreckovic V 1 , Marina Lj 5 , Knezevic D 3 , Sladojevic M 4 , Petkovic A 6 & Popovic S 1
1Clinic for endocrinology, diabetes and metabolic diseases, University Clinical Center of Serbia, Department for diabetes complications; 2Clinic for endocrinology, diabetes and metabolic diseases, University Clinical Center of Serbia, Department for neuroendocrine tumours; 3Clinic for Digestive Surgery, University Clinical Center of Serbia, Department for hepato-biliopancreatic surgery; 4Clinic for Vascular and Endovascular Surgery, University Clinical Center of Serbia, Department for vascular surgery; 5Clinic for endocrinology, diabetes and metabolic diseases, University Clinical Center of Serbia, Center for infertility and gender endocrinology; 6Center for radiology and magnetic resonance, University Clinical Center of Serbia, X knife department
Background: Duration of postoperative follow-up in patients with pheochromocytoma/paraganglioma (PPGL) is still not clearly specified. It is estimated that 5-year risk of a new events (metastatic/recurrent disease) after surgery is 27% in young patients, 25% in those with paraganglioma and around 10% in older patients and those with pheochromocytoma. Arterial aneurysms (AA) accompanying PPGL are described as complication/comorbidity that affects prognosis in these patients.
Case Presentation: A 72-year old male patient with five-year history of: hypertensive crises (max 220/130 mmHg), bradycardia to tachycardia (HR 45-120/min), orthostatic hypotension, night sweating and de novo diabetes mellitus, was presented at our Clinic. He had right-side adrenalectomy 18 years before (high risk procedure due to multiple AA), after the same clinical presentation and was diagnosed with composite tumour paraganglioma-ganglioneuroma (PASS 3). In present, catecholamine hypersecretion was confirmed in 24h urine samples - high noradrenaline (6600-12000 nmol/24h) and normetanephrine (11.55-24.40 umol/24h), together with high chromogranine A 974 ng/ml. CT found retroperitoneal interaortocaval tumour 47.9x35.6mm, MIBG scintigraphy was negative, FDG/PET CT showed retroperitoneal mass (SUVmax 35.6) and nodules in mediastinum and left lung (SUVmax 3.9 and 4.8 respectively). Retroperitoneal tumour extirpation was managed, with perioperative complication - left kidney failure, due to a thrombosis of aneurysmatic left renal artery. Pathohistological diagnosis was paraganglioma (PASS 2, Ki67 5.6%). Resolution of all features was observed after surgery, adrenal medulla metabolites were in reference range and lung nodules were CT described as fibrosis. Genetic analyses on MEN2, SDH and VHL are still ongoing.
Conclusions: Current guidelines suggest that patients with PPGL should be followed for at least 10 years after surgery and with extra-adrenal tumour or genetic PPGL should be followed lifelong. Abdominal AA (aorta, renal artery) carry on an additional effect on morbidity and mortality of patients and should be monitored closely and multidisciplinary.
Article tools
My recent searches
My recently viewed abstracts
Authors
Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more