Phaeochromocytoma and Paraganglioma Excision Involving the Great Vessels
| Autor: | S. Bhattacharya, S.J. Edmondson, Umasuthan Srirangalingam, Robert Carpenter, Kirun Gunganah, W. M. Drake |
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| Rok vydání: | 2017 |
| Předmět: |
lcsh:Diseases of the circulatory (Cardiovascular) system
medicine.medical_specialty lcsh:Surgery Case Report 030204 cardiovascular system & hematology Inferior vena cava law.invention Paraganglioma 03 medical and health sciences chemistry.chemical_compound Vena cava 0302 clinical medicine law medicine.artery medicine Genetic predisposition Cardiopulmonary bypass Metanephrine Aorta Pelvis business.industry Phaeochromocytoma lcsh:RD1-811 medicine.disease Surgery medicine.anatomical_structure medicine.vein Great vessels chemistry lcsh:RC666-701 030220 oncology & carcinogenesis cardiovascular system Radiology Cardiology and Cardiovascular Medicine business |
| Zdroj: | EJVES Short Reports, Vol 35, Iss, Pp 1-6 (2017) EJVES Short Reports |
| ISSN: | 2405-6553 |
| DOI: | 10.1016/j.ejvssr.2017.02.002 |
| Popis: | Objective/background Phaeochromocytomas and paragangliomas are vascular neuroendocrine tumours distributed between the neck and the pelvis and may be associated with catecholamine secretion. The aim of the study was to describe the complex surgical management required to excise these tumours when in close proximity to the great vessels (aorta and vena cava). Methods This was a retrospective case series. Patients included those undergoing surgical excision of a phaeochromocytoma or paraganglioma involving the great vessels. Data on clinical presentation; genetic mutations; tumour location; catecholamine/metanephrine secretion; surgical strategy; pre-, intra-, and post-operative course were collated. Results Five patients (age range 16–60 years) were identified; three had thoracic paragangliomas located under the arch of the aorta, one had an abdominal paraganglioma invading the aorta, and one had a massive phaeochromocytoma invading the inferior vena cava via the adrenal vein. Three patients had predisposing germline mutations. All patients had adrenergic blockade prior to surgery. A diverse range of complex surgical techniques were employed to excise tumours, including cardiopulmonary bypass, aortic resection, grafting and venotomy of the vena cava. Early post-operative complications were limited. Conclusions Excision of phaeochromocytomas and paragangliomas involving the great vessels is high risk surgery optimally undertaken within a multidisciplinary setting in a tertiary referral centre. Comprehensive radiological and biochemical assessment, meticulous pre-operative preparation and close intra- and post-operative monitoring are essential. Radiological imaging may be unable to resolve the tumour extent and anatomy pre-operatively and direct visualisation of the tumour may be the only way to clarify the surgical strategy. Pre-operative knowledge of the genetic predisposition may influence surgical management. Highlights • Highly vascular tumours which may be associated with catecholamine secretion. • Comprehensive pre- and intra-operative preparation is essential. • Final surgical strategy often requires direct tumour visualisation. • The surgical strategy may alter for germline associated tumours. • Management should be delivered by experienced multidisciplinary teams. |
| Databáze: | OpenAIRE |
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