Phaeochromocytoma and Paraganglioma Excision Involving the Great Vessels

Abstract

Objective/backgroundPhaeochromocytomas 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).MethodsThis 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.ResultsFive 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.ConclusionsExcision 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.

Successful Resection of a Non-functional Paraganglioma with Celiac Trunk Invasion Followed by Common Hepatic Artery Reimplantation – A Case Report and Literature Review

“…In all cases the tumors were resected en bloc with the vascular segment which had been invaded, the vascular continuity being reestablished by aorta transection and cardiopulmonary bypass (in three cases presenting intrathoracic lesions), aorta resection and Dacron graft (in the case presenting abdominal aorta invasion due to an abdominal paraganglioma) and respectively by inferior vena cava patch wall resection en bloc with nephrectomy and adrenalectomy in one case; however in this case remnant tumoral tissue was seen in the adrenal bed. The postoperative course was uneventful in all cases; when it comes to the long-term outcomes, all the four cases in which a complete resection was achieved no recurrent lesions were encountered after a follow-up period ranging between one and eight years (1).…”

“…Paragangliomas are well vascularized tumors with a sympathetic or parasympathetic origin that might develop in any location between the skull base and pelvis with an estimated rare of malignancy of 17% (1,2). They usually arise from specialized neural crest cells, which are usually encountered in the medullary part of the adrenal glands; however, in up to 10% of cases an extra-adrenal location is reported, the most common sites being the para-aortic and peri-nephric spaces (3)(4)(5).…”

Paediatric Abdominal Oncovascular Surgery: a Single-centre Experience and Review of the Literature

“…For more common tumours like WT and HBL, protocols are available and they dictate neoadjuvant NACT 24,30 . On the contrary, Paragangliomas have typically a poor response to NACT and therefore primary en-bloc resection of mass and vessels must be considered 31,32,33,21 After resection of the involved vessel, a decision needs to be made regards simple ligation, or reconstruction. 34 This will depend on whether an artery or vein is involved, and what it supplies: most arteries and the portal vein will require reconstruction, whilst the IVC can be ligated with impunity.…”

“…The IVC may also be involved by other retroperitoneum tumours such as teratomas and paragangliomas. 20,21 Pheochromocytomas and Paragangliomas can encase the IVC, occasionally requiring aggressive resection: cavectomy for invasive paragangliomas has been described in both adults and children. 16,22,21 Retroperitoneal sarcomas may also involve the IVC and cavectomy for such tumours is also reported.…”

“…20,21 Pheochromocytomas and Paragangliomas can encase the IVC, occasionally requiring aggressive resection: cavectomy for invasive paragangliomas has been described in both adults and children. 16,22,21 Retroperitoneal sarcomas may also involve the IVC and cavectomy for such tumours is also reported. 5 The IVC may be involved in Hepatoblastomas as well 23 .…”