Excision of phaeochromocytoma and paraganglioma involving the great vessels

U Srirangalingam; K Gunganah; L Parvanta; K Metcalfe; G M Besser; A Sahdev; H S Flora; S Bhattacharya; R Carpenter; S Akker; S J Edmondson; W Drake

doi:10.1530/endoabs.37.EP1149

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Endocrine Abstracts (2015) 37 EP1149 | DOI: 10.1530/endoabs.37.EP1149

ECE2015 Eposter Presentations Endocrine tumours (69 abstracts)

Excision of phaeochromocytoma and paraganglioma involving the great vessels

U Srirangalingam ² , K Gunganah ¹ , L Parvanta ¹ , K Metcalfe ¹ , G M Besser ¹ , A Sahdev ¹ , H S Flora ¹ , S Bhattacharya ¹ , R Carpenter ¹ , S Akker ¹ , S J Edmondson ¹ & W Drake ¹

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¹St Bartholomew’s Hospital, London, UK; ²Royal Free Hospital, London, UK.

Objective: The aim of the study was to describe the surgical management of phaeochromocytomas and paragangliomas which lie in close proximity to or involve the great vessels including the aorta and vena cava.

Design: Retrospective case-series. Patients: five subjects undergoing surgical excision of either a phaeochromocytoma or paraganglioma involving the great vessels seen at St Bartholomew’s Hospital, UK (2004–2013).

Measurements: Clinical presentation, genetic mutations, tumour location, catecholamine secretion, pre-, intra- and post-operative course.

Results: Of the five subjects (age range 16–60 years), three had thoracic paragangliomas located under the arch of the aorta, one had an abdominal paraganglioma in which preoperative imaging was unable to delineate that the tumour was invading the aorta and one had a massive phaeochromocytoma invading the IVC. Three of the four subjects tested had predisposing germline mutations. All subjects had alpha and β adrenergic blockade prior to surgery. The thoracic paragangliomas were excised following cardiopulmonary bypass and aortic transection to access the tumours. The abdominal paraganglioma invading the aorta was resected with part of the aorta to clear disease and required insertion of an aortic Dacron graft. The phaeochromocytoma invading the IVC was resected en bloc with the right kidney with a venotomy to extract to resect the tumour nodule invading the IVC. Only one subject experienced an early post-operative complication, which was managed conservatively, and all subjects made a good recovery from surgery.

Conclusion: Excision of phaeochromocytoma and paraganglioma involving the great vessels is high-risk surgery and should be undertaken in a tertiary referral centre within a multidisciplinary setting. Subjects require comprehensive radiological and biochemical assessment. Meticulous pre-operative preparation and appropriate intra- and post-operative back-up are essential. In some cases radiological imaging is unable to resolve the tumour anatomy and extent 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.