Horm Metab Res 2012; 44(05): 373-378
DOI: 10.1055/s-0032-1306275
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© Georg Thieme Verlag KG Stuttgart · New York

Perioperative Management of Pheochromocytoma/Paraganglioma: Is There a State of the Art?

M. Mannelli
1   Department of Clinical Pathophysiology, University of Florence, Florence, Italy
,
H. Dralle
2   Department of General, Visceral and Vascular Surgery, University of Halle-Wittemberg, Halle/Saale, Germany
,
J.W. M. Lenders
3   Department of Medicine, Division of General Internal Medicine, Radboud Adrenal Centre, University Nijmegen Medical Centre, Nijmegen, The Netherlands
4   Department of Medicine III, Carl Gustav Carus University Medical Centre, Dresden, Germany
› Author Affiliations
Further Information

Publication History

received 14 November 2011

accepted 08 February 2012

Publication Date:
19 April 2012 (online)

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Abstract

Pheochromocytoma and paraganglioma are rare tumors of sympathetic or parasympathetic origin, presenting with a highly variable clinical picture. Rarity, as well as biological, clinical, and genetic heterogeneity are barriers to initiate prospective studies that help to establish clinical guidelines. The best management of these patients relies on the experience of a multidisciplinary team. The ultimate outcome can benefit from adequate pre-surgical evaluation and treatment as well as an accurate post-surgical follow-up. Long-term follow-up is mandatory in all patients, but is particularly important in specific familial cases such as those with an SDHB mutation where the risks of recurrence are higher. The surgical approach varies depending on tumor size, location, and surgeon’s personal attitude and experience. In this paper, we summarize recommendations, based mostly on authors’ and other experts’ personal experiences, for the best possible management of patients prior, during and after surgery, as well as when pheochromocytoma is diagnosed during pregnancy.