Digestive Tract Neuroendocrine Tumours and Pheochromocytomas/Paragangliomas
the minimum follow-up requirement. In the case of persistence or recurrence, localisation studies should be sought.
Sixty per cent of malignant pheochromocytoma sites show avid 131I-MIBG uptake.103
In specialised centres such tumours can be treated
with therapeutic 131I-MIBG in single or fractionated doses totaling 200– 1,400mCi. Approximately 30% of tumours show an objective response to therapy (40% biochemical response) and 40% of tumours remain stable (20% biochemically).108
Radioiodine therapy may lead to serious adverse events, particularly in terms of bone marrow suppression.109–111
Less experience has been obtained with labelled somatostatin analogues.81
Overall, combination chemotherapy with dacarbazine cyclophosphamide and vincristine does not confer any significant survival benefit.112
etoposide plus lomustine with 5-fluorouracil.108 ablation of metastatic foci can also be used.113
Another experimental modality is sunitinib, a multiple tyrosine kinase inhibitor.114 Prognosis
and overall 16% of patients operated on for pheochromocytoma/paraganglioma have recurrent disease within 10 years.115
In a large cohort of
The life expectancy of patients with benign pheochromocytoma/ paraganglioma that has been successfully excised may not be different from that of the general population. Nevertheless, half of the patients that are successfully operated on have continued hypertension63,103
In a recent case series of children with pheochromocytoma/ paraganglioma, all were found to harbour a predisposing mutation but were alive after follow-up of five years.116
patients with pheochromocytoma, however, mortality from a second neoplasia was four-fold higher compared with that of the general population.117
Recently levels of the adrenomedullin RDC1 receptor were reported to be four times higher in malignant than in benign pheochromocytomas. Cells expressing SNAIL were frequent in metastatic pheochromocytomas compared to being absent in tumours without metastases. Despite these reports, the use of adrenomedullin RDC1 receptor and SNAIL levels as prognostic factors in pheochromocytoma/paraganglioma is not yet envisaged.71,118
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New modalities for the medical management of malignant pheochromocytomas are being assessed. Among these, the combination of temozoline and thalidomide or therapeutic somatostatin analogues have shown some encouraging results.108
n
Ioannis Ilias is an Endocrinologist at the E Venizelou Hospital in Athens. He was an Assistant Professor of Clinical Pharmacology at the University of Patras in Greece, and a Fellow at the US National Institutes of Health. He has collaborated with Dr Pacak on research focused on the diagnostic localisation of pheochromocytomas/paragangliomas. Dr Ilias has co-authored 111 articles in peer-reviewed journals.
Karel Pacak is Senior Investigator and Chief of the Section on Medical Neuroendocrinology at The Eunice Kennedy Shriver National Institute of Child Health and Human Development at the US National Institutes of Health (NIH). He is a Professor at the First Faculty of Medicine at Charles University and an Adjunct Professor at Georgetown University. He established the International Symposia on Pheochromocytoma, has written 244 original articles, 76 book chapters and five
books. He received his MD and PhD from Charles University, Prague. Professor Pacak completed his residency at the Washington Hospital Center and specialised in endocrinology, diabetes and metabolism at the NIH.
Other regimens include etoposide plus cisplatin or Radiofrequency
Pheochromocytoma-paraganglioma syndrome can be diagnosed reliably by immunohistochemistry.73
There are no criteria to predict survival following malignant pheochromocytoma. Surprisingly, however, 15-year survival rates of almost 50% have been reported with a combination of therapeutic modalities.119
Modalities Currently being Evaluated/Introduced into Practice
Quantification of pheochromocytoma/paraganglioma vesicular monoamine transporter content indicates whether the tumours are MIBG-avid.95,96,120
As PET imaging accessibility increases worldwide, more patients with pheochromocytoma/paraganglioma will be evaluated with PET studies. Ultratrace 131I-MIBG has very high specific activity and holds promise for future therapeutic applications.48
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17. Zuetenhorst JM, Taal BG, Oncologist, 2005;10:123–31. 18. Gabriel M, Decristoforo C, Kendler D, et al., J Nucl Med, 2007;48:508–18.
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20. Prasad V, Ambrosini V, Hommann M, et al., Eur J Nucl Med Mol Imaging, 2010;37(1):67–77.
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