Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

Kim Francis Andersen; Rahim Altaf; Anders Krarup-Hansen; Bjarne Kromann-Andersen; Thomas Horn; Niels Juel Christensen; Helle Westergren Hendel

doi:10.1016/j.ctrv.2010.07.002

Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

Kim Francis Andersen, Rahim Altaf, Anders Krarup-Hansen, Bjarne Kromann-Andersen, Thomas Horn, Niels Juel Christensen, Helle Westergren Hendel

61 Citations (Scopus)

Abstract

Approximately 10% of the pheochromocytomas and 20% of the paragangliomas are malignant with poor survival. As the biological behaviour of these tumours cannot be predicted with certainty from pathology the diagnosis of malignancy is difficult. Genetic testing is gaining impact as mutations in the tumour suppressor gene Von Hippel-Lindau and the mitochondrial succinate dehydrogenase enzyme complex subunit B (SDHB) are associated with malignancy. Excess release of catecholamines is characteristic for pheochromocytomas. High levels of chromogranin A, that is co-stored and co-secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using ¹²³I-MIBG, somatostatin analogues, ¹⁸F-DOPA, and ¹⁸F-FDG. These modalities are recommended in patients with extra-adrenal and suspected metastatic/malignant disease, in case of distorted post-operative anatomy, and when suspected recurrence. The sensitivities of ¹²³I-MIBG scintigraphy or ¹⁸F-DOPA PET are relatively low in SDHB mutated tumours, but high using ¹⁸F-FDG. Specific PET imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules needed for carcinogenesis and tumour growth show encouraging results.

Original language	English
Journal	Cancer Treatment Reviews
Volume	37
Issue number	2
Pages (from-to)	111-9
Number of pages	9
ISSN	0305-7372
DOIs	https://doi.org/10.1016/j.ctrv.2010.07.002
Publication status	Published - 1 Apr 2011

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title = "Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach",

abstract = "Approximately 10% of the pheochromocytomas and 20% of the paragangliomas are malignant with poor survival. As the biological behaviour of these tumours cannot be predicted with certainty from pathology the diagnosis of malignancy is difficult. Genetic testing is gaining impact as mutations in the tumour suppressor gene Von Hippel-Lindau and the mitochondrial succinate dehydrogenase enzyme complex subunit B (SDHB) are associated with malignancy. Excess release of catecholamines is characteristic for pheochromocytomas. High levels of chromogranin A, that is co-stored and co-secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using 123I-MIBG, somatostatin analogues, 18F-DOPA, and 18F-FDG. These modalities are recommended in patients with extra-adrenal and suspected metastatic/malignant disease, in case of distorted post-operative anatomy, and when suspected recurrence. The sensitivities of 123I-MIBG scintigraphy or 18F-DOPA PET are relatively low in SDHB mutated tumours, but high using 18F-FDG. Specific PET imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules needed for carcinogenesis and tumour growth show encouraging results.",

author = "Andersen, {Kim Francis} and Rahim Altaf and Anders Krarup-Hansen and Bjarne Kromann-Andersen and Thomas Horn and Christensen, {Niels Juel} and Hendel, {Helle Westergren}",

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doi = "10.1016/j.ctrv.2010.07.002",

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T1 - Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

AU - Andersen, Kim Francis

AU - Altaf, Rahim

AU - Krarup-Hansen, Anders

AU - Kromann-Andersen, Bjarne

AU - Horn, Thomas

AU - Christensen, Niels Juel

AU - Hendel, Helle Westergren

PY - 2011/4/1

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N2 - Approximately 10% of the pheochromocytomas and 20% of the paragangliomas are malignant with poor survival. As the biological behaviour of these tumours cannot be predicted with certainty from pathology the diagnosis of malignancy is difficult. Genetic testing is gaining impact as mutations in the tumour suppressor gene Von Hippel-Lindau and the mitochondrial succinate dehydrogenase enzyme complex subunit B (SDHB) are associated with malignancy. Excess release of catecholamines is characteristic for pheochromocytomas. High levels of chromogranin A, that is co-stored and co-secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using 123I-MIBG, somatostatin analogues, 18F-DOPA, and 18F-FDG. These modalities are recommended in patients with extra-adrenal and suspected metastatic/malignant disease, in case of distorted post-operative anatomy, and when suspected recurrence. The sensitivities of 123I-MIBG scintigraphy or 18F-DOPA PET are relatively low in SDHB mutated tumours, but high using 18F-FDG. Specific PET imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules needed for carcinogenesis and tumour growth show encouraging results.

AB - Approximately 10% of the pheochromocytomas and 20% of the paragangliomas are malignant with poor survival. As the biological behaviour of these tumours cannot be predicted with certainty from pathology the diagnosis of malignancy is difficult. Genetic testing is gaining impact as mutations in the tumour suppressor gene Von Hippel-Lindau and the mitochondrial succinate dehydrogenase enzyme complex subunit B (SDHB) are associated with malignancy. Excess release of catecholamines is characteristic for pheochromocytomas. High levels of chromogranin A, that is co-stored and co-secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using 123I-MIBG, somatostatin analogues, 18F-DOPA, and 18F-FDG. These modalities are recommended in patients with extra-adrenal and suspected metastatic/malignant disease, in case of distorted post-operative anatomy, and when suspected recurrence. The sensitivities of 123I-MIBG scintigraphy or 18F-DOPA PET are relatively low in SDHB mutated tumours, but high using 18F-FDG. Specific PET imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules needed for carcinogenesis and tumour growth show encouraging results.

U2 - 10.1016/j.ctrv.2010.07.002

DO - 10.1016/j.ctrv.2010.07.002

M3 - Review

C2 - 20675056

SN - 0305-7372

VL - 37

SP - 111

EP - 119

JO - Cancer Treatment Reviews

JF - Cancer Treatment Reviews

IS - 2

ER -

Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

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