European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma

Abstract

Purpose

Diverse radionuclide imaging techniques are available for the diagnosis, staging, and follow-up of phaeochromocytoma and paraganglioma (PPGL). Beyond their ability to detect and localise the disease, these imaging approaches variably characterise these tumours at the cellular and molecular levels and can guide therapy. Here we present updated guidelines jointly approved by the EANM and SNMMI for assisting nuclear medicine practitioners in not only the selection and performance of currently available single-photon emission computed tomography and positron emission tomography procedures, but also the interpretation and reporting of the results.

Methods

Guidelines from related fields and relevant literature have been considered in consultation with leading experts involved in the management of PPGL. The provided information should be applied according to local laws and regulations as well as the availability of various radiopharmaceuticals.

Conclusion

Since the European Association of Nuclear Medicine 2012 guidelines, the excellent results obtained with gallium-68 (68Ga)-labelled somatostatin analogues (SSAs) in recent years have simplified the imaging approach for PPGL patients that can also be used for selecting patients for peptide receptor radionuclide therapy as a potential alternative or complement to the traditional theranostic approach with iodine-123 (123I)/iodine-131 (131I)-labelled meta-iodobenzylguanidine. Genomic characterisation of subgroups with differing risk of lesion development and subsequent metastatic spread is refining the use of molecular imaging in the personalised approach to hereditary PPGL patients for detection, staging, and follow-up surveillance.

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Abbreviations

AADC:

Aromatic L-amino acid decarboxylase

BAT:

Brown adipose tissue

CI:

Confidence interval

CTAC:

CT-based attenuation correction

CT:

Computed tomography

COMT:

Catechol-O-methyltransferase

DA:

Dopamine

DOPA:

Dihydroxyphenylalanine

DTPA:

Diethylenetriaminepentaacetic acid

EGLN1:

egl-9 family hypoxia inducible factor 1α

EGLN2:

egl-9 family hypoxia-inducible factor 2α

EPAS1/HIF2A:

Endothelial PAS domain protein 1/hypoxia-inducible factor 2α

EANM:

European Association of Nuclear Medicine

EPI:

Epinephrine

FH:

Fumarate hydratase

GEP:

Gastroenteropancreatic

GI:

Gastrointestinal

GIST:

Gastrointestinal stromal tumour

HNPGL:

Head and neck paraganglioma

5HT:

5-hydroxytryptamine

MAX:

Myc-associated factor X

MEN:

Multiple endocrine neoplasia

MIBG:

Meta-iodobenzylguanidine

MRI:

Magnetic resonance imaging

MTC:

Medullary thyroid carcinoma

NE:

Norepinephrine

NET:

Neuroendocrine tumour

NF1:

Neurofibromin 1

NPV:

Negative predictive value

PET:

Positron emission tomography

PGL:

Paraganglioma

PHD 1/2:

Prolyl hydroxylase 1/2

PPGL:

Phaeochromocytoma and paraganglioma

PHEO:

Phaeochromocytoma

PPV:

Positive predictive value

PRRT:

Peptide receptor radionuclide therapy

RET:

Rearranged during transfection proto-oncogene

RCC:

Renal cell carcinoma

SDH:

Succinate dehydrogenase

SDHA, -B, -C, -D:

Succinate dehydrogenase subunits A, B, C, and D

SDHx:

Succinate dehydrogenase subunits

SNMMI:

Society of Nuclear Medicine and Molecular Imaging

SPECT:

Single-photon emission computed tomography

SSAs:

Somatostatin analogues

SSTa:

Somatostatin agonist

SSTR:

Somatostatin receptor

SSTR1, -2, -3, -4, -5:

Somatostatin receptor subtypes 1, 2, 3, 4, and 5

SUV:

Standardised uptake value

TLC:

Thin-layer chromatography

TMEM127:

Transmembrane protein 127

VHL:

von Hippel–Lindau

VMAT:

Vesicular monoamine transporter

WHO:

World Health Organization

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Acknowledgements

We thank the EANM Committees, EANM National Societies, and the SNMMI bodies for their review and contribution.

K.P. was supported by the Intramural Research Program of NIH, NICHD.

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Correspondence to David Taïeb.

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RH has received research funding from Ipsen, Novartis, and Clarity Pharmaceuticals and holds stock in Telix Pharmaceuticals on behalf of his institution.

NPT has received consultant/advisory board/honoraria (current/past) from Y-mAbs, Progenics, Bayer, and MedImmune/AstraZeneca and sponsored trials/research support from ImaginAb, Genentech, and Actinium Pharma/FluoroPharma.

The other authors declare that they have no conflict of interest.

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Taïeb, D., Hicks, R.J., Hindié, E. et al. European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 46, 2112–2137 (2019). https://doi.org/10.1007/s00259-019-04398-1

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Keywords

  • Guidelines
  • Radionuclide imaging
  • Phaeochromocytoma
  • Paraganglioma
  • Targeted radionuclide therapy
  • Positron emission tomography
  • Somatostatin analogues