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

Taïeb, David; Hicks, Rodney J.; Hindié, Elif; Guillet, Benjamin; Avram, Anca M.; Ghedini, Pietro; Timmers, Henri; Scott, Aaron; Elojeimy, Saeed; Rubello, Domenico; Virgolini, Irene; Fanti, Stefano; Balogova, Sona; Pandit‐Taskar, Neeta; Pacák, Karel

doi:10.1007/s00259-019-04398-1

Cited by 271 publications

(323 citation statements)

References 153 publications

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“…Data from NIH on apparently sporadic PHEOs also demonstrates similar effectiveness between 18 F-FDOPA and 68 Ga-DOTATATE PET/CT (15). There are at least 20 known (18,19). Our patient had elevations in normetanephrine, metanephrines and epinephrine, thus not clearly identifying into one biochemical phenotype.…”

Section: Discussionmentioning

confidence: 60%

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

Tepede

Welch

Lee

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Pheochromocytoma (PHEO) in multiple endocrine neoplasia type 1 (MEN1) is extremely rare. The incidence is reported as less than 2%. We report a case of a 76-year-old male with familial MEN1 who was found to have unilateral PHEO. Although the patient was normotensive and asymptomatic, routine screening imaging with CT demonstrated bilateral adrenal masses. The left adrenal mass grew from 2.5 to 3.9 cm over 4 years with attenuation values of 9 Hounsfield units (HU) pre-contrast and 15 HU post-contrast washout. Laboratory evaluation demonstrated an adrenergic biochemical phenotype. Both 18F-fluorodeoxyglucose (18F-FDG) PET/CT and 123I-metaiodobenzylguanidine (123I-mIBG) scintigraphy demonstrated bilateral adrenal uptake. In contrast, 18F-fluorodihydroxyphenylalanine (18F-FDOPA) PET/CT demonstrated unilateral left adrenal uptake (28.7 standardized uptake value (SUV)) and physiologic right adrenal uptake. The patient underwent an uneventful left adrenalectomy with pathology consistent for PHEO. Post-operatively, he had biochemical normalization. A review of the literature suggests that adrenal tumors >2 cm may be at higher risk for pheochromocytoma in patients with MEN1. Despite a lack of symptoms related to catecholamine excess, enlarging adrenal nodules should be biochemically screened for PHEO. 18F-FDOPA PET/CT may be beneficial for localization in these patients. Learning points: 18F-FDOPA PET/CT is a beneficial imaging modality for identifying pheochromocytoma in MEN1 patients. Adrenal adenomas should undergo routine biochemical workup for PHEO in MEN1 and can have serious peri-operative complications if not recognized, given that MEN1 patients undergo frequent surgical interventions. MEN1 is implicated in the tumorigenesis of PHEO in this patient.

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Section: Discussionmentioning

confidence: 60%

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

Tepede

Welch

Lee

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…Beyond their ability to specifically detect and localize PPGLs, these imaging approaches variably characterize these tumors at cellular and molecular levels. More recently, gallium-68 ( 68 Ga)-labeled somatostatin receptor analogs (SSAs) positron emission tomography-computed tomography (PET/CT) is being increasingly performed to detect PPGLs and assess their somatostatin receptor (SSTR) expression which is paramount for PRRT in inoperable or locally aggressive/metastatic disease (Nolting et al 2019, Taieb et al 2019.…”

Section: :11mentioning

confidence: 99%

Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma in the era of genomic characterization of disease subgroups

Taïeb

Jha

Treglia

2019

Endocrine-Related Cancer

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In recent years, advancement in genetics has profoundly helped to gain a more comprehensive molecular, pathogenic, and prognostic picture of pheochromocytomas and paragangliomas (PPGLs). Newly discovered molecular targets, particularly those that target cell membranes or signaling pathways have helped move nuclear medicine in the forefront of PPGL precision medicine. This is mainly based on the introduction and increasing experience of various PET radiopharmaceuticals across PPGL genotypes quickly followed by implementation of novel radiotherapies and revised imaging algorithms. Particularly, 68Ga-labeled-SSAs have shown excellent results in the diagnosis and staging of PPGLs and in selecting patients for PRRT as a potential alternative to 123/131I-MIBG theranostics. PRRT using 90Y/177Lu-DOTA-SSAs has shown promise for treatment of PPGLs with improvement of clinical symptoms and/or disease control. However, more well-designed prospective studies are required to confirm these findings, in order to fully exploit PRRT’s antitumoral properties to obtain the final FDA approval. Such an approval has recently been obtained for high‐specific-activity 131I-MIBG for inoperable/metastatic PPGL. The increasing experience and encouraging preliminary results of these radiotherapeutic approaches in PPGLs now raises an important question of how to further integrate them into PPGL management (e.g. monotherapy or in combination with other systemic therapies), carefully taking into account the PPGLs locations, genotypes, and growth rate. Thus, targeted radionuclide therapy (TRT) should preferably be performed at specialized centers with an experienced interdisciplinary team. Future perspectives include the introduction of dosimetry and biomarkers for therapeutic responses for more individualized treatment plans, α-emitting isotopes, and the combination of TRT with other systemic therapies.

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“…1 This is of prime importance as alteration of mitochondrial metabolism through inactivation of the TCA cycle and electron transport chain are also found in several cancers, signifying that these metabolic changes constitute an emerging metabolic hallmark of cancer. 1 This is of prime importance as alteration of mitochondrial metabolism through inactivation of the TCA cycle and electron transport chain are also found in several cancers, signifying that these metabolic changes constitute an emerging metabolic hallmark of cancer.…”

Section: Introductionmentioning

confidence: 99%

Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A ¹⁸F‐FDOPA PET/CT study in head and neck paragangliomas

Reichert

Fakhry

Lavieille

et al. 2019

Clinical Endocrinology

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Objectives: Nuclear imaging findings by virtue of phenotyping disease heavily depend on genetic background, metabolites, cell membrane specific targets and signalling pathways. PPGL related to succinate dehydrogenase subunits mutations (SDHx mutations) are less differentiated than other subgroups and therefore may lack to concentrate 18 F-FDOPA, a precursor of catecholamines biosynthesis. However, this 18 F-FDOPA negative phenotype has been reported mostly in SDHx-PPGL of sympathetic origin, suggesting that both genotype status and location (from sympathetic vs parasympathetic paraganglia; adrenal vs extra-adrenal) could influence 18 F-FDOPA uptake. The aim of this study was to test if SDHx drives 18 F-FDOPA uptake in presence of normal epinephrine/norepinephrine concentrations. Design: Retrospective studyPatients: A cohort of 86 head and neck PPGL patients (including three metastatic) with normal metanephrines underwent 18 F-FDOPA PET/CT. The relationships between 18 F-FDOPA uptake and tumour genotype were evaluated. Results:In nonmetastatic HNPGL (50 non-SDHx/33 SDHx), no significant difference was observed between these two groups for SUVmax (P = .256), SUVmean (P = .188), MTV 42% (P = .596) and total lesion uptake (P = .144). Metastatic HNPGL also had high elevated uptake values. Conclusions:Our results suggest that SDH deficiency or metastatic behaviour have no influence on 18 F-FDOPA uptake in HNPGL probably due to their very-well differentiation status, even at metastatic stage. The potential prognosticator value of 18 F-FDOPA uptake would need to be further explored in the setting of metastatic PPGL of sympathetic origin.

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

Cited by 271 publications

References 153 publications

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma in the era of genomic characterization of disease subgroups

Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A ¹⁸F‐FDOPA PET/CT study in head and neck paragangliomas

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

Cited by 271 publications

References 153 publications

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma in the era of genomic characterization of disease subgroups

Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A 18F‐FDOPA PET/CT study in head and neck paragangliomas

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Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A ¹⁸F‐FDOPA PET/CT study in head and neck paragangliomas