Qualitative and quantitative biomarker of response to radiotherapy is needed in paragangliomas. We aim at assessing the added value of 1H-SRM for the evaluation of early response to EBR therapy in patients with cervical SDHx PGL.
Paragangliomas (PGL) are rare neuroendocrine tumors inherited in 40% of cases. SDHx genes (SDHA, SDHB, SDHC, SDHD), encoding the 4 subunits of the mitochondrial enzyme succinate dehydrogenase (SDH), are the most frequently involved. Accumulation of succinate, the substrate for SDH, is a very specific biomarker for these mutations. Recently, we have demonstrated the feasability of detecting and quantifying succinate in tumors in vivo, by magnetic resonance spectroscopy (1H-SRM). Patients carrying these mutations frequently develop cervical PGL for which the treatment of choice is external beam radiation therapy (EBR). The objective of this project is to determine the feasibility of using 1H-SRM for the evaluation of early response to EBR therapy in patients with cervical SDHx PGL.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
DIAGNOSTIC
Masking
NONE
Enrollment
32
PRESS asymetric monovoxel " PROBE ", TE 144 ms, TR 2500 ms, 768 or 1024 averages
Hôpital Lariboisière
Paris, France
RECRUITINGHôpital Pitié-Salpêtrière
Paris, France
RECRUITINGHopital européen Georges Pompidou
Paris, France
RECRUITINGDisappearance of succinate accumulation at M12
Absence of succinate accumulation detected in vivo by 1H-SRM, in cervical PGLs with SDHx mutations, at 12 months after EBR, reviewed by 2 independant experts blinded to all other clinical and genetics data.
Time frame: 12 months
Disapperance of succinate accumulation at M3
Absence of succinate accumulation detected in vivo by 1H-SRM, in cervical PGLs with SDHx mutations, at 3 months after EBR, reviewed by 2 independant experts blinded to all other clinical and genetics data.
Time frame: 3 months
Performance of cervical MRI at M3 and M12 after EBR
Reference diagnostic for response assessed by MRI at 3 months and 12 months after EBR: 1/ Complete response : tumour disappearance ; 2/ Partial response : decrease by at least 30% of tumour volume and no clinical symptomatology worsening ; 3/ stable : stability of tumour size and no clinical symptomatology worsening; 4/ Progression : increase by at least 20% of tumour volume and/or clinical symptomatological worsening
Time frame: 12 months
Performance of PET/CT at M12 after EBR
PERCIST criteria (PET response criteria in solid tumors) assessed by PET/CT at 3 months and 12 months after EBR
Time frame: 12 months
Metastatic evolution or death at M12
Occurence of an event related to the disease during 12 months follow-up (metastatic evolution of the disease or death). Metastases diagnostic is assessed remotely by presence in chromaffin tissue (http://www.sfendocrino.org/article/599/recommandations)
Time frame: 12 months
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