Abstract | BACKGROUND: To investigate the diagnostic and prognostic performances of 18F-FDG PET/CT measures of metabolic tumour burden in patients with neurofibromatosis type-1 (NF1), suspect of malignant transformation. METHODS: This retrospective study included 49 patients (15-60 years old, 30 women) with a diagnosis of NF1, followed in our Reference Centre for Rare Neuromuscular Diseases, who presented clinical signs of tumour progression ( pain, neurological deficit, tumour growth). Quantitative metabolic parameters were measured on 149 tumoral targets, using semi-automatic software and the best cut off values to predict transformation was assessed by Receiver Operating Characteristics (ROC) analysis. Prognostic value of PET/CT metabolic parameters was assessed by Kaplan-Meier estimates of overall survival. RESULTS: Lesions were histologically documented in 40 patients: a sarcomatous transformation was found in 16, a dysplastic neurofibroma (NF) in 7, and a benign NF in 17; in the remaining 9 patients, a minimal follow-up of 12 mo (median 59 mo) confirmed the absence of transformation. The optimal cut off values for detection of malignant transformation were, in decreasing order of area under the ROC curves, a tumour-to-liver (T/L) ratio >2.5, SUVmax > 4.5, total lesion glycolysis (TLG) > 377, total metabolic tumour volume (TMTV) > 88 cm3, and heterogeneity index (HIsuv) > 1.69. The best prognostic marker was the TLG: the 4-y estimates of survival were 97% [95% CI, 90% - 100%] in patients with TLG ≤ 377 vs. 27% [95% CI, 5% - 49%] in patients with TLG > 377 (P < 0.0001; χ2 27.85; hazard ratio 13.27 [95% CI, 3.72-47.35]). T/L ratio, SUVmax and TMTV demonstrated slightly lower performance to predict survival, with χ2 ranging 14.41-19.12. The HIsuv index was not predictive of survival. CONCLUSION: Our study demonstrates that TLG and TMTV, as PET/CT measures of metabolic tumour burden, may be used clinically to identify sarcomatous transformation in patients with NF1 and predict overall survival, with a higher specificity for the TLG. Conventional measures such as the SUVmax, and T/L ratio also demonstrate high prognostic value.
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Authors | Axel Van Der Gucht, Ouidad Zehou, Soraya Djelbani-Ahmed, Laurence Valeyrie-Allanore, Nicolas Ortonne, Pierre Brugières, Pierre Wolkenstein, Alain Luciani, Alain Rahmouni, Emilie Sbidian, Emmanuel Itti |
Journal | PloS one
(PLoS One)
Vol. 11
Issue 3
Pg. e0151809
( 2016)
ISSN: 1932-6203 [Electronic] United States |
PMID | 26987124
(Publication Type: Journal Article)
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Chemical References |
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Topics |
- Adolescent
- Adult
- Cell Transformation, Neoplastic
(metabolism, pathology)
- Female
- Fluorodeoxyglucose F18
- Humans
- Male
- Middle Aged
- Neurofibromatosis 1
(diagnostic imaging, metabolism, mortality, pathology)
- Prognosis
- Radionuclide Imaging
- Retrospective Studies
- Sarcoma
(diagnostic imaging, metabolism, mortality, pathology)
- Survival Rate
- Tumor Burden
- Young Adult
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