Top

Published in:

Open Access 01-02-2022 | Neurofibromatosis | Clinical Study

Diagnostic value of ¹⁸F-FDG PET-CT in detecting malignant peripheral nerve sheath tumors among adult and pediatric neurofibromatosis type 1 patients

Authors: Ritch T. J. Geitenbeek, Enrico Martin, Laura H. Graven, Martijn P. G. Broen, Monique H. M. E. Anten, Jochem A. J. van der Pol, Cornelis Verhoef, Walter Taal

Published in: Journal of Neuro-Oncology | Issue 3/2022

Abstract

Purpose

Detecting malignant peripheral nerve sheath tumors (MPNSTs) remains difficult. ¹⁸F-FDG PET-CT has been shown helpful, but ideal threshold values of semi-quantitative markers remain unclear, partially because of variation among scanners. Using EU-certified scanners diagnostic accuracy of ideal and commonly used ¹⁸F-FDG PET-CT thresholds were investigated and differences between adult and pediatric lesions were evaluated.

Methods

A retrospective cohort study was performed including patients from two hospitals with a clinical or radiological suspicion of MPNST between 2013 and 2019. Several markers were studied for ideal threshold values and differences among adults and children. A diagnostic algorithm was subsequently developed.

Results

Sixty patients were included (10 MPNSTs). Ideal threshold values were 5.8 for SUVmax (sensitivity 0.70, specificity 0.92), 5.0 for SUVpeak (sensitivity 0.70, specificity 0.97), 1.7 for TLmax (sensitivity 0.90, specificity 0.86), and 2.3 for TLmean (sensitivity 0.90, specificity 0.79). The standard TLmean threshold value of 2.0 yielded a sensitivity of 0.90 and specificity of 0.74, while the standard SUVmax threshold value of 3.5 yielded a sensitivity of 0.80 and specificity of 0.63. SUVmax and adjusted SUV for lean body mass (SUL) were lower in children, but tumor-to-liver ratios were similar in adult and pediatric lesions. Using TLmean > 2.0 or TLmean < 2.0 and SUVmax > 3.5, a sensitivity and specificity of 1.00 and 0.63 can be achieved.

Conclusion

¹⁸F-FDG PET-CT offers adequate accuracy to detect MPNSTs. SUV values in pediatric MPNSTs may be lower, but tumor-to-liver ratios are not. By combining TLmean and SUVmax values, a 100% sensitivity can be achieved with acceptable specificity.

Available only for authorised users

Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG (2005) A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. J Neurosurg 102(2):246–255. https://doi.org/10.3171/jns.2005.102.2.0246CrossRefPubMed

Montano N, D’Alessandris QG, D’Ercole M et al (2016) Tumors of the peripheral nervous system: analysis of prognostic factors in a series with long-term follow-up and review of the literature. J Neurosurg 125(2):363–371. https://doi.org/10.3171/2015.6.JNS15596CrossRefPubMed

Friedman JM (1999) Epidemiology of neurofibromatosis type 1. Am J Med Genet 89(1):1–6CrossRef

Listernick R, Charrow J (1990) Neurofibromatosis type 1 in childhood. J Pediatr 116(6):845–853. https://doi.org/10.1016/S0022-3476(05)80639-0CrossRefPubMed

Staedtke V, Bai R-Y, Blakeley JO (2017) Cancer of the peripheral nerve in neurofibromatosis type 1. Neurotherapeutics 14(2):298–306. https://doi.org/10.1007/s13311-017-0518-yCrossRefPubMedPubMedCentral

Le Guellec S, Decouvelaere A-V, Filleron T et al (2016) Malignant peripheral nerve sheath tumor is a challenging diagnosis: a systematic pathology review, immunohistochemistry, and molecular analysis in 160 patients from the french sarcoma group database. Am J Surg Pathol 40(7):896–908. https://doi.org/10.1097/PAS.0000000000000655CrossRefPubMed

Brennan MF, Antonescu CR, Moraco N, Singer S (2014) Lessons learned from the study of 10,000 patients with soft tissue sarcoma. Ann Surg 260(3):416–422. https://doi.org/10.1097/SLA.0000000000000869CrossRefPubMed

Evans DGR, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (2002) Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 39(5):311–314. https://doi.org/10.1136/jmg.39.5.311CrossRefPubMedPubMedCentral

Ferner RE, Huson SM, Thomas N et al (2007) Guidelines for the diagnosis and management of individuals with neurofibromatosis. J Med Genet 44(2):81–88. https://doi.org/10.1136/jmg.2006.045906CrossRefPubMed

10.

Valentin T, Le Cesne A, Ray-Coquard I et al (2016) Management and prognosis of malignant peripheral nerve sheath tumors: the experience of the French Sarcoma Group (GSF-GETO). Eur J Cancer 56:77–84. https://doi.org/10.1016/j.ejca.2015.12.015CrossRefPubMed

11.

Martin E, Coert JH, Flucke UE et al (2019) A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours. Eur J Cancer 124:77–87. https://doi.org/10.1016/j.ejca.2019.10.014CrossRefPubMed

12.

Stucky C-CH, Johnson KN, Gray RJ et al (2012) Malignant peripheral nerve sheath tumors (MPNST): the mayo clinic experience. Ann Surg Oncol 19(3):878–885. https://doi.org/10.1245/s10434-011-1978-7CrossRefPubMed

13.

Nelson CN, Dombi E, Rosenblum JS et al (2020) Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1. J Neurosurg 133(5):1516–1526. https://doi.org/10.3171/2019.7.JNS191353CrossRef

14.

Hajiabadi MM, Campos B, Sedlaczek O et al (2020) Interdisciplinary approach allows minimally invasive, nerve-sparing removal of retroperitoneal peripheral nerve sheath tumors. Langenbeck’s Arch Surg 405(2):199–205. https://doi.org/10.1007/s00423-019-01851-5CrossRef

15.

Dunn GP, Spiliopoulos K, Plotkin SR et al (2013) Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1: clinical article. J Neurosurg 118(1):142–148. https://doi.org/10.3171/2012.9.JNS101610CrossRefPubMed

16.

Tovmassian D, Abdul Razak M, London K (2016) The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol 2016:1–7. https://doi.org/10.1155/2016/6162182CrossRef

17.

Salamon J, Veldhoen S, Apostolova I et al (2014) F-18-FDG PET/CT for detection of malignant peripheral nerve sheath tumours in neurofibromatosis type 1: tumour-to-liver ratio is superior to an SUVmax cut-off. Eur Radiol 24(2):405–412. https://doi.org/10.1007/s00330-013-3020-xCrossRefPubMed

18.

Salamon J, Derlin T, Bannas P et al (2013) Evaluation of intratumoural heterogeneity on 18F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1. Eur J Nucl Med Mol Imaging 40(5):685–692. https://doi.org/10.1007/s00259-012-2314-6CrossRefPubMed

19.

Salamon J, Papp L, Tóth Z et al (2015) Nerve sheath tumors in neurofibromatosis type 1: assessment of whole-body metabolic tumor burden using F-18-FDG PET/CT. PLoS ONE 10(12):e0143305. https://doi.org/10.1371/journal.pone.0143305CrossRefPubMedPubMedCentral

20.

Ahlawat S, Blakeley JO, Rodriguez FJ, Fayad LM (2019) Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology 93(11):e1076–e1084. https://doi.org/10.1212/WNL.0000000000008092CrossRefPubMed

21.

Derlin T, Tornquist K, Münster S et al (2013) Comparative effectiveness of 18F-FDG PET/CT versus whole-body MRI for detection of malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Clin Nucl Med 38(1):e19–e25. https://doi.org/10.1097/RLU.0b013e318266ce84CrossRefPubMed

22.

Warbey VS, Ferner RE, Dunn JT, Calonje E, O’Doherty MJ (2009) FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1. Eur J Nucl Med Mol Imaging 36(5):754–757. https://doi.org/10.1007/s00259-008-1038-0CrossRef

23.

Ferner RE, Golding JF, Smith M et al (2008) [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol 19(2):390–394. https://doi.org/10.1093/annonc/mdm450CrossRefPubMed

24.

Martin E, Geitenbeek RTJ, Coert JH et al (2020) A Bayesian approach for diagnostic accuracy of malignant peripheral nerve sheath tumors: a systematic review and meta-analysis. Neuro Oncol. https://doi.org/10.1093/neuonc/noaa280CrossRefPubMedCentral

25.

Azizi AA, Slavc I, Theisen BE et al (2018) Monitoring of plexiform neurofibroma in children and adolescents with neurofibromatosis type 1 by [(18) F]FDG-PET imaging. Is it of value in asymptomatic patients. Pediatr Blood Cancer 65(1):e26733. https://doi.org/10.1002/pbc.26733CrossRef

26.

Moharir M, London K, Howman-Giles R et al (2010) Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging 37(7):1309–1317. https://doi.org/10.1007/s00259-010-1386-4CrossRefPubMed

27.

Tsai LL, Drubach L, Fahey F, Irons M, Voss S, Ullrich NJ (2012) [18F]-Fluorodeoxyglucose positron emission tomography in children with neurofibromatosis type 1 and plexiform neurofibromas: correlation with malignant transformation. J Neurooncol 108(3):469–475. https://doi.org/10.1007/s11060-012-0840-5CrossRefPubMed

28.

Friedrich RE, Hartmann M, Mautner VF (2007) Malignant peripheral nerve Sheath tumors (MPNST) in NF1-affected children. Anticancer Res 27(4A):1957–1960PubMed

29.

Tahari AK, Chien D, Azadi JR, Wahl RL (2014) Optimum lean body formulation for correction of standardized uptake value in PET imaging. J Nucl Med 55(9):1481–1484. https://doi.org/10.2967/jnumed.113.136986CrossRefPubMed

30.

Kinahan PE, Fletcher JW (2010) Positron emission tomography-computed tomography standardized uptake values in clinical practice and assessing response to therapy. Semin Ultrasound, CT MRI 31(6):496–505. https://doi.org/10.1053/j.sult.2010.10.001CrossRef

31.

Broski SM, Johnson GB, Howe BM et al (2016) Evaluation of 18F-FDG PET and MRI in differentiating benign and malignant peripheral nerve sheath tumors. Skeletal Radiol 45(8):1097–1105. https://doi.org/10.1007/s00256-016-2394-7CrossRefPubMed

32.

Bensaid B, Giammarile F, Mognetti T et al (2007) Intérêt de la tomographie par émission de positons au fluorodéoxyglucose 18 dans la détection des neurofibrosarcomes au cours de la neurofibromatose de type 1. Ann Dermatol Venereol 134(10):735–741. https://doi.org/10.1016/S0151-9638(07)92528-4CrossRefPubMed

33.

Benz MR, Czernin J, Dry SM et al (2010) Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer 116(2):451–458. https://doi.org/10.1002/cncr.24755CrossRefPubMed

34.

Cook GJR, Lovat E, Siddique M, Goh V, Ferner R, Warbey VS (2017) Characterisation of malignant peripheral nerve sheath tumours in neurofibromatosis-1 using heterogeneity analysis of (18)F-FDG PET. Eur J Nucl Med Mol Imaging 44(11):1845–1852. https://doi.org/10.1007/s00259-017-3733-1CrossRefPubMedPubMedCentral

35.

Lerman L, Zehou O, Ortonne N et al (2019) Interest of 18F-FDG PET/CT in neurofibromatosis type 1, 10-year experience from the national reference centre Henri-Mondor. Med Nucl 43(5–6):370–380. https://doi.org/10.1016/j.mednuc.2019.05.001LK-CrossRef

36.

Nose H, Otsuka H, Otomi Y et al (2013) Correlations between F-18 FDG PET/CT and pathological findings in soft tissue lesions. J Med Invest 60(3–4):184–190CrossRef

37.

Reinert CP, Schuhmann MU, Bender B et al (2019) Comprehensive anatomical and functional imaging in patients with type I neurofibromatosis using simultaneous FDG-PET/MRI. Eur J Nucl Med Mol Imaging 46(3):776–787. https://doi.org/10.1007/s00259-018-4227-5CrossRefPubMed

38.

Salamon J, Derlin T, Bannas P et al (2013) Evaluation of intratumoural heterogeneity on (1)(8)F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1. Eur J Nucl Med Mol Imaging 40(5):685–692. https://doi.org/10.1007/s00259-012-2314-6CrossRefPubMed

39.

Schwabe M, Spiridonov S, Yanik EL et al (2019) How effective are noninvasive tests for diagnosing malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1? Diagnosing MPNST in NF1 patients. Sarcoma 2019:4627521. https://doi.org/10.1155/2019/4627521CrossRefPubMedPubMedCentral

40.

Warbey VS, Ferner RE, Dunn JT, Calonje E, O’Doherty MJ (2009) [18F]FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1. Eur J Nucl Med Mol Imaging 36(5):751–757. https://doi.org/10.1007/s00259-008-1038-0CrossRefPubMed

41.

Combemale P, Valeyrie-Allanore L, Giammarile F et al (2014) Utility of 18F-FDG PET with a semi-quantitative index in the detection of sarcomatous transformation in patients with neurofibromatosis type 1. PLoS ONE 9(2):e85954. https://doi.org/10.1371/journal.pone.0085954CrossRefPubMedPubMedCentral

42.

Chhabra A, Soldatos T, Durand DJ, Carrino JA, McCarthy EF, Belzberg AJ (2011) The role of magnetic resonance imaging in the diagnostic evaluation of malignant peripheral nerve sheath tumors. Indian J Cancer 48(3):328–334. https://doi.org/10.4103/0019-509X.84945CrossRefPubMed

43.

Karabatsou K, Kiehl T-R, Wilson DM, Hendler A, Guha A (2009) Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery 65(4 Suppl):A160–A170CrossRef

44.

Ferner RE, Lucas JD, O’Doherty MJ et al (2000) Evaluation of 18fluorodeoxyglucose positron emission tomography (18FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatr 68(3):353–357. https://doi.org/10.1136/jnnp.68.3.353CrossRef

45.

Chirindel A, Chaudhry M, Blakeley JO, Wahl R (2015) 18F-FDG PET/CT qualitative and quantitative evaluation in neurofibromatosis type 1 patients for detection of malignant transformation: comparison of early to delayed imaging with and without liver activity normalization. J Nucl Med 56(3):379–385. https://doi.org/10.2967/jnumed.114.142372CrossRefPubMed

46.

Bredella MA, Torriani M, Hornicek F et al (2007) Value of PET in the assessment of patients with neurofibromatosis type 1. AJR 189(4):928–935. https://doi.org/10.2214/AJR.07.2060CrossRefPubMed

47.

Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM (1986) Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer J 57:2006–2021CrossRef

48.

Wong WW, Hirose T, Scheithauer BW, Schild SE, Gunderson LL (1998) Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 42:351–360CrossRef

Title: Diagnostic value of 18F-FDG PET-CT in detecting malignant peripheral nerve sheath tumors among adult and pediatric neurofibromatosis type 1 patients
Authors: Ritch T. J. Geitenbeek
Enrico Martin
Laura H. Graven
Martijn P. G. Broen
Monique H. M. E. Anten
Jochem A. J. van der Pol
Cornelis Verhoef
Walter Taal
Publication date: 01-02-2022
Publisher: Springer US
Keywords: Neurofibromatosis
Neurofibromatosis
Neurofibromatosis Type 1
Published in: Journal of Neuro-Oncology / Issue 3/2022
Print ISSN: 0167-594X
Electronic ISSN: 1573-7373
DOI: https://doi.org/10.1007/s11060-021-03936-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Diagnostic value of ¹⁸F-FDG PET-CT in detecting malignant peripheral nerve sheath tumors among adult and pediatric neurofibromatosis type 1 patients

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 3/2022

Decision making and surgical modality selection in glioblastoma patients: an international multicenter survey

Psychosocial profiles of risk and resiliency in neurofibromatoses: a person-centered analysis of illness adaptation

Tumor load rather than contrast enhancement is associated with the visual function of children and adolescents with optic pathway glioma – a retrospective Magnetic Resonance Imaging study

Immunogenicity of the BNT162b2 mRNA COVID-19 vaccine in patients with primary brain tumors: a prospective cohort study

MR susceptibility imaging for detection of tumor-associated macrophages in glioblastoma

Long term outcomes following surgery for pineal region tumors