Advertisement

Skeletal Radiology

, Volume 46, Issue 1, pp 93–99 | Cite as

Tumor segmentation of whole-body magnetic resonance imaging in neurofibromatosis type 1 patients: tumor burden correlates

  • Michael A. Heffler
  • Lu Q. Le
  • Yin Xi
  • Avneesh Chhabra
Scientific Article

Abstract

Objective

Segmentation of whole-body MRI (WBMRI) to assess the feasibility, quantitate the total tumor volume (tumor burden) in patients with neurofibromatosis type 1 (NF1) and examine associations with demographic, disease-related and anthropomorphic features.

Methods

A consecutive series of patients with NF1 underwent WBMRI and were reviewed for tumors. Tumors were segmented using a semiautomated software-based tool. Tumors were classified as superficial or deep and discrete or plexiform. Segmentation times were recorded. Segmentation yielded the quantity and tumor burden of superficial, internal and plexiform tumors. Correlations between segmentation data and demographic, disease-related and anthropomorphic features were examined.

Results

Fifteen patients were evaluated (42.3 ± 13.6 years, 10 female, 5 male). Segmentation times were a median of 30 min and yielded 2,328 tumors (1,582 superficial, 746 internal and 23 plexiform). One tumor was malignant. Tumor counts ranged from 14 to 397. Tumor burden ranged from 6.95 cm3 to 571 cm3. Individual tumor volume ranged from 0.0120 cm3 to 298 cm3. Significant correlation was found between the total volume of superficial tumors and height (ρ = 0.5966, p < 0.02). Male patients had higher overall tumor burdens (p < 0.05) and higher superficial tumor burden (p < 0.03). Patients with negative family history had more tumors (p < 0.05).

Conclusion

Segmentation of WBMRI in patients with NF1 is feasible and elucidates meaningful relationships among disease phenotype, anthropomorphic and demographic features.

Keywords

Neurofibromatosis type 1 Whole-body MRI Segmentation Tumor burden Magnetic resonance imaging 

Abbreviations

BMI

Body mass index \( \left(\frac{mass\kern0.5em in\kern0.5em kg}{{\left( height\kern0.5em in\kern0.5em m\right)}^2}\right) \)

MPNST

Malignant peripheral nerve sheath tumor

NF1

Neurofibromatosis type 1 (also known as von Recklinghausen disease)

STIR

Short tau inversion recovery

WBMRI

Whole-body magnetic resonance imaging

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

256_2016_2522_MOESM1_ESM.jpg (122 kb)
Figure 1 A 45-year-old female with known NF1 and right neck swelling. (A) Coronal composed STIR image, (B) maximum intensity projection image, and (C, D) reconstructed sagittal STIR images from the same contiguous data set (3.5-mm-thick slices and 0 mm interslice gap) confirm the plexiform neurofibroma (arrows). (JPG 121 kb)

References

  1. 1.
    Rasmussen SA, Friedman JM. NF1 gene and neurofibromatosis 1. Am J Epidemiol. 2000;151:33–40.CrossRefPubMedGoogle Scholar
  2. 2.
    Plotkin SR, Bredella MA, Cai W, et al. Quantitative assessment of whole-body tumor burden in adult patients with neurofibromatosis. PloS one. 2012;7:e35711.Google Scholar
  3. 3.
    Reynolds RM, Browning GG, Nawroz I, Campbell IW. Von Recklinghausen’s neurofibromatosis: neurofibromatosis type 1. Lancet. 2003;361:1552–4.CrossRefPubMedGoogle Scholar
  4. 4.
    Mulvihill JJ, Parry DM, Sherman JL, Pikus A, Kaiser-Kupfer MI, Eldridge R. NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med. 1990;113:39–52.CrossRefPubMedGoogle Scholar
  5. 5.
    Derlin T, Tornquist K, Munster S, et al. 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. 2013;38:e19–25.CrossRefPubMedGoogle Scholar
  6. 6.
    Salamon J, Mautner VF, Adam G, Derlin T. Multimodal imaging in neurofibromatosis type 1-associated nerve sheath tumors. RoFo Fortschr auf dem Gebiet der Rontgenstr und der Nuklearmed. 2015;187:1084–92.CrossRefGoogle Scholar
  7. 7.
    Cai W, Kassarjian A, Bredella MA, et al. Tumor burden in patients with neurofibromatosis types 1 and 2 and schwannomatosis: determination on whole-body MR images. Radiology. 2009;250:665–73.CrossRefPubMedGoogle Scholar
  8. 8.
    Dugoff L, Sujansky E. Neurofibromatosis type 1 and pregnancy. Am J Med Genet. 1996;66:7–10.CrossRefPubMedGoogle Scholar
  9. 9.
    Lammert M, Mautner VF, Kluwe L. Do hormonal contraceptives stimulate growth of neurofibromas? A survey on 59 NF1 patients. BMC Cancer. 2005;5:16.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    McLaughlin ME, Jacks T. Progesterone receptor expression in neurofibromas. Cancer Res. 2003;63:752–5.PubMedGoogle Scholar
  11. 11.
    Ahlawat S, Fayad LM, Khan MS, et al. Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 2016;87:S31–9.CrossRefPubMedGoogle Scholar
  12. 12.
    Ahlawat S, Baig A, Blakeley JO, Jacobs MA, Fayad LM. Multiparametric whole-body anatomic, functional, and metabolic imaging characteristics of peripheral lesions in patients with schwannomatosis. J Magn Reson Imag. 2016;44(4):794-803.Google Scholar

Copyright information

© ISS 2016

Authors and Affiliations

  • Michael A. Heffler
    • 1
  • Lu Q. Le
    • 2
  • Yin Xi
    • 1
  • Avneesh Chhabra
    • 1
  1. 1.Department of RadiologyUniversity of Texas Southwestern Medical CenterDallasUSA
  2. 2.Department of DermatologyUniversity of Texas Southwestern Medical CenterDallasUSA

Personalised recommendations