The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease

Justus Martijn Brinkman, Johannes L. Bron, Paul I.J.M. Wuisman, Paul J. van Diest, Emile F.I. Comans, Carla F.M. Molthoff

Research output: Contribution to journalArticle

Abstract

Background: Malignant peripheral nerve sheath tumours (MPNST) are known to develop in patients with Neurofibromatosis type I (NF1) resulting in a decreased overall survival. The association between NF1 and the development of such MPNST has been investigated in detail. The biological behaviour however of multiple disseminated neurofibromas in patients with NF1 and the risk factors for malignant transformation remain unknown. Clinical signs are unreliable and additional imaging techniques are therefore required. Of such, positron emission tomography using [18F]-2-fluoro-2-deoxy-D-glucose (18FDG PET) is used to detect malignant changes in neurofibromas. Case presentation: A case is presented of a patient suffering from NF1 with clinical signs of malignant change and accumulation of 18FDG in multiple neurofibromas. Histopathological examination of 20 lesions however, did not reveal any malignant features. There was no statistically significant relation between 18FDG accumulation and malignant change, but rather with pain, size and growth. Conclusion: This case adds to the knowledge of the diverse biological behaviour of neurofibromas in patients with NF1

Original languageEnglish (US)
Article number130
JournalWorld Journal of Surgical Oncology
Volume5
DOIs
StatePublished - Nov 12 2007

ASJC Scopus subject areas

  • Surgery
  • Oncology

Fingerprint Dive into the research topics of 'The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease'. Together they form a unique fingerprint.

Cite this