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Association between benign and malignant peripheral nerve sheath tumors in NF1

From the Department of Medical Genetics (Ms. Tucker and Dr. Friedman), University of British Columbia, Vancouver, Canada; Department of Dermatology (Drs. Wolkenstein, Revuz, and Zeller), Henri-Mondor Hospital, Créteil, France.

Address correspondence and reprint requests to Tracy Tucker, Medical Genetics Research Unit, Children's and Women's Hospital, Box 153, 4500 Oak Street, Room J106, Vancouver, BC V6H 3N1 Canada; e-mail: tbtucker{at}interchange.ubc.ca

Objective: People with neurofibromatosis type 1 (NF1) have a 10% lifetime risk of developing a malignant peripheral nerve sheath tumor (MPNST). MPNSTs are often metastatic and are a frequent cause of death among people with NF1. Clinical evidence suggests that most MPNSTs in people with NF1 develop from preexisting plexiform neurofibromas. However, it is not known whether an individual's risk of developing an MPNST is associated with the burden of benign neurofibromas. The authors conducted a study to determine whether people with NF1 who have benign neurofibromas of various kinds are at greater risk of developing MPNSTs than patients with NF1 who lack these benign tumors.

Methods: Clinical information on 476 NF1 probands in the Henri Mondor Database was analyzed by logistic regression to examine associations between MPNSTs and internal plexiform, superficial plexiform, subcutaneous, and cutaneous neurofibromas.

Results: Individuals with subcutaneous neurofibromas were approximately three times more likely to have internal plexiform neurofibromas or MPNSTs than individuals without subcutaneous neurofibromas. Individuals with internal plexiform neurofibromas were 20 times more likely to have MPNSTs than individuals without internal plexiform neurofibromas. When this analysis was done with both subcutaneous and internal plexiform neurofibromas as explanatory variables, only the association of MPNSTs with internal plexiform neurofibromas remained significant.

Conclusions: The observation that malignant peripheral nerve sheath tumors are strongly associated with internal plexiform neurofibromas suggests that patients with neurofibromatosis type 1 with these benign tumors warrant increased surveillance for malignancy.

Received December 21, 2004. Accepted in final form April 15, 2005.

This article has been cited by other articles:

				V.-F. Mautner, F. A. Asuagbor, E. Dombi, C. Funsterer, L. Kluwe, R. Wenzel, B. C. Widemann, and J. M. Friedman Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1 Neuro-oncol, August 1, 2008; 10(4): 593 - 598. [Abstract] [Full Text] [PDF]

				J A McCaughan, S M Holloway, R Davidson, and W W K Lam Further evidence of the increased risk for malignant peripheral nerve sheath tumour from a Scottish cohort of patients with neurofibromatosis type 1 J. Med. Genet., July 1, 2007; 44(7): 463 - 466. [Abstract] [Full Text] [PDF]