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Journal of Bone and Joint Surgery - British Volume, Vol 91-B, Issue 9, 1223-1226.
doi: 10.1302/0301-620X.91B9.22299  
Copyright © 2009 by British Editorial Society of Bone and Joint Surgery
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Bone sarcomas arising in patients with neurofibromatosis type 1

M. Chowdhry, MRCS, Orthopaedic ST3 Registrar1; C. Hughes, BA, Research and Data Manager1; R. J. Grimer, FRCS, Consultant Orthopaedic Surgeon1; V. Sumathi, FRCPath, Consultant Histopathologist1; S. Wilson, PhD, Professor of Clinical Epidemiology School of Health and Population Sciences2; and L. Jeys, FRCS, Consultant Orthopaedic Surgeon1

1 Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham B31 2AP, UK.
2 University of Birmingham, University Road West, Birmingham B15 2TT, UK.

Correspondence should be sent to Mr R. J. Grimer; e-mail: rob.grimer{at}roh.nhs.uk

We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured.

This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.






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Hip, Knee, Trauma, Upper limb, Foot & Ankle, Paediatrics, Oncology, Spine, Arthroplasty, General