Article Text

Management of paediatric desmoid fibromatosis
  1. HK Holme1,
  2. C Westbury1,
  3. D Morgenstern1,
  4. G Erturan2,
  5. R Tirabosco3,
  6. R Pollock4,
  7. T Briggs4,
  8. A Cassoni1,
  9. M Michelagnoii1
  1. 1The London Sarcoma Service, University College London Hospital, London, UK
  2. 2Department of Orthopaedics, Nuffield Orthopaedic Centre, Oxford, UK
  3. 3Department of Pathology, Royal National Orthopaedic Hospital, London, UK
  4. 4The London Sarcoma Service, Royal National Orthopaedic Hospital, London, UK


Aims Review the experience of a single institution's multi-disciplinary sarcoma group with regards management of fibromatosis.

Methods Retrospective analysis of case notes of all young people (<21 years), with histological diagnosis of desmoid fibromatosis confirmed by expert pathology review, treated between 2003-2010.

Results Thirty-two records were analysed; 4 children seen as second opinion only were excluded. There were 22 girls and 6 boys (3.7:1 ratio). Median age at diagnosis was 13.9 years.

Twenty-three out of 28 children underwent surgical resection; 4 had complete microscopic resection, despite which 3 relapsed at a median of 13 months. Of the remaining 19 children with intra-lesional/unknown disease clearance, 4 remain in continued radiological remission at a median of 50 months, 4 had residual disease on MRI, and 11 had progressive disease at median 10.5 months after surgery.

Hormonal therapies and/or non-steroidal anti-inflammatory drugs were used for 12 children; 8 had stable disease at median 43 months, whilst 3 progressed at a median of 20 months and response assessment is awaited in 1. Three with stable disease had concomitant radiotherapy.

Eleven children were treated with cytotoxic chemotherapy (11 episodes), with progression after 4 episodes at a median of 29 months from diagnosis, while post 6 episodes stable/responsive disease was achieved at a median 35 months. Response assessment in 1 child is awaited.

Eleven children received radiotherapy, with 1 child receiving 2 separate courses (different anatomical sites). Three children had progressive disease following radiotherapy (including the child with multi-focal disease) at a median of 67 months, whilst the remaining 8 (72%) children achieved stable/responsive disease at a median of 51.5 months.

Conclusions With a caveat of small numbers, complete resection is technically more demanding and does not necessarily have a clear outcome advantage compared with incomplete histological resection. We propose excision should only be attempted if associated morbidity is low. Chemotherapeutic and non-cytotoxic agents are an alternative to avoid surgery and radiotherapy in the growing child. Radiotherapy was associated with induction of responsive/stable disease in 72% and is a useful therapy, although concerns about the impact on the growing child will limit its wider use in this patient group.

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