Desmoid Tumour / Agressive Fibromatosis

 

Desmoid MRI 1Desmoid MRI 2Desmoid MRI 3

 

Aggressive Fibromatosis / Desmoid Tumours

 

Definition

 

Deep fibromatosis

- rare monoclonal fibroblastic proliferation

- aggressive, rapidly growing, locally invasive

 

Different from superficial fibromatosis

- small, slow growing

- palmar fibromatosis www.boneschool.com/dupuytrens

- plantar fibromatosis www.boneschool.com/plantar-fibromatosis

 

Deep / Desmoid tumours

 

Types

 

Intra- or Extra-abdominal

 

Primary / Sporadic

 

Associated with familial adenomatous polyposis / Gardener's syndrome

 

Epidemiology

 

3rd to 4th decade

 

Presentation

 

Mass

Neurological compression

 

MRI

 

Soft tissue mass

 

Histology

 

Spindle shaped fibroblasts with plump nuclei & mildly eosinophilic cytoplasm

 

Management

 

Options

 

Desmoid tumour working group consensus 2018

- First line is surveillance

- Surgery if adjacent to the abdominal wall and failing surveillance

- Medical therapy if failing surveillance and not abdominal wall

- Ablation therapy (cryotherapy or radiotherapy) as an alternative to medical therapy, on a case-by-case basis

- Priority of treatment is pain control

 

Natural history



Bonvalot et al Ann Surg Oncol 2023

- 100 patients with desmoid tumours treated with observation

- 58% had tumour regression

- 48% had progression, with 28% requiring active treatment

 

Operative versus nonoperative

 

Penel et al Eur J Cancer 2017

- 771 cases

- overall event free survival at 2 years 56%

- no difference between surgery or observation

 

Cryotherapy

 

Kurtz et al Eur J Cancer 2021

- prospective study of cryotherapy in 50 patients with desmoid tumours

- non progressive disease in 86% with pain control and better functional status

 

Wide resection +/- radiotherapy

 

Nuyttrens et al Cancer 2000

- systematic review

- local control surgery: 61%

- local control surgery + radiotherapy: 75%

- local control radiotherapy: 78%