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Extra-Abdominal Fibromatosis : The Birmingham Experience. Rafiq Abed Lee Jeys Seggy Abudu Rob Grimer Roger Tillman Simon Carter. Royal Orthopaedic Hospital, Birmingham UK. Clinical Course. Locally aggressive tumour with a high potential for local recurrence after resection,

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extra abdominal fibromatosis the birmingham experience

Extra-Abdominal Fibromatosis :The Birmingham Experience

Rafiq Abed

Lee Jeys

Seggy Abudu

Rob Grimer

Roger Tillman

Simon Carter

Royal Orthopaedic Hospital, Birmingham UK

clinical course
Clinical Course
  • Locally aggressive tumour with a high potential for local recurrence after resection,
  • It exhibits self limiting behaviour
  • Shows growth arrest or spontaneous regression in many patients
natural history
Natural History

Dalen et al, Acta Orthop Scand 2003

  • 30 patients followed for a mean of 28 years (range 20 – 54 years)
  • 29 excised
  • LR 12 patients
  • > 1 LR in 8 patients
  • 3 spontaneous regression
  • 28 years – 29 tumour free, 1 stable disease @11 years
  • Fibromatosis has a high capacity for self limitation.
our experience demographics
Our Experience : Demographics
  • 181 patients seen in tertiary referral centre
  • Exclusions - 12 less than 1 year follow up

- 9 lost to follow up

  • Study Group - 160 patients

- 84 female 76 male (1.1:1)

- mean age 35.6 years

(range 1 – 96)

previous treatment
Previous Treatment
  • 114 no previous treatment
  • 46 treated elsewhere and presenting with recurrent disease
  • Follow up 13 – 205 months ( mean 49 months)
non surgical treatment
Non surgical treatment
  • 1 observed for 3 years with progressive disease
  • 4 patients inoperable
  • 2 patients radiotherapy alone
  • 2 patients tamoxifen
  • 2 patients NSAID
  • All had stable disease
does recurrence at presentation affect outcome
Does recurrence at presentation affect outcome?
  • Our series - 147 patients

- 106 primary - 30%

- 41 recurrent - 67%

  • Milan (2003) - 203 patients

- 128 primary - 24% - 75 recurrent - 41%

outcome of recurence
Outcome of Recurence
  • Mean time to recurrence 18.6 months (4 -158 months)
  • 37 females, 22 males (1.6:1)
  • 40 further surgery
    • LR in 58%
  • 6 Excision, Radiotherapy + Chemotherapy
    • LR in 66%
  • 9 observed
    • All stable disease
  • 2 Radiotherapy + chemotherapy
    • NED at 68 and 108 months
  • 1 Tamoxifen
    • Stable disease at 119 months
  • 1 Chemotherapy
    • Stable disease at 79 months
is recurrence associated with margins
Is recurrence associated with margins?
  • Margins – difficult to assess macroscopically
  • ‘Univariate analysis margins not associated’ - Sorensen et al; Acta Orth Scand 2002.
  • ‘Recurrence did not correlate with surgical margins’ – Phillips et al; Br J Surg 2004.
  • ‘+ve margins did not affect local control significantly’ – Sharma S Afr J Surg 2006.
is recurrence associated with margins1
Is recurrence associated with margins?
  • Nuyttens et al; Cancer 2000 (April 1st!)
  • Recurrence rate -ve margins 28%

+ve margins 59%

  • Complete surgical clearance does not prevent recurrence.
  • Incomplete margins do not mean recurrence.
  • Should we therefore perform surgery with high morbidity to achieve adequate margins?
is recurrence associated with margins2
Is recurrence associated with margins?
  • Lewis et al; Ann Surg 1999
  • ‘aggressive attempts at achieving negative margins may result in unnecessary morbidity. Function and structure preserving procedures should be the primary goal’
is recurrence associated with margins3
Is recurrence associated with margins?
  • Gronchi et al J Clin Oncol 2003
  • ‘Presence of microscopic disease does not necessarily affect long term disease free survival in patients with primary presentation of extra abdominal desmoid tumours’
effect of delay on outcome
Effect of Delay on Outcome
  • 8 observed for 9 – 55 months ( mean 33.8) then operated
    • 3 asymptomatic
    • 5 close to N/V bundle
  • Operated for - Pain (2 patients)

- Progression (6 patients)

  • 7 intralesional excision no recurrence (fu 9 -52 months, mean 24.5)
  • 1 debulking but progressive disease despite chemo + radiotherapy
  • Delay in treatment by period of observation does not influence outcome
radiotherapy
Radiotherapy
  • Alone - 22% local recurrence.
  • Combined with surgery – 6% local recurrence.
  • Complications – fibrosis

paraesthesia

oedema

fracture

late malignancy

pharmacology
Pharmacology
  • Response rates – 40 – 50%

but duration variable and ……

‘should be used in patients with progressive disease following failure of local treatment.’

(Mendenhall et al; Am J Clin Onc 2005)

birmingham policy
Birmingham Policy
  • First surgery has best chance of cure.
  • Therefore if symptomatic and resectable with the possibility of achieving adequate margins and limited morbidity – resect.
slide25
If recurrent and symptomatic - second excision if morbidity low, consider radiotherapy if risk of local recurrence high.
but remember
But remember -
  • Fibromatosis does not need treatment
  • Can spontaneously regress
  • Is an enigma
  • Avoid unnecessary morbidity
  • Get the patients before some one else does!
  • Always bigger than the MRI suggests.