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, It exhibits self limiting behaviour Shows growth arrest or spontaneous regression in many patients

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 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 114 no previous treatment 46 treated elsewhere and presenting with recurrent disease Follow up 13 – 205 months ( mean 49 months)

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

Results of surgical Treatment All patients Primary presentation Recurrent presentation Number treated with surgery 147 106 41 No recurrence 88 (59%) 74 (70%) 15 (33%) Recurrence 59 (41%) 32 (30%) 27 (67%)

Recurrence Rates after Surgery. Ballo 1999 30% @ 5 years Sorensen 2002 73% @ 5 years Phillips 2004 19.3% @ 3 years Nyttens 2000 39%

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%

Recurrence rate after Excision

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

Does the Margin of Excision Influence Recurrence?

Recurrence and Margins Number of Patients (147) Number of recurrences (60) % Debulking 3 100% Intralesional 79 30 38% Marginal 55 23 42% Wide 10 4 40%

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 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 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 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 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 Alone - 22% local recurrence. Combined with surgery – 6% local recurrence. Complications – fibrosis paraesthesia oedema fracture late malignancy

(Mendenhall et al; Am J Clin Onc 2005) 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 First surgery has best chance of cure. Therefore if symptomatic and resectable with the possibility of achieving adequate margins and limited morbidity – resect.

If recurrent and asymptomatic observe.

If recurrent and symptomatic - second excision if morbidity low, consider radiotherapy if risk of local recurrence high.

If progressive and inoperable pharmacological +/- radiotherapy.

In selected patients whose only surgical option is amputation … observe.

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.

Thank you