Extra-Abdominal Fibromatosis : The Birmingham Experience

1. Extra-Abdominal Fibromatosis :The Birmingham Experience Rafiq Abed Lee Jeys Seggy Abudu Rob Grimer Roger Tillman Simon Carter Royal Orthopaedic Hospital, Birmingham UK

4. 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

5. 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.

6. 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)

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

8. 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

9. Results of surgical Treatment

10. Recurrence Rates after Surgery.

11. 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%

12. Recurrence rate after Excision

13. 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

14. Does the Margin of Excision Influence Recurrence?

15. Recurrence and Margins

16. 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.

17. 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?

18. 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’

19. 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’

20. 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

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

22. 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)

23. 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.

24. If recurrent and asymptomatic observe.

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

26. If progressive and inoperable pharmacological +/- radiotherapy.

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

28. 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.

29. Thank you