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 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 patients
- 84 female 76 male (1.1:1)
- mean age 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
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%)

10. Recurrence Rates after Surgery.
Ballo 1999
5 years
Sorensen 2002
5 years
Phillips 2004
3 years
Nyttens 2000
39%

11. Does recurrence at presentation affect outcome?
Our series patients
primary - 30%
- 41 recurrent - 67%
Milan (2003) patients
primary - 24% recurrent - 41%

12. Recurrence rate after Excision

13. Outcome of Recurence
Mean time to recurrence 18.6 months ( 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
Number of Patients
(147)
Number of recurrences (60) %
Debulking 3
100%
Intralesional 79 30
38%
Marginal 55 23
42%
Wide 10 4
40%

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 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. (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)

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