extra-abdominal fibromatosis : the birmingham experience rafiq abed lee jeys seggy abudu rob grimer...
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Extra-Abdominal Fibromatosis :
The Birmingham Experience
Rafiq AbedLee Jeys
Seggy AbuduRob Grimer
Roger TillmanSimon Carter
Royal Orthopaedic Hospital, Birmingham UK
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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
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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.
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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)
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Previous Treatment
• 114 no previous treatment• 46 treated elsewhere and
presenting with recurrent disease
• Follow up 13 – 205 months ( mean 49 months)
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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
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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%)
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Recurrence Rates after Surgery.
Ballo 1999 30% @ 5 years
Sorensen 2002 73% @ 5 years
Phillips 2004 19.3% @ 3 years
Nyttens 2000 39%
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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%
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Recurrence rate after Excision
0
10
20
30
40
50
60
70
1stexcision
2ndexcision
3rdexcision
4thexcision
5thexcision
recurrencerate %
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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
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Does the Margin of Excision Influence Recurrence?
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Recurrence and Margins
Margin Number of Patients(147)
Number of recurrences (60)
%
Debulking 3 3 100%
Intralesional 79 30 38%
Marginal 55 23 42%
Wide 10 4 40%
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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.
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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?
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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’
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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’
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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
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Radiotherapy
• Alone - 22% local recurrence.• Combined with surgery – 6% local recurrence.
• Complications – fibrosisparaesthesiaoedemafracturelate malignancy
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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)
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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.
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If recurrent and asymptomatic observe.
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• If recurrent and symptomatic - second excision if morbidity low, consider radiotherapy if risk of local recurrence high.
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If progressive and inoperable pharmacological +/- radiotherapy.
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• In selected patients whose only surgical option is amputation … observe.
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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.
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Thank you