jurnal a critical analysis of treatment strategies in desmoids.pptrevisi.ppt

7/27/2019 Jurnal A critical analysis of treatment strategies in desmoids.pptREVISI.ppt

1/26

Dipresentasikan Oleh : dr. Ardean Bernandito

Pembimbing : dr. Kunta Setiaji SpB (K) Onk


2/26

Introduction

Treatment for desmoid tumours (deep fibromatoses) has been

modelled on that for low-grade soft tissue sarcomas.

Desmoids tumours are usually not life-threatening.

Treatment efficacy is difficult to evaluate because of the

unpredictable behaviour of desmoids.

The management of desmoid tumours should be reassessed.

Treatment should be different from that for sarcomas and other

cancers.


3/26

Introduction The role of surgery in treating desmoids has therefore been

questioned.

Some authors now suggest a wait-and-see policy in selectedcases.

The aim of this research is to analyse the natural history of a

series of desmoid tumours identify clinical variables theprognostic factors better treatment outcomes.


4/26

Patients and methods Long-term follow-up (median 123 months) of a series of 106

treated patients with 69 primary and 37 recurrent desmoids, inorder to study natural history and outcome.

Patients treated or followed-up at our centre for desmoidtumours from 1976 - 2007 were included

The Union Internationale Contre le Cancer (UICC) residualdisease classification (R) was used to assess quality of surgery

Last follow-up was December 2007.


5/26

Patient and methods Functional impairment was classified as absent, moderate

(compensated by treatment) or major (disability or pain notcompensated by treatment).

Tumour growth patterns were analysed by calculating :

The intervals between the date of first event or treatmentand date of first recurrence or progression ( time to firstrecurrence);

The intervals between the first and the last tumoural eventsbefore stabilisation or regression ( time to progression);and

The intervals between last progression and final follow-up( stable period).


6/26

Patient characteristics


7/26

Treatment policy During the 32-year period, various treatments were available.

Primary tumours without complementary treatment Surgerywas advised

When the tumours were deemed to be inoperable, a first-linemedical treatment was delivered (SERMs, especially tamoxifen,non-steroidal anti-inflammatory drugs (NSAIDs), inhibitors(antiCox2) in the last decade, imatinib mesylate in the last 3

years, interferon and chemotherapy). Whenever possible,surgery was performed secondarily.

In recurrent tumours, re-excision followed by radiotherapy wasproposed


8/26

Treatments Number of patients : 106 patients

92 patiens ( 63 primary and 29 recurrent ) Excision

14 patiens ( 6 primary and 8 recurrent ) Biopsy /noexcision

23 patients ( 7 initially and 16 after recurence )radiotherapy at a median dose of 50 Gy

11 patients ( 5 primary and 6 recurent ) chemotherapy

44 patients (20 primary and 22 recurrent ) variousmedical treatments


9/26

Outcomes 47 patients remained with some functional

impairment ( 28 Moderate and 19 Major )

At a median follow-up of 123 months (range : 3 - 482

months) : 1 patient had died of treatment consequences

(a postoperative vascular rupture after surgery for arecurrent mesenteric desmoid )

10 had died of other causes (DOC ) 24 patients were alive with disease (AWD)

71 were alive without evidence of disease (NED)


10/26

Result : variabels influencing outcomes Primary vs recurrent desmoids

The female/male ratio was 2.8/1 vs. 1.2/1 in primary vs recurrentpresentations a higher risk of recurrence in men than in

women

Completeness of surgery was better in primary than in recurrentdisease

After complete resection (R0 + R1 + Rx) local recurrence was9/58 in primary disease and 14/25 in recurrent disease

Functional impairment was less frequent after treatment forprimary tumours than for recurrent tumours (32% vs. 68%).

The proportion of surviving patients with no disease/remainingdisease was higher in the primary than in the recurrent group


11/26

Result : Prefential Tumour Locations


12/26

Result : Gender There were 71 women and 35 men, giving a sex ratio 2/1.

Women were younger than men (median age 39 vs 44years), had more primary tumours (72% vs. 51%) and had

tumours preponderantly located in the trunk walls andpelvic girdle

Residual disease (R2 or biopsy only) after surgery inprimary or recurrent disease occurred in 10/71 women and

in 13/35 men. Finally, 55 women remained without evidence of disease

vs 16 men.


13/26

Result : Tumor Growth patterns


14/26

Results : Type of resection


15/26

Results : Type of resection Local recurrence (LR) after complete resection(R0 + R1 +

Rx) occurred in 23/83 patients.

There was LR in 2/22 patients after resection R0 and in

19/56 patients after resection R1 ( p < 0.05 according theChi2 test)

This nearly fourfold difference in LR after resections R0 orR1 did not translate into a significant difference in outcomeat final follow-up with 19/22 and 43/56 patients

NED, respectively. No significant difference in overallsurvival was seen between patients having completeresection (83) or not (23) with 73 and 22 patients alive,respectively


16/26

Results : Number of surgeries


17/26

Results : Radiotherapy

7 patients underwent radiotherapy initially, including 3 afterincomplete surgery 6 of them had a recurrence, 5 patients

were NED, 2 AWD.

Functional impairment 6 patients and was major in 3 of them.

16 patients had radiotherapy at recurrence 6 of them had a recurrence ( 12 patients were NED, 3 AWD and 1 patient

died of other causes) .

Functional impairment 12 patients and was major in 4 of

them. In total, 12/23 patients a recurrence after radiotherapy and

18/ 23 functional impairments consecutive to multipletreatments including radiotherapy.


18/26

Results : FAP

Linked desmoids 3 patients had a confirmed APC gene mutation indicating

an FAP-linked desmoid, 7 patients had unconfirmedsuspicion of FAP and 8 a possible FAP.

There were more mesenteric tumours in the first twogroups (4/10) and a longer median period of tumourprogression (48 months) than in the general population.

Functional impairment occurred in 5/18 patients, similar to

functional results in the general population. Majorfunctional impairments in 4 patients all were due torepetitive treatments and their complication


19/26

Result : Desmoids typically evolved actively over a median period of 3

years, and stabilised thereafter.

Recurrences or progression most commonly occurred between 14and 17 months.

Risk factors for recurrence were presentation (primary vs.recurrent), gender, tumour location and resection margins.However, survival was independent from these factors, withequivalent survival whether resection had been performed ornot.

Tumour control and functional outcome depended on locationand presentation.

Functional impairment was proportional to number ofoperations and whether patients had received radiotherapy.

Recurrences were observed in 12/23 patients after radiotherapy.


20/26

Decision-making acording to tumour

presentation Decisions should be based on the treatment benefit/risk ratio

and on tumour presentation.

Primary lower girdle and trunk wall tumours in women seem tobenefit most from surgery. The recommended surgey full-thickness trunk wall excision with or without mesh repair.

Primary upper girdle and trunk wall tumours wait and seepolicy before deciding on treatment,

When a tumour remains quiescent and asymptomatic surgery may be an option.Painful or rapidly growing tumour medical treatment,

preferably including NSAIDs or antiCox2s


21/26

Locally advanced desmoids and in popliteal andmesenteric locations Surgery should be avoided

Wait-and-see and standard medical treatments should beproposed first.

Apart from recurrent abdominal wall tumours in women,where repeat surgery can be helpful, intensifying treatmentwith repeated surgery or radiotherapy appears deleteriousin recurrent desmoids observation or medicaltreatment.

Location is an essential factor when deciding on treatmentoptions.


22/26

Conclusion : The long-term follow-up in this series suggests that

desmoid tumours stabilise over time and can eventuallyregress in some cases, after an active phase ofdevelopment lasting an average of 3 years.

Surgery could re-activate the tumour process, henceprolonging the active phase.

A wait-and-see policy is the best for evaluating therapidity of growth.

Treatment options should take into account tumour

presentations with a view to relieving symptoms but not toeradicating the tumour at all costs.

Treatment associations and new targeted therapies shouldbe evaluated.


23/26

References


24/26

References


25/26

References


26/26

References

jurnal a critical analysis of treatment strategies in desmoids.pptrevisi.ppt

Documents