Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life
G. Duhil de Bénazé
(1, 2)
,
M. Vigan
(2)
,
N. Corradini
(3, 4)
,
V. Minard-Colin
(5, 6)
,
A. Marie-Cardine
(7)
,
C. Verite
(8)
,
A.S. Defachelles
(9)
,
E. Thebaud
(10)
,
M.P. Castex
(11)
,
N. Sirvent
(12)
,
D. Bodet
(13)
,
L. Mansuy
(14)
,
A. Rome
(15)
,
A. Petit
(16)
,
D. Plantaz
(17)
,
A. Jourdain
(18)
,
P. Mary
(16)
,
M. Carton
(2)
,
D. Orbach
(2)
1
CHU -
Hôpital Archet 2 [Nice]
2 Institut Curie [Paris]
3 IHOPe - Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL]
4 Centre régional de lutte contre le cancer
5 IGR - Institut Gustave Roussy
6 Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy]
7 CHU Rouen
8 CHU Bordeaux
9 UNICANCER/Lille - Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille]
10 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
11 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
12 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
13 CHU Caen
14 CHU Nancy - Centre Hospitalier Universitaire de Nancy
15 CHU Marseille
16 CHU Trousseau [APHP]
17 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
18 CHU Trousseau [Tours]
2 Institut Curie [Paris]
3 IHOPe - Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL]
4 Centre régional de lutte contre le cancer
5 IGR - Institut Gustave Roussy
6 Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy]
7 CHU Rouen
8 CHU Bordeaux
9 UNICANCER/Lille - Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille]
10 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
11 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
12 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
13 CHU Caen
14 CHU Nancy - Centre Hospitalier Universitaire de Nancy
15 CHU Marseille
16 CHU Trousseau [APHP]
17 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
18 CHU Trousseau [Tours]
G. Duhil de Bénazé
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Résumé
Background: With recent conservative strategies, prognosis of patients with desmoid-type fibromatosis (DTF) is about function preservation. We analyzed the long-term quality of life (QoL) of pediatric patients with DTF.Methods: All French young patients (<21years) treated between 2005 and 2016 for a DTF in the EpSSG NRSTS-05 study were analyzed. A first wait-and-see strategy was recommended. Patients' QoL was analyzed with the internationally validated Child Health Questionnaire (CHQ). We focused on the relevant subscales scores: physical functioning (PF), role social limitations physical (RP), bodily pain (BP), general health perception (GH) and physical (PhS) and psychosocial (PsS) summary measures.Results: Among the 81 patients, 52 families answered the CHQ (median delay since diagnosis = 6.2years; min2.2-max13.3 years). Median age at diagnosis was 11.5 years. Primary site: limbs (52%), head/neck (27%), or trunk (21%). Five year-Progression Free Survival was 39.1% (95%CI: 27.7-50.5%). As initial management for these 52 patients, 30 patients were first observed (57%), 13 had surgery (25%) and 9 received chemotherapy (18%). Total burden of therapy was exclusive surgery (9pts/18%), exclusive chemotherapy (18pts/35%), surgery + chemotherapy (13pts/25%), chemotherapy + radiotherapy (1 pt), surgery + chemotherapy + radiotherapy (1 pt), wait and see (10 pt). Regarding the parent forms, patients have significant lower PF (86.0vs.96.1; p = 0.03), RP (82.0vs.93.6; p = 0.04), GH (60vs.73; p < 0.005) and PhS (46.2 vs.53; p = 0.02) scores compared to healthy population. Comparison of QoL subscales scores according to initial strategy (wait-and-see vs.surgery/chemotherapy) did not reveal any difference (PF = 87.3vs.84.9; p = 0.80/RP = 83.4vs.78.7; p = 0.72/BP = 78.9vs.78.2; p = 0.95/GH = 59.7vs60; p = 0.97). Similar results were found using the children or adult forms.Conclusions: Initial wait-and-see strategy does not affect long term functional impairment.
Domaines
Sciences du Vivant [q-bio]Format du dépôt | Fichier |
---|---|
Type de dépôt | Article dans une revue |
Titre |
en
Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life
|
Résumé |
en
Background: With recent conservative strategies, prognosis of patients with desmoid-type fibromatosis (DTF) is about function preservation. We analyzed the long-term quality of life (QoL) of pediatric patients with DTF.Methods: All French young patients (<21years) treated between 2005 and 2016 for a DTF in the EpSSG NRSTS-05 study were analyzed. A first wait-and-see strategy was recommended. Patients' QoL was analyzed with the internationally validated Child Health Questionnaire (CHQ). We focused on the relevant subscales scores: physical functioning (PF), role social limitations physical (RP), bodily pain (BP), general health perception (GH) and physical (PhS) and psychosocial (PsS) summary measures.Results: Among the 81 patients, 52 families answered the CHQ (median delay since diagnosis = 6.2years; min2.2-max13.3 years). Median age at diagnosis was 11.5 years. Primary site: limbs (52%), head/neck (27%), or trunk (21%). Five year-Progression Free Survival was 39.1% (95%CI: 27.7-50.5%). As initial management for these 52 patients, 30 patients were first observed (57%), 13 had surgery (25%) and 9 received chemotherapy (18%). Total burden of therapy was exclusive surgery (9pts/18%), exclusive chemotherapy (18pts/35%), surgery + chemotherapy (13pts/25%), chemotherapy + radiotherapy (1 pt), surgery + chemotherapy + radiotherapy (1 pt), wait and see (10 pt). Regarding the parent forms, patients have significant lower PF (86.0vs.96.1; p = 0.03), RP (82.0vs.93.6; p = 0.04), GH (60vs.73; p < 0.005) and PhS (46.2 vs.53; p = 0.02) scores compared to healthy population. Comparison of QoL subscales scores according to initial strategy (wait-and-see vs.surgery/chemotherapy) did not reveal any difference (PF = 87.3vs.84.9; p = 0.80/RP = 83.4vs.78.7; p = 0.72/BP = 78.9vs.78.2; p = 0.95/GH = 59.7vs60; p = 0.97). Similar results were found using the children or adult forms.Conclusions: Initial wait-and-see strategy does not affect long term functional impairment.
|
Auteur(s) |
G. Duhil de Bénazé
1, 2
, M. Vigan
2
, N. Corradini
3, 4
, V. Minard-Colin
5, 6
, A. Marie-Cardine
7
, C. Verite
8
, A.S. Defachelles
9
, E. Thebaud
10
, M.P. Castex
11
, N. Sirvent
12
, D. Bodet
13
, L. Mansuy
14
, A. Rome
15
, A. Petit
16
, D. Plantaz
17
, A. Jourdain
18
, P. Mary
16
, M. Carton
2
, D. Orbach
2
1
CHU -
Hôpital Archet 2 [Nice]
( 337250 )
- CS 23079
06202 Nice cedex 3
- France
2
Institut Curie [Paris]
( 301888 )
- 26 rue d'Ulm - 75248 Paris
- France
3
IHOPe -
Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL]
( 348446 )
- 1 place Professeur Joseph Renault
69008 Lyon
- France
4
Centre régional de lutte contre le cancer
( 300544 )
-
- France
5
IGR -
Institut Gustave Roussy
( 156104 )
- 114, rue Édouard-Vaillant 94805 Villejuif Cedex -France
- France
6
Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy]
( 456891 )
- France
7
CHU Rouen
( 300147 )
- 1 rue de Germont 76031 Rouen cedex
- France
8
CHU Bordeaux
( 300122 )
- 12 rue Dubernat
33400 Talence
- France
9
UNICANCER/Lille -
Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille]
( 301886 )
- 3, rue Frédéric Combemale, 59020 Lille
- France
10
CHU Nantes -
Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
( 474972 )
- 1 Place Alexis-Ricordeau, 44000 Nantes
- France
11
CHU Toulouse -
Centre Hospitalier Universitaire de Toulouse
( 300075 )
- regroupe les hôpitaux Purpan, Rangueil, Pierre-Paul Riquet, Larey, Paule de Viguier, Hôtel Dieu Saint-Jacques, La Grave, etc.
- France
12
CHRU Montpellier -
Centre Hospitalier Régional Universitaire [Montpellier]
( 258773 )
- 191 avenue du doyen Gaston Giraud 34295 Montpellier
- France
13
CHU Caen
( 300185 )
- Avenue de la Côte de Nacre 14000 Caen
- France
14
CHU Nancy -
Centre Hospitalier Universitaire de Nancy
( 537110 )
- Siège social : 29 avenue du Maréchal de Lattre de Tassigny, CO 60034, 54035 NANCY CEDEX Hôpital Central : 29 Avenue du Maréchal de Lattre de Tassigny, 54035 Nancy Hôpitaux de Brabois / Enfants : Rue du Morvan, 54500 Vandœuvre-lès-Nancy
- France
15
CHU Marseille
( 301298 )
-
- France
16
CHU Trousseau [APHP]
( 360410 )
- 26 Avenue du Dr Arnold Netter, 75012 Paris
- France
17
CHUGA -
Centre Hospitalier Universitaire [CHU Grenoble]
( 257328 )
- CS 10217
38043 Grenoble cedex 9
- France
18
CHU Trousseau [Tours]
( 12089 )
- CHU Trousseau, Unité de Médecine et Physiologie Spatiale, F-37044 Tours
- France
|
Licence |
Paternité - Pas d'utilisation commerciale
|
Langue du document |
Anglais
|
Nom de la revue |
|
Vulgarisation |
Non
|
Comité de lecture |
Oui
|
Audience |
Internationale
|
Date de publication |
2020-07
|
Volume |
46
|
Numéro |
7
|
Page/Identifiant |
1294-1300
|
Domaine(s) |
|
Mots-clés |
en
Child health questionnaire, Children, Conservative strategy, Desmoid-type tumor, Quality of life
|
DOI | 10.1016/j.ejso.2020.02.028 |
PII | S0748-7983(20)30130-X |
Pubmed Id | 32173177 |
Origine :
Fichiers produits par l'(les) auteur(s)
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