Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life

G Duhil de Bénazé; M Vigan; N Corradini; V Minard-Colin; A Marie-Cardine; C Verite; A S Defachelles; E Thebaud; M P Castex; N Sirvent; D Bodet; L Mansuy; A Rome; A Petit; D Plantaz; A Jourdain; P Mary; M Carton; D Orbach

doi:10.1016/j.ejso.2020.02.028

Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life

Eur J Surg Oncol. 2020 Jul;46(7):1294-1300. doi: 10.1016/j.ejso.2020.02.028. Epub 2020 Feb 29.

Authors

G Duhil de Bénazé¹, M Vigan², N Corradini³, V Minard-Colin⁴, A Marie-Cardine⁵, C Verite⁶, A S Defachelles⁷, E Thebaud⁸, M P Castex⁹, N Sirvent¹⁰, D Bodet¹¹, L Mansuy¹², A Rome¹³, A Petit¹⁴, D Plantaz¹⁵, A Jourdain¹⁶, P Mary¹⁷, M Carton², D Orbach¹⁸

Affiliations

¹ University Hospital of Nice- Archet 2, Department of Pediatric Hematology-Oncology, Nice, France; Institut Curie, SIREDO Oncology Center Care- Innovation and Research for Children and AYA with Cancer- PSL Research University, Paris, France. Electronic address: duhildebenaze.g@chu-nice.fr.
² Institut Curie- PSL Research University, Biometry Unit, Paris, France.
³ IHOPe, Centre Régional de Lutte Contre le Cancer- Centre Léon Bérard, Lyon, France.
⁴ Gustave Roussy Cancer Campus, Department of Children and Adolescents Oncology, Villejuif, France.
⁵ Rouen University Hospital, Department of Pediatric Hematology-Oncology, Rouen, France.
⁶ Bordeaux University Hospital, Pediatric Hematology Department, Bordeaux, France.
⁷ Centre Oscar Lambret, Service D'Oncologie Pédiatrique, Lille, France.
⁸ University Hospital Nantes, Pediatric Oncology Department, Nantes, France.
⁹ Children's Hospital of Toulouse- CHU Toulouse, Pediatric Hemato-oncology Department, Toulouse, France.
¹⁰ University Hospital of Montpellier, Department of Pediatric Onco-Hematology, Montpellier, France.
¹¹ Caen University Hospital, Department of Pediatric Hematology and Oncology, Caen, France.
¹² Children's University Hospital, Department of Pediatric Hematology and Oncology, Nancy, France.
¹³ Centre Hospitalier Universitaire, Department of Pediatric Oncology, Marseille, France.
¹⁴ Assistance Publique-Hôpitaux de Paris AP-HP- GH HUEP- Armand Trousseau Hospital, Department of Pediatric Hematology and Oncology, Paris, France.
¹⁵ University Hospital Centre of Grenoble, Department of Pediatric Hematology-Oncology, Grenoble, France.
¹⁶ CHU Tours, Department of Pediatric Oncology and Hematology, Tours, France.
¹⁷ Pediatric Orthopedics Department, Armand Trousseau Hospital, Paris, France.
¹⁸ Institut Curie, SIREDO Oncology Center Care- Innovation and Research for Children and AYA with Cancer- PSL Research University, Paris, France.

PMID: 32173177
DOI: 10.1016/j.ejso.2020.02.028

Abstract

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.

Keywords: Child health questionnaire; Children; Conservative strategy; Desmoid-type tumor; Quality of life.

Publication types

Comparative Study
Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Antineoplastic Agents / therapeutic use
Cancer Pain / etiology
Child
Child, Preschool
Combined Modality Therapy
Female
Fibromatosis, Aggressive / complications
Fibromatosis, Aggressive / therapy*
Health Status
Humans
Infant
Male
Physical Functional Performance
Progression-Free Survival
Quality of Life*
Radiotherapy
Social Participation
Surgical Procedures, Operative
Surveys and Questionnaires
Watchful Waiting*

Substances

Antineoplastic Agents