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The Desmoid Tumor Research Foundation

February 2020

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

Author Information

Duhil de Benaze G(1), Vigan M(2), Corradini N(3), Minard-Colin V(4), Marie-Cardine A(5), Verite C(6), Defachelles AS(7), Thebaud E(8), Castex MP(9), Sirvent N(10), Bodet D(11), Mansuy L(12), Rome A(13), Petit A(14), Plantaz D(15), Jourdain A(16), Mary P(17), Carton M(2), Orbach D(18).

(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. (2)Institut Curie- PSL Research University, Biometry Unit, Paris, France. (3)IHOPe, Centre Regional de Lutte Contre le Cancer- Centre Leon Berard, 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 Pediatrique, 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-Hopitaux 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.

Publication

Eur J Surg Oncol

Summary

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. 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. 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. Initial wait-and-see strategy does not affect long term functional impairment.

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