The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series

The Lancet Child & Adolescent Health
Daniel OrbachAndrea Ferrari

Abstract

In 2005, the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) proposed a conservative treatment algorithm-consisting of an initial wait-and-see strategy, non-mutilating surgery, and minimal-morbidity chemotherapy (in the case of tumour progression)-for paediatric patients with desmoid-type fibromatosis. We aimed to investigate the outcomes of this algorithm. In this case series, patients (<25 years) with desmoid-type fibromatosis from 57 centres in eight countries were prospectively registered through a web-based system. Diagnosis was based on histological analysis of the tumour specimen after biopsy or surgery, and we classified patients by tumour site, clinical stage (TNM system), and post-surgical stage (Intergroup Rhabdomyosarcoma Study system). Progression-free survival was defined as the time from diagnosis until disease progression (clinical or radiological progressive disease, relapse, or death from any cause). From Oct 1, 2005, to July 31, 2016, 173 patients (median age 11·4 years [IQR 4·0-14·1], 88 [51%] male patients) were registered. After excluding patients with missing data, 54 (35%) patients had no immediate therapy (wait-and-see strategy), 47 (31%) had immediate surgery, and 53 (34%) had immediate chem...Continue Reading

Citations

Jan 3, 2021·Annals of Surgical Oncology·Marco FioreBernd Kasper
Mar 3, 2021·Current Treatment Options in Oncology·Wen Shen LooiMichael S Rutenberg
Mar 17, 2021·British Journal of Cancer·Monika Sparber-SauerAndrea Ferrari
Feb 1, 2020·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·UNKNOWN Desmoid Tumor Working Group
May 7, 2019·International Journal of Pediatric Otorhinolaryngology·Antoine PaulDaniel Orbach
Mar 17, 2020·European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology·G Duhil de BénazéD Orbach
Dec 17, 2019·European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology·C CollignonD Orbach
Sep 21, 2020·Bulletin du cancer·Charlotte CollignonDaniel Orbach
Oct 7, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Nina PerošaRoman Bošnjak

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