Clinical trials in desmoid-type fibromatosis in children and adults: A systematic review.
Pediatr Blood Cancer
; 69(9): e29831, 2022 09.
Article
em En
| MEDLINE
| ID: mdl-35714333
ABSTRACT
Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm, which occurs in children and adults, with a peak incidence in young adults. For the majority of the patients, DTF is a chronic and symptomatic disease, which affects health-related quality of life. Systemic treatment regimens tend to differ for patients treated by pediatric oncologists compared to medical oncologists. This systematic review identified 14 clinical trials in children and adults with DTF. Tumor response and progression-free survival rates varied widely between studies and study populations. Treatment choices for patients with DTF are based on a paucity of (randomized) trials. Treatment principles of DTF are similar in pediatric and adult oncology, but the treatment itself is different. This seems mostly driven by a lack of tyrosine kinase inhibitor (TKI) accessibility in pediatric oncology. An insufficient number of studies examined patient-reported outcomes, which are extremely important for patients with a chronic disease like DTF.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias de Tecidos Moles
/
Fibromatose Agressiva
Tipo de estudo:
Clinical_trials
/
Systematic_reviews
Limite:
Adult
/
Child
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Humans
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article