Finding the common thread in rare diseases
Menée à partir de données portant sur 63 patients atteints d'un léiomyosarcome vasculaire traité par résection (âge médian au diagnostic : 58 ans ; 65 % de femmes), cette étude évalue le taux de survie spécifique à 5 ans, les délais de récidive locale ou distante ainsi que l'association entre l'expression tumorale de biomarqueurs (bêta-caténine, récepteur du facteur de croissance apparenté à l'insuline, ...) et la survie spécifique des patients
Soft-tissue sarcomas are extremely rare, with an estimated 11 930 new cases in the United States in 2015, representing less than 1% of all new cancer cases.1 They are a heterogeneous group of diseases with more than 80 different histologic types and subtypes that have widely different clinical behaviors and outcomes.2 Leiomyosarcomas involving the inferior vena cava (IVC) are the rarest of the rare; even at tertiary referral centers, no more than approximately 1 case per year is seen.3 In this issue of JAMA Surgery, Roland et al4 describe one of the largest single-institution reviews of leiomyosarcomas arising from the IVC and other vessels, adding to our very limited understanding of these rare diseases.
JAMA Surgery , commentaire, 2015