Tailoring Desmoid Treatment

Abstract

Desmoid-type fibromatosis (DF) is a rare disease, also known as aggressive fibromatosis or desmoid tumor. DF is characterized by infiltrative growth with a tendency toward local recurrence but an inability to metastasize. The incidence of DF is low. The pathogenesis of this rare disease is not fully understood. Genetic mutations seem to be major factor, in addition to hormonal influences. The treatment options for DF are dependent on the localization of the tumor. The classical treatment of extra-abdominal and abdominal DF implies primary surgery, with radiotherapy on indication. During the past decade, this advice switched based on accumulating evidence to active surveillance and in case of progressive disease surgery, radiotherapy or systemic treatment based on localization. The natural behavior of DF ranges from spontaneous regression to rapid invasive growth. It is challenging to predict the behavior of a tumor in an individual. This thesis is about tailoring the treatment of DF. Current medical care is patient oriented, with a trend toward individualized treatment strategies. This thesis focusses on changes in epidemiology, treatment modalities and the ability to predict tumor behavior