Abstract
The desmoid tumor is a locally aggressive proliferative disease within the family of soft-tissue sarcomas. Despite its relatively good prognosis, the clinical management of desmoid tumors requires constant multidisciplinary evaluation due to its highly variable clinical behavior. Recently, active surveillance has being regarded as the appropriate strategy at diagnosis, as indolent persistence or spontaneous regressions are not uncommon. Here, we review the most recent advances in desmoid tumor therapy, including low-dose chemotherapy and treatment with tyrosine kinase inhibitors. We also explore the recent improvements in our knowledge of the molecular biology of this disease, which are leading to clinical trials with targeted agents.
Highlights
Desmoid tumor(s) (DT)— known as desmoid-type fibromatosis—is a monoclonal, non-metastasizing, locally aggressive, often multifocal, fibroblastic proliferative disease within the family of soft-tissue sarcomas [1]
I study of PF-03084014, impressive activity was observed against DT, with five out of seven patients (71%) experiencing partial response and the other two (29%) having stable disease [76], supporting a potential role of Gamma-secretase inhibitors (GSIs) in the medical therapy of refractory DT
The proven efficacy of low-dose chemotherapy and—hopefully and even more so—tyrosine kinase inhibitor (TKI) and other targeted agents will further confirm the prominent role of medical therapy in the management of DT
Summary
Desmoid tumor(s) (DT)— known as desmoid-type fibromatosis—is a monoclonal, non-metastasizing, locally aggressive, often multifocal, fibroblastic proliferative disease within the family of soft-tissue sarcomas [1]. 35 years, mainly women at reproductive age. They can arise in any body district: they are more commonly extra-abdominal (in the abdominal wall, limbs and girdles); less frequently, they are found intra-abdominally, often in the mesentery [2]. Two main categories of DT are recognized: sporadic DT and familial adenomatous polyposis (FAP)-associated DT. DT are diagnosed in about 10–15% of patients affected by FAP syndrome, a risk 800- to 1000-fold higher than in the general population [3]. Sporadic and FAP-associated DT share morphologic and biologic characteristics, . FAP-associated DT are largely more often intra-abdominal compared to sporadic DT [5].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
