Aggressive fibromatoses or desmoid tumor (DT) is a very rare soft tissue neoplasms with incidence 2 – 4 cases per billion every year [1]. Fibromatoses is fibroblast origin benign tumor lesion, locally invasive but never metastatic and with high recurrence rate [2]. There are two types of DT – superficial and the deep group. The deep group is divided into extra abdominal, intra-abdominal and abd...

BACKGROUND Fibromatosis or desmoid tumor covers a broad spectrum of benign fibrous tissue proliferations. It is characterized by infiltrative growth and a tendency towards recurrence; however, unlike sarcoma, it never metastasizes. CASE PRESENTATION We report on a case of extraabdominal fibromatosis originating from the retroperitoneal space in a 43-year-old woman. Seven years earlier she had...

A phase I trial of PF-03084014, an oral reversible γ-secretase inhibitor, in solid tumor malignancies shows drug tolerability in patients. Evidence of Notch pathway inhibition was demonstrated in peripheral blood. A surprisingly high rate of response was seen in desmoid tumors, a rare but sometimes locally aggressive sarcoma.