Two cases are described of hepatic fibropolycystic disease (Brunt, 1973). Both had neonatal pyloric stenosis, one craniostenosis and the other acromegaly and neuro-fibromatosis.

A rare case of isolated giant mesenteric fibromatosis is presented. The tumor originated from the fibrous mesenteric tissue. The patient underwent laparotomy because of abdominal discomfort and sub-occlusive symptoms due to the giant mass. Differential diagnosis of mesenteric masses is discussed and the Authors also review the literature concerning this rare disease.

Our understanding of the process of contracture of the fingers is based on extrinsic and intrinsic theories. Yet there has always been an inherent contradiction in these concepts. Additionally, nearly all of these theories proceed from the assumption of a contractio digitorum and this assumption is reflected in basic research as well. The author presents a concept, which describes the finger co...

Matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs) regulate the degradation of extracellular matrix components and play important roles in the progression of select neoplastic processes. The locally invasive soft tissue tumor, aggressive fibromatosis (also called desmoid tumor), is caused by mutations resulting in beta-catenin-mediated T-cell factor (tcf)-dependent transcrip...

10518 Background: We present updated results from a previously reported phase II trial assessing clinical efficacy of imatinib in progressive or recurrent aggressive fibromatosis (Fayette et al. ASCO 2007) Methods: Patients with aggressive fibromatosis not amenable to radiotherapy or non-mutilating surgery were eligible and received imatinib 400mg daily (increased to 800mg daily in case of prog...