Mastoraki A (1), Schizas D (2), Vassiliu S (2), Saliaris K (2), Giagkos G (2), Theochari M (3), Vergadis C (4), Tolia M (5), Vassiliu P (6), Felekouras E (2).
(1) First Dept of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece. Electronic address: dr_kamast@yahoo.gr. (2) First Dept of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece. (3) First Dept of Internal Medicine, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece. (4) Dept of Radiology, Laikon General Hospital, Athens, Greece. (5) Dept of Radiotherapy, School of Medicine, University of Crete, Heraklion, Greece. (6) Fourth Dept of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece.
Publication
Surg Oncol
Summary
Desmoid-type fibromatosis (DF) is a distinctly rare condition, mostly of younger adults, characterized by the development of locally aggressive tumors of mesenchymal origin. Desmoid tumors (DT) arise either sporadically or in association with FAP (familial adenomatous polyposis), although certain risk factors have also been identified, including pregnancy and antecedent surgical trauma. They can emerge from any connective tissue including muscle, fascia and aponeurosis and are therefore classified, according to location, as intra-abdominal, of the abdominal wall and extra-abdominal. Despite the lack of metastasizing potential, the course can be unpredictable. Various mutations of APC and ?-catenin genes, among others, play a catalytic role in the pathogenesis of this neoplastic entity. Surgery has lost its traditional role as first line treatment of the disease and several other treatment methods are being considered. Cytotoxic chemotherapy, non-cytotoxic systemic therapy and targeted therapy have been revealed as part of different treatment regimens. Recent progress regarding DT biology and molecular pathways has led to the development of promising novel biological agents. In any case, a multidisciplinary approach is required and is gradually employed, espe-cially in intra-abdominal DTs. In this review, we aim to present current knowledge on DF and summarize current treatment regimens as well as their effectiveness, with emphasis on the intraperitoneal type of DT.
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