Nuclear expression of β-catenin predicts the efficacy of meloxicam treatment for patients with sporadic desmoid tumors.

This study aimed to determine the prevalence of β-catenin nuclear positivity as a prognostic factor in patients with desmoid tumors (DTs) treated with meloxicam, a cyclooxygenase-2 (COX-2) selective inhibitor. Between 2003 and 2012, consecutive 31 patients with extraabdominal, sporadic DTs were prospectively treated with meloxicam as a systemic medical therapy. The nuclear expression of β-catenin can predict the efficacy of meloxicam treatment for patients with sporadic Dts.

Despite high rates of partial ablation, percutaneous image-guided cryoablation appears to be safe and effective for local control for patients with inoperable extra-abdominal desmoid tumors.

Several studies have reported that imatinib may induce tumor responses and prolonged disease stabilization in aggressive fibromatosis (AF). This effect may relate to the PDGFR-β pathway and KIT mutations. The aim of this prospective multicenter uncontrolled study was to evaluate the efficacy and safety of sunitinib in patients with advanced AF. Most of the primary sites were intra-abdominal (12, 63.2 %), and AF associated with familial adenomatous polyposis in ten patients (52.6 %). Grade 3 or 4 adverse events of sunitinib that occurred in >5 % of patients were neutropenia (33.3 %), diarrhea (5.3 %), and hand-foot syndrome (5.3 %). In 3 of 12 patients with mesenteric AF, mesenteric mass bleeding (n = 1), bowel perforation (n = 1), and bowel fistula (n = 1) with tumor mass necrosis were observed early during sunitinib treatment. Therefore, sunitinib showed potential antitumor activity and may be useful for the management of non-mesenteric AF.

This study aimed to investigate the expression profile of cyclooxygenase-2 (COX-2) in desmoid tumor specimens and to evaluate the correlation of intratumoral COX-2 expression with pain status. Our results indicated that COX-2 secretion from mast cells modulates desmoid tumor-associated pain. In addition, mast cells may at least in part contribute to the pathogenesis of desmoid tumors.

The aim of this study was to evaluate the results of surgical treatment of aggressive fibromatosis and the effects of prognostic factors on recurrence. Forty patients (24 female, 16 male; average age: 31.2 years) diagnosed with aggressive fibromatosis were evaluated retrospectively. Prognostic factors such as age, gender, localization, admission status, compartment status, surgical margin, tumor size and adjuvant radiotherapy were evaluated. There was no statistically significant relation between prognostic factors and recurrence. Clinical results of the patients receiving adjuvant radiotherapy were more satisfactory. Conclusion: Adjuvant radiotherapy administration appears to be a rational treatment method instead of sacrificing function to achieve wide surgical margins.

Very late effects of radiotherapy occur within decades after the initial exposure. Their development is induced by low doses of ionizing radiation (from 4 Gy per radiation series) and their clinical manifestations are difficult to distinguish from other independent diseases diagnosed in individuals not formerly treated with radiation. A long time period from the exposure confounds any causal relationships between radiation and adverse events. Still, these side effects not only reduce the patients quality of life but also lead to an early morbidity and mortality, hence generating significant costs in health care and social systems. This article summarizes findings about the most common very late consequences of radiotherapy, which include cardiotoxicity, CNS toxicity, pneumotoxicity, renal toxicity and secondary malignancies. This issue is crucial in the group of children cancer patients, malignant lymphomas, testicular tumors and CNS tumors. Generally, the risk of very late effects of radiotherapy (RT) should be considered in all patients irradiated at a relatively early age with a high chance of long/ term survival. The risk of very late effects of RT is also one of the key limiting factors in the use of RT in the treatment of paients with benign lesions with longterm survival expectation, e. g. in paients with glomus tumors, neurofibromas, desmoid tumors or hemangiomas or other benign lesions (arterio venous malformations). Currently, the only known prevention of these very late adverse effects is to minimize the dose to critical structures to the lowest achievable level