Radiotherapy in desmoid tumors : Treatment response, local control, and analysis of local failures.

We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012. Radiotherapy is a valuable option for treating desmoid tumors. Radiotherapy dose appears to be significantly associated to local control.

The targeted nuclease revolution (TALENs, CRISPR/Cas9) now allows Xenopus researchers to rapidly generate custom on-demand genetic knockout models. These novel methods to perform reverse genetics are unprecedented and are fueling a wide array of human disease models within the aquatic diploid model organism Xenopus tropicalis (X. tropicalis). This emerging technology review focuses on the tools to rapidly generate genetically engineered X. tropicalis models (GEXM), with a focus on establishment of genuine genetic and clinically relevant cancer models.

A 49-year-old Pakistani male presented with “heaviness” in his chest. Chest radiograph and computed tomography (CT) confirmed a massive left-sided pleural-based opacity. Histopathology and immunohistochemistry of the resected tumour were consistent for a desmoid tumour. He was followed up for 9 months with no evidence of tumour recurrence. Predominantly pleural-based desmoid tumour is rare but should be included in the differential diagnosis of spindle cell tumours.

Efficacy of liposomal doxorubicin (LD) in treating desmoid fibromatosis (DF) in children has not been well evaluated. This retrospective case series examines five children with progressive DF, treated with LD. We report progression-free intervals (PFIs) and radiographic as well as clinical responses for each medication received. LD was well tolerated, with an average 4.5% reduction in tumor size and median PFI of 29 months. Treatment with LD conferred the longest PFI of all medical therapies pursued. Thus, LD is an important treatment option for DF in pediatrics.

Efficacy of liposomal doxorubicin (LD) in treating desmoid fibromatosis (DF) in children has not been well evaluated. This retrospective case series examines five children with progressive DF, treated with LD. We report progression-free intervals (PFIs) and radiographic as well as clinical responses for each medication received. LD was well tolerated, with an average 4.5% reduction in tumor size and median PFI of 29 months. Treatment with LD conferred the longest PFI of all medical therapies pursued. Thus, LD is an important treatment option for DF in pediatrics.

Desmoid tumours of the head and neck in children are more aggressive than their adult counterparts and are associated with high morbidity and mortality and a high recurrence rate. CT and MRI imaging assessment should preferably be performed before biopsy. External beam radiotherapy must be avoided in children as it is less effective than in adults, and is responsible for long-term cosmetic and functional sequelae and even a risk of second tumours. Treatment is surgical whenever radical resection is possible. In patients presenting an excessive risk of morbidity and mortality, chemotherapy devoid of long-term adverse effects (such as methotrexate in combination with a Vinca alkaloid) can be proposed. Long-term follow-up must be ensured due to the risk of recurrence.