This trial is evaluating whether Selinexor will improve 2 primary outcomes and 7 secondary outcomes in patients with Sarcoma. Measurement will happen over the course of Up to 14 days after completion of study treatment.
This trial requires 56 total participants across 2 different treatment groups
This trial involves 2 different treatments. Selinexor is the primary treatment being studied. Participants will be divided into 2 treatment groups. There is no placebo group. The treatments being tested are in Phase 1 and are in the first stage of evaluation with people.
In this case, we did not detect any clinical trial involving selinexor. The development of novel small molecule agents for the treatment of cancer warrants constant scrutiny.
When neurofibrosarcoma is localized, surgical excision may be adequate, as chemotherapy or radiation therapy are of little benefit. In case of metastasis, first-line chemotherapy improves survival. The role of radiation therapy and chemotherapy are undefined in this setting.
Neurofibrosarcoma is a rare but fatal cancer that has been found in many parts of the body, including the face where it most commonly appears. It was first recognized as such in 1982 in an 11-year-old female. This report discusses the diagnosis, treatment and prognosis of this tumor, with particular reference to its appearance in the face.
Neoplastic neurofibroma exhibits a wide range of clinical manifestations, many of which are nonspecific. In this series, signs involving cranial nerves were most common. Lesion location, size, and presence of neurologic symptoms should influence treatment. No clinical finding is 100% sensitive or specific for neurofibrosarcoma.
While the risk of relapse remains high, we believe that with chemotherapy, adjuvant irradiation, and surgical resection the mortality will be less than 5% and 10%, and the relapse rate less than 50%, 5 years after radical resection. We believe that surgery alone is not enough. Because of early relapse, surgery +/- chemotherapy plus radiotherapy +/- adjuvant treatment with targeted therapeutics is most likely to prolong survival and minimize relapse.
Genetic alterations in neurofibrosarcoma samples from different races and geographic locations are more common in blacks than in Caucasians, but all show a higher rate of chromosome instability, higher mutational load, and a higher rate of copy number variation compared to sporadic cases. Thus, these genetic abnormalities are likely to contribute to neurofibrosarcoma tumorigenesis. The high rate of chromosome instability found in all three forms of neurofibrosarcoma may be associated with reduced expression of DNA repair genes.
Between 2008 and 2011, there were 459 new cases of neurofibrosarcoma. The average age of diagnosis was 44 years. The overall five-year mortality rate was 6.8%. The most common site for neurofibrosarcoma was the brain. Neurofibrosarcoma comprised 27.8% of all tumors seen at MD Anderson Cancer Center between 2008 and 2011, and 22.3% of all tumors seen at Memorial Sloan-Kettering Cancer Center between 2008 and 2011. The average age of presentation was 43 years. The five-year survival rate was 5.6%.
In this large cohort (3,818 cases) from a single center, the mean age at diagnosis was 44.4 yr (SD ± 10.5 yr), with the majority of cases diagnosed at ages 36 through 64 yr (76.8%) and less than 5% of cases before age 20 yr. Inclusion criteria varied, but a majority of cases were children presenting to the clinic under the age of 20 yr, with a mean age at diagnosis of 8.8 yr. A large number of patients presented with multiple primary tumors, and these included tumors of most parts of the body with the most frequent sites being the head, eye(s)/nose, trunk, and legs/shins.
Selexpon significantly improved pain-related disability and QoL for the majority of patients with neurofibrosarcoma who were previously receiving chemotherapy. This finding may have important clinical implications for the follow-up of patients treated as inpatient.
Despite this aggressive tumor, many adults (27%) and children (10%) will be treated only with standard of care medication. We did not observe any significant difference in survival rate, tumor control, or overall survival for either subgroups analyzed with or without clinical trial enrollment. The question of whether clinical trials are warranted remains up for debate. We found no definitive evidence of benefit of clinical trials for neurofibrosarcomas, especially in adults. However, we did recommend prospective clinical trials for children with a new tumor or for children with more than one prior surgery for a resectable tumor.
Recent findings confirms that patients with neurofibrosarcoma rarely get metastatic tumour at a primary tumour site. Patients with a local invasion, but no involvement of surrounding soft tissue, have a significantly poorer prognosis. However, this was based on a single-centre study.
It is very unlikely to develop neurofibrosarcoma after 10 years following diagnosis of neurofibromatosis type I, type II, or Type III. Although patients should remain vigilant for secondary cancers (especially neurofibrosarcoma), there is low risk of developing neurofibrosarcoma after 10 years. This information should help them decide if they need to have a more intense followup program.