Chordoma

Researchers are evaluating the use of Gallium-68-DOTATATE PETMRI in patients with somatostatin receptor-positive SSTR-positive central nervous system CNS tumors, mainly focusing on meningioma but also including other tumor types such as esthesioneuroblastoma, hemangioblastoma, medulloblastoma, paraganglioma, pituitary adenoma, and SSTR-positive systemic cancers metastatic to the brain. The study aims to assess the diagnostic usefulness of this imaging technique, especially in distinguishing tumor recurrence from post-treatment changes, with a particular interest in cases where tumor location limits surgical removal or where patients have higher-grade disease or previous radiation treatment. This is a Phase 4 interventional study sponsored by Weill Medical College of Cornell University.

Spinal cord gliomas are rare malignant tumors that develop within the spinal cord, often appearing around age 35 and slightly more common in males. These tumors typically cause symptoms like axial pain and tend to grow in a lateral, infiltrative pattern. Treatments are limited mainly to surgery, and the choice of surgical approach can affect neurological outcomes and quality of life. This research compares two surgical methods to find the best approach for tumor removal and patient recovery. The study compares the dorsolateral sulcus DLS approach and the traditional posterior median sulcus PMS approach for spinal cord tumor removal. The DLS approach is thought to better preserve neurological function by avoiding critical midline structures, while the PMS approach offers direct midline access but risks more damage. Patients with laterally located tumors, confirmed by imaging and pathology, undergo surgery via one of these two methods. Outcomes will be assessed over time to evaluate pain, neurological function, quality of life, tumor removal extent, and survival. Participants will be evaluated before surgery and followed up at 1, 3, 6, and 12 months after surgery with clinical exams, imaging, and questionnaires like the Visual Analog Scale for pain and SF-36 for quality of life. Neurological function is measured using established grading scales, and tumor progression and survival are monitored up to 24 and 36 months, respectively. The study aims to determine which surgical approach best balances effective tumor removal with preservation of neurological health.

Researchers are investigating a treatment for children, adolescents, and young adults with relapsed or refractory solid tumors. The study evaluates the safety and potential survival benefits of an allogeneic stem cell transplant depleted of alphabeta T cells and CD19 B cells combined with zoledronic acid. This approach aims to enhance the immune systems graft versus tumor effect and improve event-free and overall survival rates in these patients. Participants receive a stem cell transplant processed using the Miltenyi CliniMACS Prodigy system to remove specific immune cells. Zoledronic acid is given intravenously on days 28, 56, 84, 112, and 140 post-transplant. The study includes a phase Ib dose-finding portion to determine the maximum tolerated dose of zoledronic acid, followed by a phase II portion where all subjects receive this dose. During the trial, participants are closely monitored through various assessments including disease-free survival and incidence of acute graft-versus-host disease at multiple time points up to two years after transplant. The research team evaluates overall survival rates at 100 days, 1 year, and 2 years post-transplant. The study spans several years, with participants undergoing treatment and follow-up evaluations to track safety and effectiveness.

Researchers are creating a secure electronic health record EHR-based registry to collect clinical and research data on rare diseases, specifically bone and soft tissue tumors. This observational study aims to help identify clinical and molecular features related to prognosis and therapy response, encourage collaboration between institutions, and standardize biospecimen collection for biomarker analysis in patients treated and monitored at Ohio State University. Participants will not receive experimental treatments but will be observed as part of routine care. They will complete quality of life questionnaires, and their medical records will be reviewed. Blood samples may be collected for up to two years to support biomarker research. The study facilitates long-term data collection and collaboration to better understand these rare tumors. During the study, participants will provide information through questionnaires and allow access to their medical records. Researchers will monitor outcomes such as overall survival over five years. The study includes ongoing collection of clinical data and biospecimens to support research. Participation duration varies, and there is no interventional treatment provided as this is an observational registry.

Researchers are evaluating proton therapy in patients with chordoma, chondrosarcoma, Ewing sarcoma, and osteosarcoma who require two radiation beams for treatment. This study aims to compare the toxicity of proton therapy delivered using a single daily beam versus the conventional approach of two daily beams. The latest ProteusOne proton therapy equipment with a compact rotating gantry enables precise targeting of tumors from different angles, potentially reducing treatment side effects. Participants are randomly assigned to receive either one daily beam, which delivers a doubled dose in each session, or two daily beams delivered according to the standard treatment plan. Both beams are conventionally delivered in the same session but require repositioning the patient before each beam, which takes extra time. This study evaluates whether delivering only one beam daily can maintain treatment effectiveness while reducing toxicity and saving time over the 2 to 7 week treatment period. During the treatment and up to five years of follow-up, patients will be monitored for side effects, quality of life, and treatment effectiveness. Researchers will measure the frequency of toxicities and assess quality of life throughout treatment and follow-up. They will also evaluate the time saved by using a single daily beam. The total participant involvement includes initial treatment and long-term follow-up visits to assess outcomes and safety.

Researchers are investigating the wide range of molecular features found in people receiving care within a large community healthcare system who are at risk of or diagnosed with cancer. The study aims to understand the genetic factors behind premalignant and malignant conditions across various cancer types and stages. This research helps advance knowledge of cancer biology and supports the discovery of biomarkers linked to clinical outcomes. Participants will undergo comprehensive molecular profiling, including somatic tumor testing from tissue andor blood samples using next-generation sequencing. Some samples may also receive whole exome or transcriptome sequencing for research purposes. Pharmacogenomic testing will help explore how individuals respond differently to medications, and participants may optionally provide microbiome samples. Participants may also consent to store biological samples in a biobank and allow their de-identified data to be used for future research. During the study, electronic health records will be reviewed both retrospectively and prospectively to connect clinical data with genomic findings. Researchers will measure how many patients undergo molecular profiling, are referred for genetic testing or targeted clinical trials, and have therapy changes based on molecular or pharmacogenomic results. The study spans five years, with long-term follow-up and data collection to support ongoing research and collaboration in cancer studies.

Chordoma is a rare type of bone cancer that develops in the skull base or spine. This research aims to better understand chordoma by studying its clinical course, appearance on imaging scans, and response to therapies and treatments. The study includes both children and adults aged 2 years and older who have been diagnosed with chordoma and are enrolled in a related NCI protocol. Participants will undergo detailed evaluations including medical history documentation, physical and neurologic exams, and imaging scans such as CT and MRI to observe tumor characteristics. Additional tests like smell tests, cognitive function assessments, and surveys of emotional and physical well-being will be conducted every 6 to 12 months. Some participants may have extra clinic visits depending on their condition. Throughout the study, participants or their healthcare providers will be contacted regularly to collect updated disease information, test results, and imaging data. The researchers will closely follow disease progression, response to treatments, and patient-reported outcomes over time. The main goal is to characterize the natural history of chordoma over a period of up to 10 years, helping to guide future treatment and care approaches.

This research aims to explore the factors that influence decision making for patients with new or recurrent central nervous system CNS tumors and their families. The focus is on understanding challenges related to treatment planning and enrolling in clinical trials. The study involves patients of various ages, including children, young adults, and their caregivers, to gather diverse perspectives on these decisions. Participants will take part in qualitative interviews designed to identify specific barriers to treatment and trial enrollment, such as transportation, childcare, and other related expenses. For children 12 years old and younger, only caregivers will be interviewed. Patients aged 13 to 17 may participate along with their caregivers if they choose, while patients 18 years and older can include their caregivers in interviews. The study will assess the impact of community and hospital resources on decision making through follow-up interviews. Throughout the study, participants will engage in interviews that are both structured and open-ended to fully capture their experiences and challenges. Researchers will measure how these resources affect treatment decisions and will identify barriers to trial enrollment within 12 months. The study aims to improve support for patients and families facing CNS tumor treatment decisions, with total participation lasting as needed for follow-up assessments.

Researchers are evaluating three treatment approachescarbon ion therapy, surgery with or without photonproton therapy, and proton therapy alonefor patients with pelvic bone sarcomas. The study aims to compare how these treatments affect disease control and quality of life, focusing on whether carbon ion therapy offers better patient-reported health outcomes and fewer side effects compared to surgery, as well as improved local control compared to proton therapy. Participants receive one of the three treatments and complete quality of life questionnaires at several points before any therapy, between 2 to 4 months, and again at 5 to 9 months after treatment completion, followed by annual assessments for up to five years. Researchers also review patients medical records to gather detailed clinical data. This observational study involves collecting patient-reported outcomes and medical information without assigning treatments. Throughout the study, patients are asked to complete questionnaires taking about 20 minutes to assess their health-related quality of life. These assessments occur at baseline and various follow-up points up to five years after treatment. Researchers monitor disease progression, local control of the sarcoma, survival outcomes, and treatment-related toxicities. The studys follow-up and data collection span several years to better understand long-term effects and outcomes of the different treatment approaches.

Researchers are exploring the effects of neoadjuvant radiation therapy RT, specifically proton therapy or stereotactic body radiation therapy SBRT, on circulating tumor DNA ctDNA and imaging biomarkers in patients with chordoma of the spine, sacrum, or skull base. The study aims to determine if combining ctDNA levels with radiomic imaging features can accurately predict the tumors histopathologic response to RT. This approach is compared against the current gold standard of pathologic findings to improve diagnostic understanding of radiographic changes after treatment. Participants will undergo neoadjuvant RT using either proton therapy or SBRT before planned surgical removal of the tumor. The study collects data on ctDNA and radiographic changes during this period. Tumor response will be assessed by analyzing tissue obtained during surgery, and imaging features will be correlated with genomic and histopathologic data. This observational study tracks these biomarkers to evaluate their sensitivity and specificity within one week after surgery. During the study, participants will have blood samples taken to measure ctDNA levels and undergo imaging scans to collect radiomic data. Researchers will assess tumor cell activity, wound healing, quality of life, and long-term outcomes such as local progression-free survival and overall survival over a three-year period. Safety and treatment toxicity are also monitored. The total participation includes pre-treatment observation, treatment, surgery, and follow-up evaluations extending up to three years.