Ongoing Clinical Trials for Peripheral Primitive Neuroectodermal Tumour of Soft Tissue
Currently, there is 1 ongoing clinical trial for peripheral primitive neuroectodermal tumour of soft tissue. This trial is investigating a targeted radiopharmaceutical therapy for children with somatostatin receptor-positive tumors, including various types of solid tumors and lymphoma. The study is being conducted across multiple European countries and aims to determine the safest and most effective dose for pediatric patients.
Clinical trial locations
- France
- Italy
- Spain
Study to Find the Right Dose and Safety of Lutetium (177Lu) Edotreotide and Arginine-Lysine in Children with Somatostatin Receptor-Positive Tumors
This trial is testing a promising treatment approach for children with tumors that have somatostatin receptors on their surface. The treatment involves using lutetium Lu 177 edotreotide, a targeted radiopharmaceutical therapy that delivers radiation directly to cancer cells while minimizing damage to healthy tissue. An arginine-lysine solution is also given to protect the kidneys during treatment.
Who can participate:
- Children between 2 and 17 years of age
- Those with a confirmed diagnosis of somatostatin receptor-positive tumors, including neuroendocrine tumors, central nervous system tumors, lymphoma, and other solid tumors
- Patients whose tumors have returned or not responded to at least one previous treatment
- Children who can perform daily activities reasonably well, as measured by Karnofsky or Lansky scores of at least 50%
- Those who have recovered from side effects of previous treatments, except for ongoing stable conditions like hair loss or controlled hypothyroidism
- If the child has received certain heart-affecting treatments before, their heart function must show an ejection fraction of 55% or higher
- Tumor samples must test positive for somatostatin receptor expression through immunohistochemistry testing
- Recent imaging scans showing that the tumor absorbs more radioactivity than the liver
- CT or MRI scans within two months before joining, confirming all significant tumor areas are somatostatin receptor-positive
- Written consent from parents or legal guardians, and the child’s agreement to participate
Who cannot participate:
- Children younger than 2 years old
- Patients whose tumors do not have somatostatin receptors
- Those who have not tried at least one other treatment previously
- Patients whose health condition makes it unsafe to receive the study treatment
- Pregnant or breastfeeding patients
- Those with other medical conditions that might interfere with the treatment
- Patients unable to follow study procedures or attend required visits
What the trial involves:
The study follows a structured approach to determine the safest and most effective dose of the treatment. It begins with an initial assessment including medical history review and imaging tests such as CT or MRI scans to confirm the tumor is somatostatin receptor-positive. If children have received previous treatments, a washout period is required to ensure those therapies don’t interfere with the trial results.
The treatment is given through an intravenous infusion directly into a vein. Along with the lutetium Lu 177 edotreotide, children also receive an arginine-lysine solution to protect their kidneys. Throughout the treatment, regular monitoring takes place through imaging tests and blood tests to measure how the body responds and to check for any side effects.
The main goal is to find the highest dose that is both safe and effective with minimal side effects. The trial will evaluate how the treatment moves through the body and how well it works in reducing tumor size. Regular check-ups will continue throughout several treatment cycles, and the study is expected to run for several years to gather enough information about the treatment’s safety and effectiveness in children.
Investigational drug: Lutetium Lu 177 edotreotide is the radiopharmaceutical therapy being tested. It works by attaching to somatostatin receptors on tumor cells and delivering targeted radiation directly to the cancer, helping to destroy tumor cells while protecting surrounding healthy tissue.
Summary
There is currently one ongoing clinical trial available for patients with peripheral primitive neuroectodermal tumour of soft tissue. This trial represents an important research effort focused on pediatric patients with somatostatin receptor-positive tumors. The study is being conducted across three European countries: France, Spain, and Italy, providing access to this experimental treatment for children in multiple locations.
The trial focuses on lutetium Lu 177 edotreotide, a targeted radiopharmaceutical therapy that represents a novel approach to treating these challenging tumors. The emphasis on dose-finding and safety evaluation is particularly important for pediatric populations, where determining the right treatment intensity is crucial for balancing effectiveness with long-term health outcomes.
The multi-year study design reflects the careful, thorough approach needed when evaluating new treatments for children with rare tumors that have not responded to standard therapies. Families interested in this trial should discuss participation with their child’s oncology team to determine if it might be an appropriate option.