The Asociación Europea y Latinoamericana SELNET para la Investigación en Sarcomas is the sponsor of the SELNET-7-1 clinical trial for patients diagnosed with malignant peripheral nerve sheath tumor (MPNST).
In this article you will learn about:
This clinical trial was activated in September 2023 and will remain available for recruitment during approximately 2 years in Spain and other countries.
At the end of this article you can find more details to participate in this study.
What is MPNST?
Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive form of cancer that develops in the cells forming the sheath surrounding peripheral nerves.
These nerves are located outside the central nervous system, encompassing the brain and spinal cord.
MPNST belongs to the sarcoma family, which primarily affects the soft tissues of the body, including muscles, fat, tendons, ligaments, lymph and blood vessels, nerves, and other supporting tissues.
This type of cancer grows rapidly and has the potential to metastasize to other parts of the body, making early detection and treatment crucial for a positive prognosis.
Prevalence of MPNST
Sarcomas are relatively uncommon, and MPNST specifically accounts for approximately 5% to 10% of all sarcoma cases [1].
This tumor predominantly affects young and middle-aged adults.
Notably, individuals with a genetic condition called neurofibromatosis type 1 (NF1) are at a higher risk of developing MPNST.
Approximately 25% to 50% of MPNST patients also have NF1, and about 8% to 13% of individuals with NF1 will develop MPNST during their lifetime [1].
MPNST Diagnosis and Treatment
In the early stages, MPNST may not present with noticeable symptoms.
However, as the tumor grows, several signs may manifest, including pain, weakness, and the presence of a palpable lump beneath the skin.
The treatment approach for MPNST depends on several factors, including the stage of the tumor, its location, and the overall health of the patient.
A multidisciplinary team of specialists is typically involved in determining the most appropriate course of action.
The primary treatment modalities for MPNST include surgery, radiation therapy, and chemotherapy in selected patients.
Surgery
Whenever possible, surgical removal of the tumor and surrounding tissue is the preferred treatment option for MPNST.
However, in some cases, surgery may not be feasible due to factors such as the tumor’s size or its proximity to vital structures.
When surgery is not an option, alternative treatments are explored.
Radiation Therapy
Radiation therapy can be used in conjunction with surgery, either before or after the procedure.
It involves the use of high-energy radiation beams to target and destroy cancer cells, reducing the risk of recurrence or metastasis.
Chemotherapy
Chemotherapy may be in indicated in selected patients with resectable tumors in order to reduce the probabilities of disease relapse.
In certain cases, chemotherapy may be combined with radiation therapy.
Additionally, chemotherapy is the cornerstone of therapy for patients with metastatic MPNST, where the cancer has spread to other parts of the body.
Prognosis for MPNST Patients
Prognosis refers to the long-term outlook for individuals diagnosed with a particular disease.
When it comes to MPNST, prognosis can vary significantly based on several factors, including:
- The tumor’s location within the body
- Whether the cancer has metastasized to other organs
- The extent to which the tumor was removed during surgery
The five-year survival rate for MPNST patients ranges from 23% to 69% [1].
To improve the chances of survival, it is imperative to collaborate with a team of experts promptly after receiving an MPNST diagnosis.
MPNST: An Unmet Medical Need
There are not systemic drugs specifically approved for patients with advanced MPNST, and the existing drugs used in soft-tissue sarcoma in general, have a modest activity in this specific histologic subtype.
Consequently, in those patients with metastatic MPNST, the prognosis continues to be poor.
In any case, patients with MPNST do not have many effective therapeutic options.
Preclinical data and the previous experience derived from the use of selinexor in a compassionate use program (which is a way of receiving a drug that is not approved yet but has already shown safety and signs of activity), suggests that selinexor could be active in patients with advanced MPNST.
Selinexor is an oral drug that blocks a nuclear protein called XPO3.
This protein plays a relevant role in malignant neoplasms and its blockage could help to stop tumor growth.
The SELNET-7-1 Clinical Trial
In September 2023, the Asociación Europea y Latinoamericana SELNET para la Investigación en Sarcomas initiated the SELNET-7-1 clinical trial for patients diagnosed with MPNST.
This study will remain open for recruitment for at least 2 years.
The treatment consists of the combination of two drugs: selinexor (nuclear export inhibitor) and gemcitabine (chemotherapy).
Patients eligible for participation must meet the following essential criteria:
- Age: 18-80 years
- Diagnosis of MPNST (leiomyosarcoma patients can also participate)
- Metastatic/advanced disease
- Patients who previously received at least one line of systemic therapy
The SELNET-7-1 clinical trial is initially open in 10 Spanish sites located in:
- Madrid
- Barcelona
- Zaragoza
- San Sebastián
- Canary Islands
- Murcia
The study will be activated in additional countries by the end of 2023.
SELNET-7-1 Coordinating Investigator
The SELNET-7-1 clinical trial is led by Dr. Javier Martín-Broto, Medical Oncologist at Fundación Jiménez Díaz University Hospital, Madrid, and Director of the SELNET Project.
According to Dr. Martín:
“Based on preclinical results and previous clinical cases, we are very encouraged to test the activity of the selinexor-gemcitabine combination in patients with MPNST. This could represent a very promising new therapeutic option for patients with this challenging disease”.
