Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma) | Primary Research (KOL's Insight) | Market Intelligence | Epidemiology & Market Forecast-2035

A cancer type known as an MPNST, or malignant peripheral nerve sheath tumour, affects the cells that make up the sheath that protects and envelops peripheral nerves. MPSNST has a history of rapid growth and the potential for widespread dissemination. The majority of MPNST cases are in people who are young or middle-aged. People with MPNST (NF1) are more likely to have the hereditary disease neurofibromatosis type 1 (NF1). Between 25 and 50% of MPNST patients also have NF1. In 8 to 13% of NF1 patients, an ongoing MPNST diagnosis will be made. Treating metastatic disease is extremely challenging because there is a dearth of valid information regarding the efficacy of systemic therapies for this subtype of sarcoma. Chemotherapy based on anthracyclines is typically the main form of treatment.

Description

A type of cancer called a malignant peripheral nerve sheath tumor (MPNST) affects the cells that make up the sheath that covers and protects peripheral nerves. Peripheral nerves are those (brain and spinal cord) that are not a part of the central nervous system. MPNST is an example of a sarcoma. The soft tissues of the body, which include muscle, fat, tendons, ligaments, lymphatic and blood vessels, nerves, and other connective and supporting tissue, are where this cancer develops. MPSNST has a history of rapid growth and the potential for widespread dissemination. The majority of MPNST cases are in people who are young or middle-aged. People with MPNST (NF1) are more likely to have the hereditary condition neurofibromatosis type 1 (NF1). Between 25% and 50% of MPNST patients also have NF1. In 8 to 13% of NF1 patients, a permanent MPNST diagnosis will be made.

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma) (Epidemiology)

In between 5% and 10% of all cases of rare cancer, MPNST, a particularly uncommon type of sarcoma, is diagnosed. The majority of MPNST victims are typically young to middle-aged adults. The risk of developing MPNST (NF1) is higher in those who have the hereditary condition neurofibromatosis type 1. Between 25 and 50% of people with MPNST also have NF1. In addition, 8 to 13% of NF1 patients will develop MPNST during their lifetime.

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)-Current Market Size & Forecast Trends

The market for malignant peripheral nerve sheath tumors (MPNST) is expected to grow significantly, driven by increasing awareness, advancements in treatment options, and ongoing research. Currently, MPNSTs, which are aggressive soft tissue sarcomas, represent about 5% to 10% of all sarcomas and have a low incidence rate in the general population, at approximately 0.001%. The global market for MPNST treatments is projected to reach around USD 1 billion by 2035, reflecting a compound annual growth rate (CAGR) of about 8%. This growth is supported by the rising number of clinical trials focusing on targeted therapies and immunotherapies, which aim to improve patient outcomes. Additionally, the market is influenced by the high lifetime risk of developing MPNST in individuals with neurofibromatosis type 1 (NF1), which affects a significant portion of patients with this tumor type. Overall, the MPNST market is well-positioned for substantial expansion as new treatment strategies emerge and awareness of this rare sarcoma increases through 2035.

Treating metastatic disease is extremely challenging because there is a dearth of valid information regarding the efficacy of systemic therapies for this subtype of sarcoma. Chemotherapy using anthracyclines is frequently used as the main form of treatment. As it has been shown to be associated with the best outcomes, it is generally recommended to combine doxorubicin with ifosfamide. Support for other regimens is largely lacking in data. Due to the rarity of MPNST, conducting significant randomized trials is practically impossible. Various small, targeted molecules were tested on small cohorts of MPNST patients, but the outcomes of these studies did not lead to any advancements in the treatment of the disease. The prognosis is poor for patients with metastatic or unresectable disease, with a median progression-free survival of roughly four months and an overall survival of 13 months. Due to their rarity, little is known about their specific clinical manifestations, imaging characteristics, and appropriate management; however, the preferred course of treatment appears to be complete surgical resection with negative margins, followed by adjuvant radiotherapy and chemotherapy to reduce the risk of recurrence and metastatic dissemination. Cardiovascular computed tomography (CT) and cardiac magnetic resonance imaging (CMRI) can be used to assess the morphology and function of the heart. TTE is a quick, non-invasive method for determining the presence and location of a cardiac mass. Diagnosis and classification of MPNST are challenging because these tumors are difficult to distinguish from neurofibromas and frequently resemble synovial sarcomas and ossifying fibromyxoid tumors. The most efficient therapies for unselected soft tissue sarcomas are doxorubicin and ifosfamide, which have a RECIST (Response Evaluation Criteria in Solid Tumors) response rate of about 25%. Response rates were reported to be no better in MPNST at 21% in a multi-institution retrospective study that combined MPNST patients from different soft tissue sarcoma trials.

Report Highlights

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Current Market Trends

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Current & Forecasted Cases across the G8 Countries

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Market Opportunities and Sales Potential for Agents

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Patient-based Market Forecast to 2035

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Untapped Business Opportunities

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- Product Positioning Vis-a-vis Competitors' Products

Malignant Peripheral Nerve Sheath Tumor (MPNST - a rare sarcoma)- KOLs Insight