The Changing Market Landscape of Benign Soft Tissue Sarcomas: Tenosynovial Giant Cell Tumors (TGCTs), Perivascular Epithelioid Cell Neoplasm (PEComa), Neurofibroma, and Vestibular Schwannoma | Key Insights and Findings by DelveInsight

Soft tissue sarcomas can impact people of all genders, ages, and diverse racial and ethnic backgrounds. While they encompass a wide range of tumor types, they remain relatively rare. The prevalence of soft tissue sarcomas varies, with the American Cancer Society approximating that they make up approximately 2% of all cancer cases, and around 13K new soft tissue sarcomas will be diagnosed in 2023 in the United States.

LAS VEGAS, Oct. 23, 2023 /PRNewswire/ — Soft tissue sarcomas are a group of rare tumors that develop in the body’s soft tissues, including muscles, fat, nerves, and blood vessels. While sarcomas are often associated with malignant growths, there is a subset known as benign soft tissue sarcomas that exhibit non-cancerous behavior. Several types of benign soft tissue sarcomas exist, each with distinct characteristics. Some common subtypes include lipomas, which are composed of fat cells and are among the most prevalent benign soft tissue tumors; hemangiomas, consisting of blood vessels and often appearing as red or purple skin growths; and schwannomas, which originate from nerve sheath cells and can cause pain or numbness in affected areas. Although these tumors are not cancerous, they may still require medical attention if they cause discomfort, impede function, or grow significantly in size.

DelveInsight has recently released a series of epidemiology-based market reports focusing on benign soft tissue sarcomas including Tenosynovial Giant Cell Tumors (TGCTs), Perivascular Epithelioid Cell Neoplasm (PEComa), Neurofibroma, and Vestibular Schwannoma. These reports include a comprehensive understanding of current treatment practices, historical and forecasted patient pool, emerging drugs, market share of individual therapies, and historical and forecasted market size from 2019 to 2032  segmented into 7MM [the United States, the EU-4 (Italy, Spain, France, and Germany), the United Kingdom, and Japan].

Additionally, the reports feature exhaustive analysis of prominent companies working with their emerging candidates in different stages of clinical development. Let’s deep dive into the assessment of these benign soft tissue sarcoma markets individually.

To delve into the soft tissue sarcoma market landscape in more detail, access the Soft Tissue Sarcoma Market Report

Tenosynovial Giant Cell Tumors (TGCTs) Market

Tenosynovial Giant Cell Tumor, a rare tumor typically lacking malignant characteristics, affects the synovium-lined tendon sheaths, synovial joints, and soft tissues. It is further classified into two forms based on its growth pattern: Localized Tenosynovial Giant Cell Tumor (L-TGCT) and Diffuse Tenosynovial Giant Cell Tumor (D-TGCT). TGCTs exhibit a broad clinical spectrum and can affect individuals of all age groups. The specific signs and symptoms experienced by patients may vary depending on the affected location and subtype. DelveInsight estimates that the total incident cases of TGCT in the 7MM comprised ~34K cases in 2022 and are projected to increase by 2032. As per the estimates, the United States contributed to the largest incident population of TGCT, acquiring ~43% of the 7MM in 2022. Whereas EU4, the UK, and Japan accounted for around 43% and 14% of the total incident population share, respectively, in 2022.

According to real-world data analysis, surgery stands out as the primary choice for treating TGCTs. However, it’s worth noting that surgical treatments often result in recurrences, and despite their affordability compared to immunotherapeutic alternatives, they entail significant ancillary expenses, ultimately driving up the overall cost. Although TURALIO is approved exclusively in the United States, its Marketing Authorization Application (MAA) was met with rejection by the European Commission (EC) for use in the European Union (EU). The primary rationale behind this decision was the unfavorable risk-to-benefit ratio associated with the drug, mainly stemming from the potential life-threatening hepatotoxic effects, even though it is meant to treat a non-lethal condition. In the United States, TURALIO’s utilization is tightly controlled through the Risk Evaluation and Mitigation Strategy (REMS) Program, with limited availability restricted to specific medical centers.

In the coming years, significant changes and growth are anticipated in the US TGCT market. This transformation will be primarily driven by two promising products: vimseltinib and emactuzumab. As per DelveInsight analysis, the total TGCTs market size in the 7MM is ~USD 200 million in 2022 and is projected to increase at a CAGR of 8% during the study period (2019–2032). The utilization of TURALIO has been hindered by concerns related to safety and tolerability, prompting numerous patients to lean towards off-label TKIs like imatinib and monoclonal antibodies such as infliximab. Vimseltinib and emactuzumab, known for their considerably superior safety records and heightened effectiveness, are expected to find swift adoption in the treatment sphere.

Tenosynovial Giant Cell Tumors Pipeline Therapies and Companies 

• DCC-3014 (vimseltinib): Deciphera Pharmaceuticals
• Emactuzumab: SynOx Therapeutics (Celleron Therapeutics)

For a comprehensive view of the TGCT market, check out the TGCTs Market Assessment

Perivascular Epithelioid Cell Neoplasm (PEComa) Market

Perivascular epithelioid cell neoplasms (PEComas) are infrequent tumors originating in soft tissues. They predominantly develop in proximity to small blood vessels within different anatomical regions, including the lungs, gastrointestinal tract, kidneys, liver, and uterus. These tumors comprise cells with an epithelioid morphology and exhibit similarities to both melanocytes and smooth muscle cells. Additionally, PEComas have the capacity to synthesize certain proteins responsible for pigment production. According to the assessment done by DelveInsight, the total number of PEComa incident cases in the 7MM was around 400 cases in 2022 and are projected to increase by 2032. As per the estimates, the United States contributed to the largest incident population of PEComa, acquiring ~64% of the 7MM in 2022. Whereas EU4 and the UK, and Japan accounted for around 28% and 8% of the total population share, respectively, in 2022.

Specific management guidelines for PEComas are currently unavailable, and due to the limited number of cases, factors associated with the disease’s progression remain poorly defined. The most appropriate treatment for PEComas has not yet been established, but surgery remains the primary treatment modality. Both chemotherapy and radiotherapy have not demonstrated significant clinical benefits in managing PEComas. Typically, PEComas are treated with chemotherapy regimens containing anthracyclines, but their effectiveness is limited, with only a minority (approximately 10–20%) of patients responding, often with significant side effects. The role of neo-adjuvant/adjuvant therapy in patients with malignant PEComa is still a matter of debate, and a conclusive diagnosis is usually only achievable after surgical resection.

Due to the extreme rarity of PEComas, garnering commercial pharmaceutical interest can be a significant challenge. The market for surgical interventions remains largely untapped. Presently, there are no drugs in development specifically targeting PEComas. However, with advancements in diagnostic capabilities and increased awareness of the disease, the landscape of the PEComa market may undergo transformation in the near future. As per DelveInsight analysis, the total PEComa market size in the 7MM is ~USD 20 million in 2022 and is projected to increase at a significant CAGR by 2032. Crafting appropriate pricing policies is vital to ensure success in the PEComa market, ultimately facilitating the launch of a compelling product tailored to the market’s needs. These factors collectively contribute to the development of a product that fits well within the market.

To gain a deeper understanding of the PEComa market, be sure to explore the PEComa Market Outlook

Neurofibroma Market

Neurofibroma is a benign tumor that originates from nerve tissue, specifically the cells that make up the peripheral nervous system. These tumors are typically non-cancerous and can develop anywhere in the body where nerve cells are present. Neurofibromas are most commonly associated with a genetic disorder known as neurofibromatosis, which can lead to the development of multiple neurofibromas throughout a person’s life. While the prevalence of isolated neurofibromas is relatively low, occurring in about 1 in 4,000 individuals, neurofibromatosis is more widespread, affecting roughly 1 in 3,000 people worldwide. This condition can vary in its severity and impact on individuals, with symptoms ranging from mild to more debilitating.

Treatment for neurofibromas typically depends on the size, location, and symptoms they cause. In many cases, observation and monitoring may be recommended if the tumors are small and not causing any discomfort or complications. Surgical removal is often considered for larger or symptomatic neurofibromas, especially if they compress nearby nerves or organs. Additionally, some medications may be used to manage pain or other symptoms associated with neurofibromas. It’s important to note that while treatment can help alleviate symptoms and improve quality of life, complete eradication of neurofibromas is often challenging, and recurrence can occur. Management and treatment plans are typically tailored to the individual patient’s needs and circumstances. 

DelveInsight estimated that the neurofibroma market is expected to show positive growth growing at a significant CAGR by 2032. This change is mailnly characterized by a growing prevalence of neurofibromatosis, a rare genetic disorder, driving increased demand for treatment options. Moreover, pharmaceutical companies are actively researching and developing targeted therapies and drugs to address this unmet medical need. Additionally, rising awareness among healthcare professionals and patients is expected to boost early diagnosis and treatment, further shaping the evolving landscape of the neurofibroma market. Government initiatives and funding for research may also play a crucial role in driving advancements in this field.

Neurofibroma Pipeline Therapies and Companies 

• Selumetinib: AstraZeneca/Merck
• NFX-179 gel: NFlection Therapeutics, Inc.
• Mirdametinib (PD-0325901): SpringWorks Therapeutics, Inc.
• FCN-159: Shanghai Fosun Pharmaceutical Industrial Development Co. Ltd.
• Binimetinib: Array BioPharma

Explore in-depth for a comprehensive understanding of the Neurofibroma Clinical Trials

Vestibular Schwannoma Market

Vestibular schwannomas, also known as acoustic neuromas, are non-cancerous growths that typically develop slowly from the nerves responsible for balance and hearing within the inner ear. These tumors originate due to an excess of Schwann cells, which typically envelop nerve fibers like layers of an onion, providing support and insulation. Vestibular schwannomas are categorized as either sporadic unilateral or hereditary bilateral. Sporadic unilateral acoustic neuromas affect a single ear and represent the most common form of vestibular schwannoma. Through DelveInsight’s analysis, it can be observed that the vestibular schwannoma cases show an increasing trend in the forecasted period in the United States.

Currently, there are no FDA-approved drugs for vestibular schwannomas or the associated hearing loss. Microsurgery, such as the translabyrinthine or retrosigmoid approach, can be used to remove the tumor while preserving hearing and facial nerve function. Alternatively, radiation therapy, including stereotactic radiosurgery or fractionated radiation, may be employed to halt tumor growth without surgery. The choice of treatment is made after careful consideration of individual factors and should involve a multidisciplinary team of medical professionals to ensure the best possible outcome for the patient.

As per DelveInsight analysis, the vestibular schwannoma market is anticipated to grow at a significant CAGR by 2032. Advancements in minimally invasive surgical techniques and radiation therapy are driving vestibular schwannoma market growth, offering patients less invasive and more effective treatment alternatives. Additionally, increasing awareness and early diagnosis contribute to a steadily expanding market, as healthcare providers and pharmaceutical companies strive to address the evolving needs of patients with vestibular schwannomas. 

Vestibular Schwannoma Pipeline Therapies and Companies 

• Koselugo (Selumetinib): AstraZeneca
• Everolimus (RAD001): Novartis
• Brigatinib: Takeda

To access a complete analysis of the vestibular schwannoma market, visit Vestibular Schwannoma Market Assessment

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SOURCE DelveInsight Business Research, LLP