PEComa - Epidemiology Forecast - 2032

Key Highlights:

• The total number of incident cases of PEComa in the United States was around 250 cases in 2022 and is projected to increase during the forecast period (2023-2032).
• 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
• 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.
• Aadi Bioscience's FYARRO is the only approved therapy for PEComa.

The “Perivascular epithelioid cell neoplasm (PEComa) - Epidemiology Forecast - 2032” report delivers an in-depth understanding of the PEComa, historical and forecasted epidemiology in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, and Japan.

Geography Covered

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Study Period: 2019-2032

PEComa Disease Understanding

PEComa Overview

Perivascular epithelioid cell neoplasms (PEComas) are rare soft tissue tumors. They often form around small blood vessels (perivascular spaces) in various body parts such as the lungs, GI tract, kidneys, liver, and uterus. PEComas comprise cells with an epithelioid (cuboidal) shape and share some features with melanocytes and smooth muscle cells. Melanocytes are cells normally found in the skin and produce melanin to give skin its color.

PEComa can make some of the same proteins used to create pigment. PEComa also makes some of the same proteins found in smooth muscle cells, which comprise muscles not under one's conscious control, such as the muscles that make up one's internal organs and digestive tract.

According to the WHO classification, in the PEComa NOS, both benign PEComa NOS and clinically challenging tumors with a higher degree of malignancy are included (malignant PEComa NOS). A malignant PEComa encountered by clinical oncologists in their practice is abdominopelvic perivascular epithelioid cell sarcoma - the so-called malignant PEComa.

PEComas are considered a group of tumors. However, within this group are specific tumors with unique features and more likely to form in certain body parts. These specific tumors include:

• Angiomyolipomas (AMLs)
• Clear cell sugar tumors (CCT)
• Primary extrapulmonary sugar tumor (PEST)
• Lymphangioleiomyomatosis (LAM)
• Clear-cell myomelanocytic tumor (CCMT) of the falciform ligament/ligamentum teres
• Primary cutaneous PEComa (CCCMT-cutaneous clear cell myomelanocytic tumor)
• PEComas not otherwise specified (NOS)

PEComa Diagnosis

PEComas may be detected by imaging with X-ray, CT scan, or MRI. Once a tumor is detected, a biopsy is needed to examine the cellular makeup and distinguish it from other tumors.

Tissue samples from biopsies will have a characteristic appearance under the microscope to identify tumors as PEComas and differentiate them from other potential tumors.

PEComas typically have mostly epithelioid cells around blood vessels. They also contain protein markers similar to melanocytes (melanin-producing cells) and smooth muscle cells.

Malignant PEComas can be detected from biopsy as well. Malignancy is more likely in larger PEComas, to begin to grow into surrounding tissues, and have a higher percentage of actively growing cells.

Genetic testing is available to identify patients with TSC at an increased risk of developing PEComas.

For patients with LAM, a blood test for detecting increased levels of vascular endothelial growth factor D (VEGF-D) may aid in diagnosing this PEComa subtype. VEGF-D stimulates the growth of new blood vessels, and high levels may be involved in tumor spread.

PEComa Epidemiology

As the market is derived using a patient-based model, the PEComa epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by total incident cases of PEComa, PEComa cases by Gender, Stage, Clinical Presentation, and Age in the 7MM covering the United States, EU4 countries (Germany, France, Italy, and Spain) and the United Kingdom, and Japan from 2019 to 2032.

• The total number of incident cases of PEComa in the United States was around 250 cases in 2022 and is projected to increase during the forecast period (2023-2032).
• 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.
• Among the EU4 countries, Germany accounted for the largest number of PEComa cases, followed by France, whereas Spain accounted for the lowest number of cases in 2022.
• According to the estimates, there were more patients in the benign stage as compared to the malignant PEComa in the United States in 2022. The incidence is projected to increase during the forecast period.
• In the 7MM, approximately 70% of the patient share is attributed to females, whereas only 30% of males suffer from PEComa.

KOL Views

To keep up with current trends, we take KOLs and SMEs' opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts contacted for insights on PEComa evolving diagnostic landscape, include Medical/scientific writers, Medical Oncologists, and Professors, Department of Medical Oncology, Strasbourg-Europe Cancer Institute, Dana-Farber Cancer Institute, and others.

The analysts connected with 50+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM. Centers such as MD Anderson Cancer Center, Texas from UT Southwestern Medical Center in Dallas, Cancer Research UK Barts Centre in London, MD Anderson Cancer Center, etc., were contacted. Their opinion helps understand and validate current diagnostic patterns.

Scope of the Report

• The report covers a segment of key events, an executive summary, descriptive overview of PEComa, explaining its causes, signs and symptoms, and pathogenesis.
• Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, and disease progression have been provided.
• A detailed review of PEComa epidemiology, detailed assumptions, and rationale behind our approach is included in the report.
• A detailed review of current challenges in establishing the diagnosis.

PEComa Report Insights

• Patient Population
• Patient population by gender, stage, and age
• Country-wise Epidemiology Distribution

PEComa Report Key Strengths

• Ten Years Forecast
• The 7MM Coverage
• PEComa Epidemiology Segmentation

PEComa Report Assessment

• Epidemiology Segmentation
• Current Diagnostic Practices

Key Questions Answered

Epidemiology Insights:

• What are the disease risk, burdens, and unmet needs of PEComa? What will be the growth opportunities across the 7MM with respect to the patient population pertaining to PEComa?
• What is the historical and forecasted PEComa patient pool in the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan?
• Which type of molecular alterations associated with PEComas contributes the most in patients affected with PEComa?
• What is the contribution of benign and malignant cases in total incident cases PEComa?

Reasons to Buy

• Insights on patient burden/disease incidence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years
• To understand Key Opinion Leaders' perspectives around the accessibility, acceptability, and compliance-related challenges of the patients and their journey.
• Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.