PEComa Epidemiology

Key Highlights

• Among the 7MM, the highest number of incident cases of PEComa were observed in United States, comprising of nearly 250 cases in 2023 and is projected to increase during the forecast period (2023–2034).
• According to the NORD (2022), PEComas have been found in people of all ages, from children to older adults. Although they often occur in children with TSC, they also occur sporadically in people with no known risk factors.
• As per the American Association for Cancer Research (2022), PEComas occur in about one case per million people, often in children with an inherited condition called tuberous sclerosis. It is estimated that there are about 100–300 new patients annually in the US. Most PEComas are benign.
• PEComas are tend to be five to seven times more likely in females than males. As per the estimates, in the US, females accounts for approximately 70% of the total PEComas cases.
• Among the age-specifc cases of PEComas, 30 - 44 years and 45 - 59 years of age group contributes to the majority of the PEComas cases, each accounting for nearly 30% of the population. 

DelveInsight’s “Perivascular epithelioid cell neoplasm (PEComa) – Epidemiology Forecast – 2034” report delivers an in-depth understanding of the PEComa, historical and forecasted epidemiology trends 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: 2021-2034

PEComa Disease Understanding and Treatment Algorithm

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.

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.

Further details are provided in the reported

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 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan from 2021 to 2034.

• The total number of incident cases of PEComa in the 7MM was approximately 400 in 2023 and is projected to increase during the forecast period (2024–2034).
• The United States contributed to the largest incident population of PEComa, acquiring ~64% of the 7MM in 2023. Whereas EU4 and the UK, and Japan accounted for around 28% and 8% of the total population share, respectively, in 2023.
• Among the EU4 countries, Germany accounted for the largest number of PEComa cases, i.e.~30 in 2023, whereas Spain accounted for the lowest number of cases in 2023, accunting for approximately 15 cases. 
• According to DelveInsight estimates, in the US, benign stage PEComa was observed to be more prevalent than malignant PEComa, comprising of nearly 150 cases in 2023.
• As per the analysis, it was found that only ~30% of males suffer from PEComa. As per the estimates, in the US, nearly 75 cases of males having PEComa were observed in 2023.

KOL Views

To keep up with current epidemiology 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 treatment landscape, patient reliance on conventional therapies, patient’s therapy switching acceptability, and drug uptake along with challenges related to accessibility, include Medical/scientific writers, Medical Oncologists, and Professors, Department of Medical Oncology, Strasbourg-Europe Cancer Institute, Dana-Farber Cancer Institute, and others.

Delveinsight’s 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 the PEComa epidemiology trends. This will support the clients in understanding the patient distribution among different segments and associated unmet needs.

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, pathogenesis, and currently available therapies.
• Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, disease progression, and treatment guidelines.
• The report provides an edge while developing business strategies, understanding trends, expert insights/KOL views, and patient journey in the 7MM.
• A detailed review of current challenges in establishing the diagnosis.

PEComa Report Insights

• Patient Population
• Country-wise Epidemiology Distribution

PEComa Report Key Strengths

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

PEComa Report Assessment

• Current Diagnostic Practices
• Unmet Needs

Key Questions

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?
• Why do only limited patients appear with symptoms? Why is the current year diagnosis rate not high?
• 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, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• To understand the age-specific PEComa incidence cases in varying geographies over the coming years.
• A detailed overview of stage-specific and gender-specific cases of PEComa is an inclusion.
• To understand the perspective of key opinion leaders around the current challenges with establishing the diagnosis and insights on the recurrent and treatment-eligible patient pool.
• Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.