PEComa Care Delivery Across the Academic–Community Oncology Continuum

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A Case Study in Diagnosis, Treatment, and Clinical Coordination

Case Study Overview

Perivascular epithelioid cell tumors (PEComas) are ultra-rare mesenchymal malignancies that present significant diagnostic and treatment challenges across oncology settings. This white paper examines PEComa care delivery across the academic–community oncology continuum, using real-world perspectives from both settings to illustrate how diagnosis, treatment, and long-term management are operationalized in practice.

The paper highlights the impact of the FDA approval of nab-sirolimus (Fyarro®), the first therapy specifically approved for malignant PEComa, and explores how coordinated academic–community partnerships enable patients to access evidence-based care close to home while maintaining clinical rigor.

Key Insights from the White Paper

• Accurate diagnosis requires expertise and collaboration. PEComa is frequently misdiagnosed due to overlapping clinical and histologic features with more common malignancies, reinforcing the importance of expert pathology review and multidisciplinary tumor boards.
• Academic–community coordination is essential. Academic centers often establish diagnosis and treatment plans, while community practices deliver ongoing therapy, manage toxicities, and support patients locally.
• Nab-sirolimus has reshaped PEComa treatment. Adoption of this FDA-approved therapy has improved consistency of care, efficacy, and tolerability compared with historical off-label mTOR inhibitors.
• Proactive toxicity management improves adherence. Early education, anticipatory supportive care, and dose modifications help preserve quality of life and maintain treatment continuity.
• Financial navigation remains a critical component of care. Manufacturer assistance programs and dedicated financial support teams are essential to overcoming access and affordability barriers.

Why This Matters for Oncology Practice

As oncology care continues to decentralize, community practices are increasingly responsible for managing rare and complex malignancies. This white paper demonstrates that high-quality PEComa care is achievable when academic expertise and community-based delivery are aligned through clear communication, standardized protocols, and shared accountability.

For oncology providers, pharmacists, and care teams, the PEComa model offers a scalable framework for:

• Managing rare cancers with limited historical guidance
• Integrating new FDA-approved therapies into practice
• Supporting patients through coordinated, patient-centered care across settings

The lessons outlined extend beyond PEComa and can inform care models for other rare tumors where expertise, access, and continuity must be carefully balanced.

References

Hornick JL, Fletcher CDM. PEComa: what do we know so far? Histopathology. 2006;48(1):75–82. doi:10.1111/j.1365-2559.2005.02284

Bleeker JS, Quevedo JF, Folpe AL. Malignant perivascular epithelioid cell neoplasm: risk stratification and treatment strategies. Sarcoma. 2012;2012:541626. doi:10.1155/2012/541626

U.S. Food and Drug Administration. FDA approves nab-sirolimus for malignant perivascular epithelioid cell tumors. November 23, 2021.

Wagner AJ, Ravi V, Riedel RF, et al. Nab-sirolimus for patients with malignant perivascular epithelioid cell tumors. Journal of Clinical Oncology. 2021;39(33):3660–3670. doi:10.1200/JCO.21.01728

Ambroggi M, Biasini C, Del Giovane C, Fornari F, Cavanna L. Distance as a barrier to cancer diagnosis and treatment. The Oncologist. 2015;20(12):1378–1385.

Petrelli NJ. A community cancer center program: getting to the next level. Journal of the American College of Surgeons. 2010;210(3):261–270.

Oliver-Perez R, et al. Malignant perivascular epithelioid cell tumor of the uterus: a management challenge. Cancers. 2025;17(13):2185.