Case Report: Adjuvant radiation therapy for cardiac intimal sarcoma with long-term disease control

TL;DR

A 40-year-old woman with cardiac intimal sarcoma received surgery, chemotherapy, and radiation therapy, remaining disease-free for over three years.

Contribution

This case report demonstrates the feasibility of adjuvant radiation therapy in treating cardiac intimal sarcoma with advanced planning techniques.

Findings

The patient remained disease-free for 33 months after radiation therapy and 41 months from diagnosis.

Adjuvant radiation therapy was delivered safely with minimal acute toxicity.

Advanced radiation planning techniques enabled curative-intent doses to the heart.

Abstract

Cardiac intimal sarcomas (CIS) are rare, aggressive primary cardiac malignancies with limited data guiding adjuvant treatment. We report a case of a 40-year-old woman with bi-atrial CIS who underwent bi-atrial resection and mitral valve replacement, with pathology confirming an MDM2-amplified intimal sarcoma and positive margins (R1). She received four cycles of adjuvant chemotherapy followed by adjuvant radiation therapy (RT) delivered using volumetric modulated arc therapy (VMAT), planned with four-dimensional computed tomography to account for respiratory motion, to a total dose of 60 Gy with a 6-Gy sequential boost. Treatment was well tolerated, with only grade 1–2 acute toxicities that resolved by completion of therapy. At 33 months following completion of RT and 41 months from diagnosis, the patient remains alive and disease-free without evidence of recurrence or significant late toxicity. This case supports the feasibility of incorporating adjuvant RT into a multimodal treatment approach for CIS and suggests that advanced radiation planning techniques may allow safe delivery of curative-intent doses to the heart, although further studies are needed to define its role in this rare malignancy.

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