# Recurrence of an undifferentiated pleomorphic pulmonary artery sarcoma 8 years after initial presentation: a case report

**Authors:** Baudouin Bourlond, Niccolo Maurizi, Panagiotis Antiochos, Ioannis Skalidis, Katarina Auf Der Springe, Claire Royer, Pierre Monney, Olivier Muller, Mathias Kirsch

PMC · DOI: 10.3389/fcvm.2024.1378333 · 2024-06-25

## TL;DR

A rare heart tumor returned after 8 years but was successfully treated with surgery, radiotherapy, and chemotherapy.

## Contribution

This case report demonstrates a successful multimodal treatment approach for a rare pulmonary artery sarcoma recurrence.

## Key findings

- A 76-year-old patient had a recurrence of a pulmonary artery sarcoma 8 years after initial treatment.
- Combining surgery, radiotherapy, and chemotherapy led to improved survival and quality of life.
- The case highlights the importance of a multidisciplinary approach in treating rare cardiac sarcomas.

## Abstract

Primary cardiac tumors remain exceptionally rare, characterized by a poor prognosis. Among them, sarcomas originating in the pulmonary arteries constitute the most infrequent subgroup within primary cardiac sarcomas.

This report presents the case of a 76-year-old female experiencing a recurrence of an undifferentiated pleomorphic intracardiac pulmonary artery sarcoma located in the right ventricular outflow tract, manifesting 8 years after initial remission. Successful outcomes were attained through a combination of surgical resection, state-of-the-art radiotherapy, and chemotherapy. This comprehensive approach proved essential for optimizing both survival and quality of life.

The unexpectedly prolonged recurrence-free survival observed in this case underscores the effectiveness of the comprehensive multimodal treatment approach outlined in the existing literature. This highlights the pivotal role of a multidisciplinary strategy in addressing primary cardiac sarcomas, particularly those arising in the pulmonary arteries.

## Full-text entities

- **Diseases:** sarcomas (MESH:D012509), Primary cardiac tumors (MESH:D006338), undifferentiated pleomorphic pulmonary artery sarcoma (MESH:D000071079), intracardiac pulmonary artery sarcoma (MESH:C538262), cardiac sarcomas (MESH:D006331)

## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11232353/full.md

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Source: https://tomesphere.com/paper/PMC11232353