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- Title
Case report: Navigating treatment pathways for cardiac intimal sarcoma with PDGFRb N666K mutation.
- Authors
Akihiro Nishiyama; Shigeki Sato; Hiroyuki Sakaguchi; Hiroshi Kotan; Kaname Yamashita; Koushiro Ohtsubo; Keishi Mizuguchi; Hiroko Ikeda; Kenji Iino; Hirofumi Takemura; Shinji Takeuchi
- Abstract
In the realm of rare cardiac tumors, intimal sarcoma presents a formidable challenge, often requiring innovative treatment approaches. This case report presents a unique instance of primary intimal sarcoma in the left atrium, underscoring the critical role of genomic profiling in guiding treatment. Initial genomic testing unveiled a somatic, active mutation in PDGFRb (PDGFRb N666K), accompanied by MDM2 and CDK4 amplifications. This discovery directed the treatment course toward pazopanib, a PDGFRb inhibitor, following irradiation. The patient's response was remarkable, with the therapeutic efficacy of pazopanib lasting for 16.3 months. However, the patient experienced a recurrence in the left atrium, where subsequent genomic analysis revealed the absence of the PDGFRb N666K mutation and a significant reduction in PDGFRb expression. This case report illustrates the complexities and evolving nature of cardiac intimal sarcoma treatment, emphasizing the potential of PDGFRb signaling as a strategic target and highlighting the importance of adapting treatment pathways in response to genetic shifts.
- Subjects
SARCOMA; CANCER treatment; LEFT heart atrium; HEART tumors; GENOMICS; GENETIC mutation
- Publication
Frontiers in Oncology, 2024, p1
- ISSN
2234-943X
- Publication type
Article
- DOI
10.3389/fonc.2024.1362347