A case report of rare cardiac angiosarcoma and experience of immunotherapy/ targeted therapy

Abstract

Cardiac angiosarcoma is an exceptionally rare primary cardiac malignancy with an aggressive clinical course and poor prognosis, and no standardized treatment strategy has been established. Here, we report a 40-year-old man with cardiac angiosarcoma who underwent complete surgical resection, with pathology confirming a well-differentiated angiosarcoma (T1bN0M0R0). Next-generation sequencing identified a kinase insert domain Receptor (KDR; VEGFR2) alteration related to angiogenesis. Postoperatively, the patient received four cycles of adjuvant chemotherapy: albumin-bound paclitaxel in cycles 1, 2, and 4, and albumin-bound paclitaxel plus cisplatin in cycle 3. Eighteen months after the initial surgery, the disease recurred and a second resection was performed; pathology revealed mediastinal angiosarcoma and dorsal cutaneous angiosarcoma. The patient then received two cycles of the AIM regimen (doxorubicin, ifosfamide, and mesna), followed by AIM combined with the anti-angiogenic multi-target tyrosine kinase inhibitor anlotinib in subsequent cycles. Thereafter, a PD-1 inhibitor in combination with anlotinib was administered as long-term systemic therapy until disease progression. The patient achieved an overall survival of 49 months from the first surgery. This case suggests that a VEGF/VEGFR pathway–directed anti-angiogenic strategy integrated with multimodal therapy may be associated with prolonged survival in selected patients with cardiac angiosarcoma; however, this observation is hypothesis-generating and warrants further validation.