Cardiac sarcoma: new diagnostic framework proposed from two rare left atrial cases

Primary cardiac sarcomas are rare but highly aggressive malignancies that pose formidable diagnostic and therapeutic challenges. Their low incidence, heterogeneous histopathology and tendency to present at an advanced stage have historically translated into poor clinical outcomes. In a new review published in the journal Cardio-Oncology, a team from a tertiary referral centre in Singapore describes two patients with primary left atrial sarcomas and, drawing on a focused literature review, proposes a structured institutional protocol for managing this difficult disease.

Both patients underwent complete histopathological evaluation, including extensive immunohistochemistry and molecular testing. The proposed protocol includes rapid diagnostic work-up and advanced multimodal imaging among its integrated elements, alongside histopathological and molecular classification and multidisciplinary treatment planning.

The proposed protocol integrates several elements into a single coordinated pathway: fast-tracked diagnostics, advanced imaging, histopathological and molecular classification, decision-making by a multidisciplinary tumour board, radical surgical resection strategies — including cardiac autotransplantation, in which the heart is removed and reimplanted after tumour excision, when required — and coordinated perioperative systemic and radiation therapies. The framework is presented as a pragmatic institutional guide rather than a validated clinical guideline, reflecting the scarcity of high-quality evidence in such a rare disease.

On outcomes, the authors are careful to frame the available data appropriately. Evidence from contemporary, predominantly retrospective series suggests that integrated multidisciplinary management may be associated with improved survival compared with historical treatment strategies. In selected patients, reported median survival has increased from approximately 6 to 12 months to approximately 24 months. Because these figures come largely from retrospective collections rather than randomised trials, they indicate an association and a direction of travel rather than proof of causation, and they apply to selected patients rather than all comers.

The publication arrives as a shared early version, citable with a permanent DOI and subject to further edits before the final Version of Record. For clinicians, its practical value lies in consolidating the components of modern care — imaging, molecular pathology, surgery and oncologic therapy — into one sequence that a single institution can realistically execute.

Subject of Research: A diagnostic and multidisciplinary management framework for rare primary cardiac sarcomas, based on two treated cases and a literature review.

Article Title: Primary cardiac sarcoma: a pragmatic diagnostic framework for a rare and challenging entity through literature review and illustration by two cases from a tertiary referral centre

Article References: Lizwan, M., Chia, X. F. A., Poon, Y. E. L., Ewe, S. H., Ng, C. T., Yap, F. H. X., Lai, S. H., Tan, T. E., & Pang, P. Y. K. (2026). Primary cardiac sarcoma: a pragmatic diagnostic framework for a rare and challenging entity through literature review and illustration by two cases from a tertiary referral centre. Cardio-Oncology. https://doi.org/10.1186/s40959-026-00590-z

Image Credits: AI Generated

DOI: 10.1186/s40959-026-00590-z

Keywords: cardiac sarcoma, cardio-oncology, left atrium, multidisciplinary management, immunohistochemistry, molecular diagnostics, cardiac autotransplantation, rare tumours, tumour board, survival outcomes, Primary, cardiac