Key result
Surgical resection for left atrial intimal sarcoma fails to prevent death within 5 months.
Case Report (n=1)
Highlights a rare presentation of primary cardiac intimal sarcoma manifesting initially with neurological symptoms due to brain metastasis.
Cardiac intimal sarcoma with brain metastasis signals rapid fatality despite resection; leaves open optimal systemic therapies in this rare entity.
A 43-year-old man with an unremarkable medical history presented to our hospital with 2 weeks of headaches, ataxia and confusion. CT of the head revealed a large haemorrhagic cystic lesion. A subsequent chest CT revealed a large left atrial mass. The mass was subsequently biopsied with positive immunohistochemistry staining for MDM2, FLI1 and vimentin. Real-time PCR revealed MDM2 amplification, confirming the diagnosis of intimal sarcoma. The patient underwent surgical resection and reconstruction of the atrium with subsequent discharge to short-term rehabilitation, but his symptoms continued to progress. A repeat CT of the head revealed a new cerebellar mass. He underwent a second resection, but continued to experience worsening symptoms. He was diagnosed with stage IV intimal sarcoma and referred to hospice. The patient died 5 months after initial presentation. Autopsy was performed and revealed the cause of death as pneumonia. There was no involvement of the pulmonic or aortic vessels.
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Saith et al. (2015) conducted a case report in Intimal (spindle cell) sarcoma of the left atrium (n=1). Surgical resection was evaluated on Clinical course and survival. A 43-year-old man with intimal sarcoma of the left atrium underwent surgical resection but experienced disease progression and died 5 months after initial presentation.
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