Intimal (spindle cell) sarcoma of the left atrium presenting with abnormal neurological examination

Abstract

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.

Primary cardiac atrial sarcomas. Report of two histologically different cases and review of the literature

“…35-50% ex-Table II. Immunohistochemical and molecular characteristics of intimal sarcoma (InS) [4,9,10,12,13,15,16,17,18,19,20,22,23,25,26,27,28,30,31,32,33,36,39,40,44] Atrial intimal sarcomas only (data from references no [9,10,12,13,15,16,17,18,19,20,22,23,25,26,27,28,30,31,32,33,36,39,40])…”

“…only* (data from references no[10,15,17,19,23,25,26,27,28,30,36, immunofenotype of the tumours with MDM2 gene amplification…”