FIP1L1-PDGFRA-Positive Hypereosinophilia Presenting with Bilateral Extracranial Carotid Artery Aneurysms

World Neurosurg. 2020 Jul:139:419-422. doi: 10.1016/j.wneu.2020.04.119. Epub 2020 Apr 29.

Abstract

Objective: To describe a case of an adult female Filipino with hypereosinophilia and bilateral carotid artery aneurysms who subsequently developed bilateral cerebral hemisphere strokes following aneurysm stenting.

Case description: A 57-year-old female patient with persistent hypereosinophilia presented with progressively enlarging bilateral neck masses, revealed to be carotid artery aneurysms on computed tomography angiography. Following surgical exploration, she later developed right-sided hemiplegia, aphasia, and right hemianopia. Cranial computed tomography revealed infarcts on both middle cerebral artery territories. Bone marrow biopsy and fluorescent in situ hybridization revealed findings suggestive of hypereosinophilic syndrome. She was started on standard aspirin and statin therapy and was discharged sixteen days after the procedure. Partial improvement of neurologic deficits was noted two months later on follow up. Chemotherapy with imatinib was initiated.

Conclusions: This patient's prothrombotic state from FIP1L1-PDGFRA-positive hypereosinophilia may have led to large carotid artery aneurysm formation and intramural thrombosis. This case demonstrates a possible and heretofore undocumented neurovascular sequela of hypereosinophilic syndrome.

Keywords: Carotid artery aneurysm; Endovascular; Hypereosinophilic syndrome; Stroke.

Publication types

  • Case Reports

MeSH terms

  • Aneurysm / complications*
  • Aneurysm / surgery
  • Carotid Artery Diseases / complications
  • Carotid Artery Diseases / surgery
  • Carotid Artery, Internal / pathology
  • Carotid Artery, Internal / surgery
  • Endovascular Procedures / adverse effects*
  • Female
  • Humans
  • Hypereosinophilic Syndrome / complications*
  • Hypereosinophilic Syndrome / genetics
  • Middle Aged
  • Oncogene Proteins, Fusion
  • Postoperative Complications / etiology*
  • Receptor, Platelet-Derived Growth Factor alpha
  • Stents
  • Stroke / etiology*
  • mRNA Cleavage and Polyadenylation Factors

Substances

  • Oncogene Proteins, Fusion
  • mRNA Cleavage and Polyadenylation Factors
  • FIP1L1-PDGFRA fusion protein, human
  • Receptor, Platelet-Derived Growth Factor alpha