Cardiac papillary fibroelastoma: retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review

Pathol Res Pract. 2013 Apr;209(4):208-14. doi: 10.1016/j.prp.2013.02.001. Epub 2013 Feb 28.


Cardiac papillary fibroelastomas (PFEs), which are mainly found in the valves, are rare benign tumors that can cause embolism. Single-center surgical experience in the treatment of this tumor is uncommon. All patients surgically treated for this neoplasm at our institution from January 1995 to October 2012 (15 patients with 17 lesions) were queried for clinical and pathologic characteristics, and the literature was reviewed. The mean age of detection was 55.8±11.48 years. Twenty percent of the patients were male. The tumor was an incidental finding in 60%. Symptoms directly related to PFEs occurred in 40% of patients. The most common clinical presentation in symptomatic cases was embolism (40%), mostly transient ischemic attack or stroke. Cardiac valves were predominantly involved (76.5%); the most commonly valve affected was the aortic valve (29.4%), followed by the mitral valve (17.6%). Concurrent valvular disease was observed in 41.7% of patients. The mean size of tumors was 11.4±7.9mm (range 2-25mm). In 86.7% of the patients, the tumor was solitary. The mitral valve was the most common origin of tumor systemic embolism. Fronds core could be the end stage of hypermature elastic fibers, which grows in apposition to young fibers. Simple surgical excision or valvular reconstruction was accomplished in most patients (58.3%). PFE is usually small, appears singly, and can cause potentially serious complications. Symptomatic and left side tumors should undergo surgical excision with valve-spare surgery when possible. Surgical removal of PFE is safe, efficacious, and definitive.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Cardiac Valve Annuloplasty
  • Female
  • Fibroma / complications
  • Fibroma / pathology*
  • Fibroma / surgery
  • Heart Neoplasms / complications
  • Heart Neoplasms / pathology*
  • Heart Neoplasms / surgery
  • Heart Valve Diseases / etiology
  • Heart Valve Prosthesis Implantation
  • Heart Valves / pathology*
  • Heart Valves / surgery
  • Humans
  • Intracranial Embolism / etiology
  • Ischemic Attack, Transient / etiology
  • Male
  • Middle Aged
  • Retrospective Studies
  • Spain
  • Stroke / etiology
  • Treatment Outcome
  • Tumor Burden