Heart
Benign tumors / other nonneoplastic
Papillary fibroelastoma


Minor changes: 10 September 2020

Copyright: 2007-2020, PathologyOutlines.com, Inc.

PubMed Search: Papillary fibroelastoma [title] review [ptyp]

Ryan Kendziora, M.D.
Melanie C. Bois, M.D.
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Cite this page: Kendziora R, Bois MC. Papillary fibroelastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hearttumorfibroelastoma.html. Accessed October 24th, 2020.
Definition / general
  • Papillary fibroelastoma is a benign papillary growth of endocardium and avascular fibroelastic tissue
  • Usually benign and discovered incidentally but can embolize to distal sites
Essential features
  • Papillary fibroelastoma is the most common cardiac tumor
  • Benign behavior but may result in morbidity / mortality from downstream (thrombo) embolization
  • Associated with rKRAS mutations in up to 80% of lesions
Terminology
  • Fibroelastic papilloma (not recommended)
  • Cardiac papilloma (not recommended)
  • Giant Lambl excrescence (not recommended)
ICD coding
  • ICD-10: D15.1 - Benign neoplasm of heart
  • ICD-11: 2F01 - Benign neoplasm of respiratory or intrathoracic organs
Epidemiology
Sites
  • May arise on any endocardial lined surface (e.g. valves, chamber walls)
Etiology
Clinical features
  • Considerable variation to clinical presentation
    • Most commonly are asymptomatic and diagnosed incidentally on routine imaging or at the time of cardiac surgery for an alternate indication
    • May present with syncope, myocardial infarction or sudden death
  • Stroke / ischemia occurs in 33% diagnosed on imaging (J Am Coll Cardiol 2015;65:2420)
    • Embolism thought to be secondary to tumor fragmentation or from thrombi formed on the surface of the lesion
  • Higher incidence in individuals with prior cardiac surgery, thoracic radiation or underlying structural heart disease / cardiomyopathy
Diagnosis
  • Imaging is the most classic means of diagnosis
  • Echocardiography is common due to ubiquitous nature of the modality
  • CT also useful as it provides high spacial resolution
Radiology description
  • Characteristic appearance on imaging (J Am Coll Cardiol 2018;72:202)
  • All modalities:
    • Mobile, frond-like lesion on stalk
  • Special considerations:
    • Echocardiogram: speckled appearance with stippling at tumor edge
    • Magnetic resonance: may show some T2 hyperintensity but enhancement is uncommon
Radiology images

Images hosted on other servers:
Echocardiography

Echocardiography

Echocardiography and magnetic resonance imaging

Echocardiography and MRI

Prognostic factors
Case reports
Treatment
  • Surgical resection is recommended
  • Anticoagulation may be considered in patients who are not surgical candidates (Am Heart J 2003;146:404)
Clinical images

Images hosted on other servers:
Tricuspid valve

Tricuspid valve

Gross description
  • Arborizing, thin strands of tan-white tissue, usually arising from a common stalk
  • Likened to a sea anemone
  • Fronds may not be apparent unless tumor is placed in an aqueous medium
Gross images

Contributed by Melanie C. Bois, M.D.
Surgical excision

Surgical excision

In nonaqueous medium

In nonaqueous medium


AFIP images
Tumor in noncoronary sinus

Tumor in noncoronary sinus



Images hosted on other servers:
Multiple small fronds resembling a sea anemone

Multiple small fronds resembling a sea anemone

Causing sudden death due to occlusion of right coronary ostium

Causing sudden
death due to
occlusion of right
coronary ostium
Microscopic (histologic) description
  • Multiple, branching fronds of paucicellular, avascular fibroelastic tissue lined by a single layer of endocardium
  • Must have multiple branching fronds; Lambl excrescences look identically microscopically but are simple, nonbranching projections of fibroelastic tissue
  • May show hydropic change
  • Histologic characteristics do not appear to correlate with rKRAS mutation status
Microscopic (histologic) images

Contributed by Melanie C. Bois, M.D.
Arborizing fronds

Arborizing fronds

Avascular cores

Avascular cores


AFIP images
Arising from aortic valve cusp

Arising from aortic valve cusp

Movat pentachrome stain

Movat pentachrome stain

Avascular fronds lined by endothelial cells

Avascular fronds lined by endothelial cells

Unusual presentation

Unusual presentation

Virtual slides

Images hosted on other servers:
Papillary fibroelastoma

Papillary fibroelastoma

Positive stains
  • Elastic fibers: Verhoeff van Gieson (VVG) or other elastic stain highlights elastic core of fronds
Negative stains
Molecular / cytogenetics description
Videos

Resection of large aortic valve papillary fibroelastoma

Sample pathology report
  • Heart, aortic valve, excision:
    • Papillary fibroelastoma (evaluated with elastic stain)
Differential diagnosis
  • Lambl excrescence:
    • Similar histology but lacks complex architecture and anastomosing fronds
    • Only occurs on the closing edge of valves
  • Cardiac myxoma:
    • May show similar gross features (particularly when papillary fibroelastoma is collapsed)
    • Contains lepidic or myxoma cells that are calretinin positive
  • Valvular vegetation:
    • May show similar gross features
    • Infectious vegetation shows mixed inflammation with or without microorganisms
    • Nonbacterial thrombotic endocarditis is a sterile vegetation consisting predominantly of fibrin and blood products
Board review style question #1

This tumor was surgically excised from the aortic valve of a 58 year old man. This patient was most likely to have prior exposure to which of the following?

  1. Cisplatin
  2. Inferior vena cava (IVC) filter
  3. Smoking
  4. Thoracic irradiation
  5. Vancomycin
Board review answer #1
D. Thoracic irradiation. Endocardial injury is associated with the development of papillary fibroelastomas. Damage may be from iatrogenic causes (e.g. thoracic irradiation, prior cardiac surgery) or from hemodynamic changes secondary to intrinsic structural heart disease. Chemotherapies and antibiotics have not been associated with papillary fibroelastoma development. IVC filters do not contact the heart.

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Reference: Papillary fibroelastoma
Board review style question #2

A 63 year old woman experienced multiple transient ischemic attacks and was subsequently found to have a large, mobile cardiac mass attached to her aortic valve by a stalk on echocardiography. Why would her cardiologist recommend surgical excision?

  1. Definitive diagnosis and triage to chemotherapeutic regimen
  2. Genetic testing for identification of at risk family members
  3. Potential for malignant transformation
  4. Risk of thrombus formation with subsequent thromboembolization
Board review answer #2
D. Risk of thrombus formation with subsequent thromboembolization. Papillary fibroelastomas undergo surgical excision because of their (thrombo) embolic potential. The most common mechanism is thought to be related to thrombus formation with subsequent embolization; however, portions of the tumor may fragment and result in downstream ischemia. They have not been documented to show malignant transformation and surgical resection is considered curative without need for subsequent chemotherapy. They are not thought to be hereditary in origin.

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Reference: Papillary fibroelastoma
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