Cardiac tumors - malignant

Topic Completed: 1 November 2013

Revised: 27 September 2019

Copyright: 2007-2019, PathologyOutlines.com, Inc.

PubMed Search: Angiosarcoma [title] heart review[ptyp]

Nat Pernick, M.D.
Page views in 2018: 537
Page views in 2019 to date: 554
Cite this page: Pernick N. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hearttumorangiosarcoma.html. Accessed December 8th, 2019.
Definition / general
  • Common primary malignancy of heart, although still rare
  • Usually atrium; right sided more common than left
  • Often men, mean age 40 years
  • Metastases present at diagnosis in 89%, usually to liver and lung (may present as diffuse hemorrhage (Arch Pathol Lab Med 2001;125:1562))
Case reports
  • Usually no effective treatment
  • Poor prognosis with death commonly in a few months
Gross description
  • Large, infiltrating, pink to dark brown, lobulated mass
  • Often invades vena cava and tricuspid valve
Gross images

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Various images

Microscopic (histologic) description
  • Usually poorly differentiated
  • Anastomosing vascular channels forming dilated sinusoids lined by atypical cells
  • Patterns include spindle cell, angiomatous, undifferentiated / solid
  • Prominent mitotic activity
  • Hemorrhage and necrosis common
Microscopic (histologic) images
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Right atrial mass in 20 year old woman

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Atypical papillary
fronds lined by
endothelial cells
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Cellular area with plump,
atypical endothelial cells
and mitotic activity, but no
obvious vascular channels
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Spindle cell tumor with
poorly formed vascular
structures and extravasated
red blood cells

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Metastasis to lung

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Factor VIII related antigen+ within cytoplasm

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Neovascular channels contain RBCs within a fibrous stroma

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Fig 1: transesophageal echocardiography shows large echo dense
mass involving interatrial septum and protruding into right atrium;
Fig 2: anastomosing vascular channels composed of
pleomorphic / hyperchromatic spindle cells

Cytology description
  • Single or clusters of pleomorphic spindle cells in a background of hemorrhage
  • Occasional microacinar or pseudoacinar structures lined by abnormal spindle or polygonal cells (Diagn Cytopathol 2007;35:164)
Positive stains
Negative stains
Electron microscopy description
Electron microscopy images

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Abundant cytoplasm
with membrane
bound vesicles
and rough ER
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Focal endothelial differentiation

Differential diagnosis
  • Hemangioendothelioma: flattened endothelial cells, distinct vascular channels
  • Kaposi sarcoma: usually small and epicardial with minimal myocardial infiltration
  • Metastatic angiosarcoma: more common than primary tumor, cardiac metastases associated with widely disseminated disease
Additional references
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