Heart & vascular pathology
Malignant tumors
Angiosarcoma
Author: Nat Pernick, M.D.
Last author update: 1 November 2013
Last staff update: 19 September 2023
Copyright: 2007-2023, PathologyOutlines.com, Inc.
PubMed Search: Angiosarcoma [title] heart review[ptyp]
Table of Contents
Definition / general | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Electron microscopy images | Differential diagnosis | Additional referencesCite this page: Pernick N. Angiosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/hearttumorangiosarcoma.html. Accessed September 25th, 2023.
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
- 36 year old man with HIV (Arch Pathol Lab Med 2005;129:943)
- 54 year old man with persistent pericardial effusion (Arch Pathol Lab Med 2005;129:117)
- Patient with cutaneous metastases (Arch Pathol Lab Med 1998;122:273)
Treatment
- 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
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
AFIP images
Right atrial mass in 20 year old woman
fronds lined by
hyperchromatic
endothelial cells
atypical endothelial cells
and mitotic activity, but no
obvious vascular channels
poorly formed vascular
structures and extravasated
red blood cells
Metastasis to lung
Factor VIII related antigen+ within cytoplasm
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
- CD31 (most specific)
- CD34, reticulin (highlights vascular channels, shows tumor cells on luminal side of vessels), thrombomodulin, Factor VIII (20%)
Negative stains
Electron microscopy description
- Thin and delicate cytoplasmic processes, Weibel-Palade bodies, distorted extracellular fibrous matrix (Eur Rev Med Pharmacol Sci 2005;9:231)
Electron microscopy images
Images hosted on other servers:
with membrane
bound vesicles
and rough ER
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
