Angiosarcoma of Soft Tissue

Angiosarcoma of Soft Tissue

Thomas Mentzel, MD

Gross pathology shows an ill-defined hemorrhagic neoplasm in deep soft tissue, a rare location for angiosarcoma.

Hematoxylin & eosin shows anastomosing vascular structures lined by enlarged, atypical endothelial cells in this well-differentiated angiosarcoma.



  • Hemangiosarcoma

  • Hemangioblastoma

  • Malignant hemangioendothelioma


  • Malignant mesenchymal neoplasm of cells recapitulating variably morphologic and functional features composed of endothelial cells


Developmental Anomaly

  • Develops rarely in association with genetic syndromes

    • Klippel-Trenaunay syndrome

    • Maffucci syndrome

Environmental Exposure

  • Rarely develops adjacent to foreign material or synthetic vascular grafts



  • Incidence

    • Rare

      • < 1% of all sarcomas

    • More frequent in superficial locations

      • 1/4 of angiosarcomas arise in deep soft tissues

  • Age

    • Occur at any age but most common in older adults

    • Very rare in childhood

  • Gender

    • M > F


  • Deep soft tissues

  • Lower extremities > upper extremities

  • Trunk > head/neck region

  • Significant proportion arises in abdomen and retroperitoneum

  • Rarely multifocal


  • Slow growing

  • Deep mass

  • Usually large mass

  • Hematologic abnormalities

  • Thrombocytopenia may be present

  • Arteriovenous shunting may be present

  • Rarely arise in nonlipogenic component of dedifferentiated liposarcomas

  • Rarely arise in benign or malignant nerve sheath tumors

  • Very rarely arise in preexisting benign hemangioma


  • Surgical approaches

    • Aggressive surgical resection with wide tumor-free margins

  • Adjuvant therapy

    • Response to chemotherapy

    • Inhibition of angiogenesis


  • Poor prognosis irrespective of grade of malignancy

    • Local recurrence in 20-30%

    • Distant metastases in 50%

    • 5-year survival 20-30% at best


General Features

  • Infiltrating neoplasm

  • Areas of hemorrhage


Histologic Features

  • Angiosarcoma

    • Usually no relationship to preexisting vessels

    • Irregular infiltrating vascular spaces

    • Anastomosing vascular spaces

    • Variably pleomorphic endothelial tumor cells

    • Endothelial multilayering

    • Endothelial papillary formations

    • Solid areas common

    • Neoplastic vascular structures encircled by reticulin fibers

    • No complete rim of actin(+) (myo)pericytes

    • Often intracytoplasmic lumina

    • Lumina may contain erythrocytes

    • Prominent nuclear atypia

    • Numerous mitoses

    • Areas of hemorrhage and necrosis may be present

    • Rare predominantly spindle cell morphology

    • Clear distinction between lymphatic and vascular differentiation remains problematic

  • Epithelioid angiosarcoma

    • More frequent in deep soft tissues

    • Often rapid growth

    • Very aggressive clinical course

    • Solid sheets of large epithelioid cells

    • Tumor cells with abundant eosinophilic cytoplasm and large vesicular nuclei

    • Prominent cytologic atypia

    • Numerous mitoses

    • Often areas of tumor necrosis

  • Rare granular cell variant

  • Rare inflammatory variant

Predominant Pattern/Injury Type

  • Diffuse

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiosarcoma of Soft Tissue
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