Angiosarcoma

Angiosarcoma
Cyril Fisher, MD, DSc, FRCPath
Key Facts
Terminology
  • Malignant mesenchymal neoplasm of cells recapitulating variable morphologic and functional features of endothelial cells
Clinical Issues
  • Deep soft tissues
    • Lower extremities, followed by upper extremities
    • Trunk > head and neck
    • Significant proportion intraabdominal and retroperitoneal
  • Rare (more frequent in superficial locations)
    • < 1% of all sarcomas
  • Any age, but most common in older adults
  • Poor prognosis irrespective of grade of malignancy
    • 5-year survival 20-30% at best
  • Aggressive surgical resection with wide tumor-free margins
Microscopic Pathology
  • Irregular, anastomosing vascular spaces
  • Variably pleomorphic endothelial tumor cells
  • Endothelial multilayering and papillary formation
  • Solid areas common
  • No complete rim of actin positive myopericytes
  • Often intracytoplasmic lumina
  • Prominent nuclear atypia
  • Numerous mitoses
  • Expression of endothelial markers
  • Epithelioid angiosarcomas occur relatively frequently in deep soft tissues
    • Solid sheets of large epithelioid cells in epithelioid angiosarcoma
Cutaneous angiosarcoma of the scalp in an elderly man. The dermis contains dilated vascular channels image lined by plump endothelial cells. There are also spindle cell areas image and focal hemorrhage.
Higher magnification shows well-differentiated angiosarcoma comprising irregularly shaped anastomosing vascular structures with open lumina lined by enlarged, atypical endothelial cells image.
TERMINOLOGY
Synonyms
  • Hemangiosarcoma
  • Malignant hemangioblastoma
  • Malignant hemangioendothelioma
Definitions
  • Malignant mesenchymal neoplasm of cells recapitulating variable morphologic and functional features of endothelial cells
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
  • Develops rarely in association with genetic syndromes
    • Klippel-Trenaunay syndrome
    • Maffucci syndrome
  • In longstanding congenital lymphedema
Environmental Exposure
  • Rarely develops adjacent to foreign material or synthetic vascular grafts
  • In limbs with longstanding lymphedema
    • After mastectomy: Stewart-Treves syndrome
  • Post-therapeutic irradiation
CLINICAL ISSUES
Epidemiology
  • Incidence
    • Rare; < 1% of all sarcomas
    • More frequent in superficial locations
      • 1/4 of angiosarcomas arise in deep soft tissues
  • Age
    • Occurs at any age, but most common in older adults
    • Rare subset in childhood
  • Gender
    • M > F
Site
  • Skin of head and neck
  • Skin of limbs in longstanding lymphedema
  • Skin of breast following therapeutic irradiation for carcinoma
  • Deep soft tissue
    • Lower extremities > upper extremities
    • Trunk > head/neck region
    • Significant proportion arises in abdomen and retroperitoneum
    • Rarely multifocal
Presentation
  • Slow growing
  • Deep mass
    • Usually large mass
    • Hematologic abnormalities
    • Thrombocytopenia may be present
    • Arteriovenous shunting may be present
    • Rarely arises in nonlipogenic component of dedifferentiated liposarcomas
    • Rarely arises in benign or malignant nerve sheath tumors
    • Very rarely arises in preexisting benign hemangioma
  • Skin lesions
    • Plaque or nodule with purple discoloration and bruising
Treatment
  • Surgical approaches
    • Aggressive surgical resection with wide tumor-free margins
  • Adjuvant therapy
    • Response to chemotherapy
    • Inhibition of angiogenesis
Prognosis
  • Poor prognosis irrespective of grade of malignancy
    • Local recurrence in 20-30%
    • Distant metastases in 50%
    • 5-year survival 20-30% at best
MACROSCOPIC FEATURES
General Features
  • Infiltrating neoplasm
  • Areas of hemorrhage
MICROSCOPIC PATHOLOGY
Histologic Features
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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiosarcoma

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