Angiosarcoma



Angiosarcoma


Cyril Fisher, MD, DSc, FRCPath










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

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiosarcoma
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