Cutaneous Angiosarcoma



Cutaneous Angiosarcoma


Thomas Mentzel, MD









Clinical photograph shows an ill-defined, erythematous and plaque-like, hemorrhagic, cutaneous neoplasm in this male patient.






Hematoxylin & eosin shows a well-differentiated cutaneous angiosarcoma arising in sun-damaged skin.


TERMINOLOGY


Abbreviations



  • Angiosarcoma (AS)


Synonyms



  • Hemangiosarcoma


  • Lymphangiosarcoma


  • Malignant hemangioendothelioma


Definitions



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


  • Clear differentiation between lymphangiosarcoma and sarcoma with blood vessel differentiation remains problematic


ETIOLOGY/PATHOGENESIS


Developmental Anomaly



  • Congenital lymphedema


Environmental Exposure



  • Chronic lymphedema, i.e., after mastectomy (Stewart-Treves syndrome)


  • Radiotherapy


  • Sun exposure


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare


    • Increasing rate due to use of radiotherapy and prolonged actinic damage


  • Age



    • More frequent in elderly patients


    • Very rare in children


  • Gender



    • M > F


Site



  • Relatively frequent in head and neck area


  • Radiation fields


  • Areas of acquired or congenital lymphedema


Presentation



  • Idiopathic (actinic) angiosarcoma



    • Arises predominantly in actinically damaged skin


    • Scalp, upper forehead, face


    • Elderly patients


    • Often initially mistaken for inflammatory lesion or cutaneous lymphoma/pseudolymphoma


  • Radiation-induced angiosarcoma



    • Arises at variable times after therapeutic irradiation (e.g., for breast cancer)


  • Angiosarcoma associated with chronic lymphedema



    • Congenital lymphedema


    • Acquired lymphedema (Stewart-Treves syndrome)


  • Ill-defined lesions


  • Plaque-like, red, indurated lesions


  • Bruise-like lesions


  • Nodular or multinodular appearance of older lesions


  • Often multifocal


Treatment



  • Surgical approaches



    • Wide excision with clear margins


  • Adjuvant therapy



    • Benefit of adjuvant/neoadjuvant chemotherapy unclear


Prognosis



  • Poor prognosis



    • Repeated local recurrences


    • Metastases occur often after repeated recurrences



      • Lymph node


      • Lung


    • 5-year survival (15-20%)



    • Necrosis and epithelioid cytomorphology represent adverse prognostic factors


IMAGE FINDINGS


General Features



  • Best diagnostic clue



    • Ill-defined, plaque-like lesions


  • Location



    • Actinically damaged skin of scalp and face


Specimen Radiographic Findings



  • Diagnostic pathologic vascular structures may be present


MACROSCOPIC FEATURES


General Features



  • Ill-defined, hemorrhagic lesions


  • Flat or ulcerated epidermis


  • Often sponge-like appearance

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cutaneous Angiosarcoma
Premium Wordpress Themes by UFO Themes
%d bloggers like this: