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Angiosarcoma

DEFINITION AND PATHOGENESIS

  • Angiosarcoma of the subcutaneous and deep tissues may or may not be associated with chronic lymphedema
  • May occur after radical mastectomy and XRT with chronic lymphedema (Stewart-Treves syndrome)
  • Lymphedema may also be congenital or infection associated
  • Mouse model (exposure to dimethylhydrazine)
  • A rare but highly malignant lesion
  • Exposure to vinyl chloride or extravasated Thorotrast (thorium dioxid) reported with angiosarcoma
  • Reported in immunocompromised pts, such as renal transplant pts

IMPORTANCE

  • Rarely occur as a deep soft tissue sarcoma (<1% of all soft tissue sarcomas)

CLINICAL FEATURES

  • May be multifocal

RADIOLOGIC FEATURES

  • Vascular, areas of solid tumor on MRI
  • ± phleboliths

GROSS PATHOLOGY

  • Extremely vascular

HISTOLOGIC FEATURES

  • Spindled areas resembling fibrosarcoma
  • Wide range of atypia and cell types: spindle-cell, epithelioid cells, hemangioma-like to solid cellular areas, bland to bizarre endothelial cells
  • Variable mitotic numbers
  • ± primitive signet ring luminae
  • ± necrotic areas
  • Intraluminal projections and fronds of papillary endothelium
  • Epitheliod type
    • Preminent chondrohyaline matrix
    • Nests of endothelial cells with small immature luminae
    • Hemangioendothelioma
  • Spindle cell type
    • Looks like cavernous hemangioma with spindle cells (simulates Kaposi's sarcoma) and epithelioid endothelial cells
    • Malignant endovascular papillary angioendothelioma
    • Vascular channels are large
  • Expression of vascular endothelial growth factor in ?80%, regulated by the p53/MDM-2 pathway
  • Cytology
    • Blood may obscure cellular detail
    • Fixatives with hemolytic properties may help
    • Spindle-cell and epithelioid morphology
  • Immunohistochemistry:
    • + factor VIII-AG
    • + vimentin
    • + CD 34 antigen (weakly)
    • + reticulin stain
  • EM:
    • If well-differentiated, features of normal endothelium may be present

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Other sarcomas, soft tissue renal cell metastases

DISEASE COURSE AND TREATMENT

  • Prompt dx and wide resection while the lesion is small offers the best chance of survival
  • LR/metastatic rate
    • Epithelioid hemangioendothelioma: 15/30%
    • Spindle hemangioendothelioma: 67%/rare
    • Malignant endovascular papillary angioendothelioma: -/rare
  • Has recurred in a STSG (remote) location after resection from a different site (Koebner phenomenon)

SPECIAL CONSIDERATIONS

HEMANGIOENDOTHELIOMA

  • An intermediate vascular malignancy, may be multifocal with lesions of same extremity
  • Epitheliod type
    • Located in superficial or deep tissues ± multiple lesions (skeletal, lung, liver)
    • Has been reported to occur in a bone infarct associated with Gaucher disease (see under dysplasia)
  • Spindle cell type
    • Occurs distally in the dermal and subcutaneous tissues of the extremities, by young adult-hood, rarely in deeper structures
    • May occur with enchondromatoses as a variant of Maffucci's syndrome (check bone scan)
  • Rarely metastasizes

MALIGNANT ENDOVASCULAR PAPILLARY ANGIOENDOTHELIOMA (Dabska tumor)

  • occurs mostly in children in the skin or subcutaneous tissue
  • LN metastases have been reported

RETIFORM HEMANGIOENDOTHELIOMA

INFANTILE HEMANGIOENDOTHELIOMA

  • ?10% of all hemangioendotheliomas
  • Rare metastases
  • Wide resection ? chemotx
  • Rapid growth, ? life-threatening hemorrhage

CONGENITAL COMPOSITE HEMANGIOENDOTHELIOMA

  • LR and LN metastases reported

KAPOSIFORM HEMANGIOENDOTHELIOMA

  • KAPOSI'S SARCOMA

described first in 1872, probably hyperplasia of vascular tissue in response to a viral infection (herpes virus 8, HHV8)

  • M predominance, acral location, multifocality, spindle cells (resembling well-differentiated fibrosarcoma with vascular spaces) on histology
  • Red to purple macular lesion(s) which may be multifocal
  • Virus-like DNA sequences can be identified
  • ? HIV infection
  • Types
    • Classic
    • African
    • Stages
    • Plaque
    • Patch
    • Nodular

MALIGNANT HEMANGIOPERICYTOMA

  • Most often a benign lesion, but malignancy diagnosed when ? mitotitc activity, cellularity, hemorrhage, and necrosis present
  • See under the heading

    SOLITARY FIBROUS TUMOR

REFERENCES

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