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

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