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
- Rarely occur as a deep soft tissue sarcoma (<1% of all soft tissue sarcomas)
- Vascular, areas of solid tumor on MRI
- ± phleboliths
- 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
- 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
- Blood may obscure cellular detail
- Fixatives with hemolytic properties may help
- Spindle-cell and epithelioid morphology
- + factor VIII-AG
- + vimentin
- + CD 34 antigen (weakly)
- + reticulin stain
- 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)
- 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
- ?10% of all hemangioendotheliomas
- Rare metastases
- Wide resection ? chemotx
- Rapid growth, ? life-threatening hemorrhage
CONGENITAL COMPOSITE HEMANGIOENDOTHELIOMA
- LR and LN metastases reported
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
- Most often a benign lesion, but malignancy diagnosed when ? mitotitc activity, cellularity, hemorrhage, and necrosis present
- See under the heading
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