Consumptive coagulopathy
Thrombocytopenia
Highest risk for coagulopathy in retroperitoneal and intrathoracic tumors, but also seen in association with large cutaneous tumors
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Rare regional lymph node metastases
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No distant metastases reported to date
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Mortality ~ 10%, usually related to Kasabach-Merritt phenomenon
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Chemotherapy often preferred treatment
Microscopic
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Spindled cell areas resembling Kaposi sarcoma
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Zones resembling capillary hemangiomas
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Immunohistochemistry shows positive CD31, CD34, FLI-1, ERG, and podoplanin staining
Top Differential Diagnoses
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Tufted hemangioma (acquired tufted angioma)
Small cutaneous tumor
Histologically identical to kaposiform hemangioendothelioma, also occurs in children
Likely in same spectrum of tumors
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Kaposi sarcoma
Older and immunocompromised patients
Positive for HHV8
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Spindle cell hemangioma
Adults, usually distal extremities
Often partially intravascular
Spindled cells, blister cells, and ectatic vascular spaces
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
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~ 1/2 of kaposiform hemangioendothelioma cases present in 1st year of life
CLINICAL ISSUES
Epidemiology
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Age
Majority present in infancy to teenage years