Kaposiform Hemangioendothelioma

 Consumptive coagulopathy


image Thrombocytopenia

image Highest risk for coagulopathy in retroperitoneal and intrathoracic tumors, but also seen in association with large cutaneous tumors


• Rare regional lymph node metastases

• No distant metastases reported to date

• Mortality ~ 10%, usually related to Kasabach-Merritt phenomenon

• Chemotherapy often preferred treatment




Microscopic




• Spindled cell areas resembling Kaposi sarcoma

• Zones resembling capillary hemangiomas

• Minimal atypia

• Immunohistochemistry shows positive CD31, CD34, FLI-1, ERG, and podoplanin staining

• Negative for HHV8


Top Differential Diagnoses




• Tufted hemangioma (acquired tufted angioma)
image Small cutaneous tumor

image Histologically identical to kaposiform hemangioendothelioma, also occurs in children

image Likely in same spectrum of tumors

• Kaposi sarcoma
image Older and immunocompromised patients

image Positive for HHV8

• Spindle cell hemangioma
image Adults, usually distal extremities

image Often partially intravascular

image Spindled cells, blister cells, and ectatic vascular spaces

image
Kaposiform Hemangioendothelioma in Groin of Infant
Clinical photograph shows a large inguinal tumor in an infant. More superficial tumors typically present as an erythematous or violaceous mass.


image
Kaposiform Hemangioendothelioma at Low Magnification
Histologic examination at low power of kaposiform hemangioendothelioma demonstrates irregular nodules image of spindled cells in the dermis as well as ectatic vessels at periphery of nodules of spindled cells image.

image
Glomeruloid Area in Kaposiform Hemangioendothelioma
In some areas, the spindled tumor cells can have a somewhat swirling growth pattern, imparting a glomeruloid image appearance.

image
Kaposiform Hemangioendothelioma: Kaposi Sarcoma-Like Area
The tumor cells have uniform hyperchromatic spindled nuclei and are arranged in short fascicles with slit-like vascular lumina.


ETIOLOGY/PATHOGENESIS


Developmental Anomaly




• ~ 1/2 of kaposiform hemangioendothelioma cases present in 1st year of life


CLINICAL ISSUES


Epidemiology




• Age
image Majority present in infancy to teenage years


Presentation




• Painful or painless mass
image Presents as superficial or deep mass

image Cutaneous lesions present as violaceous plaques

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Kaposiform Hemangioendothelioma

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