Kaposiform Hemangioendothelioma

Steven D. Billings, MD

Key Facts

Clinical Issues

Presents in infants and young children
Deep tumors, often multiple nodules
Associated with Kasabach-Merritt phenomenon (especially retroperitoneal tumors)
- Consumptive coagulopathy
- Thrombocytopenia
Rare regional lymph node metastases
No distant metastases reported to date
Mortality approximately 10%, related to local effects of tumor or Kasabach-Merritt phenomenon

Microscopic Pathology

Clinical photograph shows a large inguinal tumor in an infant. More superficial tumors typically present as an erythematous or violaceous mass.

The tumor is composed of irregular nodules

of spindled endothelial cells. At the periphery of the nodules, ectatic vessels

are often present.

ETIOLOGY/PATHOGENESIS

Developmental Anomaly

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

CLINICAL ISSUES

Epidemiology

Presentation

Jul 8, 2016 | Posted by drzezo in PATHOLOGY & LABORATORY MEDICINE | Comments Off

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