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KHE. associated with the Kasabach-Merritt and lymphangiomatosisKHE may be present at birth in neonates, or they can develop in infants within the first few months after birth. truncal location is the most common site. An ill-defined purpuric mass is a common presentation of KHE. KHE. Clinically
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1. kaposi hemangioendothelioma By
Dr.Saad almohizea
2. KHE associated with the Kasabach-Merritt and lymphangiomatosis
KHE may be present at birth in neonates, or they can develop in infants within the first few months after birth.
truncal location is the most common site.
An ill-defined purpuric mass is a common presentation of KHE
3. KHE Clinically nonspecific
they can appear as tender rapidly expanding red plaques, nodules, grouped papules, or telangiectasias.
The histology is distinctive:appearance intermediate between capillary hemangioma and Kaposi's sarcoma
nonmetastasizing
locally aggressive behavior, rapidly invading surrounding tissues and structures
4. COMPLICATIONS COSMETIC
COAGLOPATHY
LOCAL
INFECTION
HYPERTROPHY
5. KHE KHE has a mortality rate of 24%, which is related to coagulopathy or complications of local tumor infiltration.
Patients with tumors on the trunk or proximal extremities (scalp, proximal thigh, axilla ) have a higher incidence of lymphangiomatosis and Kasabach-Merritt syndrome
6. DDX Kaposi's sarcoma
cellular hemangiomas (infantile and juvenile hemangioendotheliomas), tufted angioma
spindle cell hemangioendothelioma
angiosarcoma
7. TREATMENT Best results are surgical excision with a wide margin
prone to recur
Glucocorticoids
CYCLOPOSHAMIDE
vincristine
66% of retroperitoneal and 30% of cutaneous lesions are fatal