KAPOSIFORM HEMANGIOENDOTHELIOMA

By Gary M. White, MD


Kaposiform hemangioendothelioma (KHE) is a rare vascular proliferation of childhood with histologic features resembling Kaposi's sarcoma. The term hemangioendothelioma signifies a neoplasm somewhere between hemangioma and sarcoma. Other terms for KHE are Kaposi-like hemangioendothelioma and hemangioma with Kaposi'’s sarcoma-like features.

Clinical

Lesions reported in patients with KHE include multinodular soft tissue masses, purpuric macules, plaques and multiple telangiectatic papules. A rapidly growing skin or soft-tissue vascular tumor, especially when complicated by KMS in a child beyond the normal age for hemangioma proliferation, should prompt biopsy to rule out KHE.

Treatment

A specialist should be consulted. Localized lesions may be excised but most KHE are too infiltrative and inoperable. Oral rapamycin (Sirolimus) has been reported to be effective in managing cases of KHE complicated by KMP or in the absence of KMP through the inhibition of vasculogenesis and fibrotic pathways [Pediatr Dermatol. 2016 Mar-Apr;33(2):e88-92]. Other therapies that have been used in the past include beta blockers, systemic steroids, vincristine, low-dose radiation and alcohol injection. See also Kasabach-Merritt syndrome.

References

A one-month-old baby girl presented with a congenital circumferential diffuse swelling of entire right leg with reddish purple discoloration, which developed an abscess, ulceration and purulent discharge and sepsis. Hematological investigations showed anemia and thrombocytopenia. Treatment with vincristine and compression resulted in dramatic improvement over 10 months. J Indian Assoc Pediatr Surg. 2015 Oct-Dec; 20(4): 194–196.

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