Rare adult Kaposiform hemangioendothelioma with multiple-bone invasion - clinical experience and literature review.

BACKGROUND

Kaposiform hemangioendothelioma (KHE) is a borderline vascular tumor between hemangioma and malignant angiosarcoma. While KHE has strong local invasion with rare spontaneous regression, it is not observed with distant metastasis. Even if KHE is asymptomatic or without the Kasabach-Merritt phenomenon (KMP), bone or joint invasion should clearly receive proactive treatment. KHE commonly affects infants/children but is rarely seen in adults.

CASE REPORT

We reported a rare adult KHE case with an invasion of >10 separate forearm/hand bones, who underwent multiple-lesion resection and finger amputation after tumor recurrence. Tumor recurrence and KMP were not observed during the 6-month follow-up after the final operation. During the hospitalization and follow-up period, the patient only received medications for infection prevention and pain relief.

CONCLUSIONS

Multiple resectable lesions were found in the distal limb, for which complete resection might not present typical features (high-intensity T2-weighted MRI), which might fail to detect all KHE lesions. Therefore, complete excision is not optimal for multiple resectable KHE lesions.