BACKGROUND: The epithelioid hemangioendothelioma is a soft tissue tumor of vascular origin. Typical localisations are subcutis, cutis, liver, lungs and bones. It has been described in 1982 by Weiss and Enzinger as a separate tumor entity. Due to the unpredictable biological behaviour of the tumor, a borderline malignancy is assumed. CASE REPORT: We report on the case of a 44-year old woman with a metastasising epithelioid hemangioendothelioma in the head and neck area. The primary tumor was located in the subcutis of the left forehead. Due to local recurrences surgical treatment was performed three times after the initial removal in 1993. At the time of the last local recurrence in 1996, a tumor in the left parotid gland was diagnosed and was the reason for a lateral parotidectomy. Pathohistologically, a metastasis of the epithelioid hemangioendothelioma was found. Postoperative radiotherapy was performed and no recurrence occurred until now (22 months follow-up). CONCLUSION: Metastatic epithelioid hemangioendotheliomas are rarely found in the head and neck area. Literature has not yet reported on metastasis formation in the parotid gland. The case illustrates the potentially malignant behaviour of epithelioid hemangioendotheliomas. Hence, therapy should consist of a combination of radical tumor removal and post-operative radiotherapy.
BACKGROUND: The epithelioid hemangioendothelioma is a soft tissue tumor of vascular origin. Typical localisations are subcutis, cutis, liver, lungs and bones. It has been described in 1982 by Weiss and Enzinger as a separate tumor entity. Due to the unpredictable biological behaviour of the tumor, a borderline malignancy is assumed. CASE REPORT: We report on the case of a 44-year old woman with a metastasising epithelioid hemangioendothelioma in the head and neck area. The primary tumor was located in the subcutis of the left forehead. Due to local recurrences surgical treatment was performed three times after the initial removal in 1993. At the time of the last local recurrence in 1996, a tumor in the left parotid gland was diagnosed and was the reason for a lateral parotidectomy. Pathohistologically, a metastasis of the epithelioid hemangioendothelioma was found. Postoperative radiotherapy was performed and no recurrence occurred until now (22 months follow-up). CONCLUSION:Metastatic epithelioid hemangioendotheliomas are rarely found in the head and neck area. Literature has not yet reported on metastasis formation in the parotid gland. The case illustrates the potentially malignant behaviour of epithelioid hemangioendotheliomas. Hence, therapy should consist of a combination of radical tumor removal and post-operative radiotherapy.