Purpose of the study: Epithelioid hemangioendothelioma is considered to be a low-grade malignant sarcoma. Surgical treatment is indicated. The multifocal form of the disease is always located on the right side, involving either a limb or the half-body. Based on a case report and a review of the literature, we discuss the nature of this tumor which may or may not be malignant and examine possible indications for non-mutilating surgery.

Case report: A 17-year-old female consulted in 1996 for lytic lesions involving the right lower limb. Most of the lesions were located on the lower portion of the femoral metaphysic and the upper tibia. The lesions appeared as lytic defects with minimal fracture-like lines on the rims. There was also a defect in the lower tibia shaft and in the foot. All lesions involved the right lower limb. Biopsy material obtained in July 1996 confirmed the diagnosis of grade I epithelioid hemangioendothelioma. Search for skeletal and visceral extension was negative. The classical treatment described in the literature is disarticulation of the hip. Considering the static presentation of the lesions, a conservative treatment was attempted. On July 17, 1996, the patient underwent surgery for curettage, liquid nitrogen cyrotherapy, and cement filling of the tibial and lower femoral defects where there was a risk of fracture. The other lesions were not treated. In 1999, the lesion located in the toe became painful and was treated by cement filling. The patient was discharged with simple surveillance. In 2005, i.e. 102 months after cement filling, this young patient was continuing her university education. No new lesion had developed. All lesions remained stable or regressed, including those which had not been treated.

Discussion: Multifocal epithelioid hemangioendothelioma is generally considered as a sarcoma. Careful examination of the literature (less than 50 cases published) shows many discordant elements. First, the lesions are always hemimelic on the right side, never described on the left, an unusual situation for a malignant process. In addition, the clinical courses described to not lead to death by metastasis. The only progression reported involves possible growth of known lesions. The malignant nature of this disease is thus questionable, even though the histological aspect is exactly the same as monofocal epithelioid hemangioendothelioma which can have a much more aggressive, clearly malignant, course.

Conclusion: Surgeons must not propose first-intention amputation for multifocal epithelioid hemangioendothelioma. Because of the favorable outcome observed in our patient warrants, the malignant nature of this condition is questionable.