Syndrome de Stewart-Treves: complication rare de lymphœdème chronique

Abstract

We report the case of a 43-year old patient with post-traumatic paraplegia and oedema of the lower limbs. He presented with tumor of the left leg occurring 2-month and half before. Clinical examination revealed a ulcerative- budding multi-nodular tumor associated with bilateral lower extremity lymphedema with multiple nodules and purplish satellites erythematous plaques (image A). Differential diagnosis was Kaposi’s sarcoma, achromic melanoma or pseudo-Kaposi’s sarcoma in a paralyzed limb. Histological examination showed tumoral proliferation of atypical epithelioid cells with fusiform dermis appearance. Immunohistochemical examination showed expression of CD 34 and CD 31 with no CK, Melan A and LCA expression. An evaluation of the extent of loco-regional recurrence and distant metastasis objectified ganglionic and osseous metastases. The diagnosis of metastatic angiosarcoma in the context of Stewart-Treves syndrome was retained and the patient was a candidate for clean amputation followed by palliative chemotherapy. The evolution was marked by the death of the patient 1 month after the amputation. Stewart-Treves syndrome is a skin lymphangiosarcoma complicating chronic lymphedema. This is a rare complication that usually occurs in the arms of women with breast cancer who underwent mastectomy with lymph node dissection. The treatment is based on a wide excision associated with radiation therapy. However the risk of local recurrence and especially of metastases (lungs and bones) is important. The prognosis remains poor with a 5-year survival rate of 10%.

The Pan African Medical Journal 2016;24