Stewart-Treves syndrome of the lower extremity: case report

Abstract

Background: Stewart-Treves syndrome is the development of angiosarcoma in the setting of chronic lymphedema. It is most commonly observed in an upper extremity after radical mastectomy for breast cancer, though it has the ability to present elsewhere under circumstances of lymphedema from alternative etiologies. The exact mechanism of neoplastic transformation in Stewart-Treves syndrome continues to be investigated, but strong research reveals local immunosuppression due to lymphedema as a key role in oncogenesis. It is a highly aggressive tumor with a poor prognosis in most individuals where aggressive surgical treatment is the standard of care which can be curative, though immunotherapy has been an evolving front with mixed results as the targetable antigenic presentation in angiosarcoma has been variable. Case Description: We present a case of Stewart-Treves syndrome of the lower extremity in a 76-year-old Caucasian female who had a past medical history of multiple comorbidities and chronic lymphedema. The patient presented with necrotic nodules and violaceous papules affecting her lower extremity concerning for malignancy. Biopsy confirmed angiosarcoma and subsequent positron emission tomography revealed extensive metastases to distant organs. In consideration of her poor prognosis and extensive comorbidities, she elected for palliative care and is now deceased. Conclusions: Early detection is critical to prevent progression of this aggressive neoplasm. Providers need to be aware of concerning findings on examination of a patient with lymphedema. Timely biopsy or referral to dermatology must occur to increase the patient’s potential for remission.