Stewart-Treves Syndrome

Etiology

• chronic postmastectomy lymphedema often with with simultaneous lymphadenectomy, lymph node irradiation, or both

Epidemiology

• rare

Pathology

• rare lymphangiosarcoma that develops in the setting of chronic lymphedema involving an extremity
• diffuse dermal invasion by atypical sinusoidal vessels that dissected the collagen bundles
• neoplastic cells were pleomorphic, with abundant mitotic figures
  • positive stain for CD31, D2-40, ERG, & C-MYC.
• metastases is frequent, particularly to the lungs & regional lymph nodes

Clinical manifestations

• delay in onset (post mastectomy) of approximately 10 years
• chronic lymphedema becoming painful over 2 weeks
• diffuse yellow pigmentation & scattered ecchymotic lesions over upper extremity
• with time, lesions lead to nodules &, eventually extensive cutaneous masses

* image

Management

• no standardized treatment guidelines
• surgery is considered the treatment of choice
• conventional chemotherapy & immunotherapy of little benefit
• prognosis is poor

More general terms

• lymphangiosarcoma
• syndrome