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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 [1]
Management:
- no standardized treatment guidelines
- surgery is considered the treatment of choice
- conventional chemotherapy & immunotherapy of little benefit
- prognosis is poor [1]
General
lymphangiosarcoma
syndrome
References
- Bernia E, Rios-Vinuela E, Requena C
Images in Dermatology: Stewart-Treves Syndrome
JAMA Dermatol. Published online April 28, 2021.
PMID: 33909015
https://jamanetwork.com/journals/jamadermatology/fullarticle/2779344