stewart-treves_syndrome_arising_in_patients_with_lymphaticovenular_anastomosis_for_chronic_lymphedema_of_the_leg.pdf | |
File Size: | 967 kb |
File Type: |
Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare complication that forms
as a result of chronic, long-standing lymphedema. Although it most commonly refers to
malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer,[1] it may
also describe lymphangiosarcomas that result from congenital and other causes of
chronic secondary lymphedema.[2]
Lymphangiosarcoma arising from cancer-related lymphedema has become much less
common with better surgical techniques, radiation therapy, and conservative
treatment.[3] The
prognosis, even with wide surgical excision and subsequent radiotherapy, is
poor.[4]
Incidence
In the 1960s, the incidence 5 years after a radical mastectomy varied from
0.07% to 0.45%.[5] Today,
it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.[6]
Treatment
The treatment of choice is a large resection or amputation of the affected limb.
Radiation therapy can precede or follow surgical treatment. Tumors that have
advanced locally or have metastasized can be treated with mono or
polychemotherapy, systemically or locally.[6]
However, chemotherapy and radiation therapy have not been shown to improve
survivorship significantly.[2]
Prognosis
Early detection is key. Untreated patients usually live 5 to 8 months after
diagnosis.[2]
as a result of chronic, long-standing lymphedema. Although it most commonly refers to
malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer,[1] it may
also describe lymphangiosarcomas that result from congenital and other causes of
chronic secondary lymphedema.[2]
Lymphangiosarcoma arising from cancer-related lymphedema has become much less
common with better surgical techniques, radiation therapy, and conservative
treatment.[3] The
prognosis, even with wide surgical excision and subsequent radiotherapy, is
poor.[4]
Incidence
In the 1960s, the incidence 5 years after a radical mastectomy varied from
0.07% to 0.45%.[5] Today,
it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.[6]
Treatment
The treatment of choice is a large resection or amputation of the affected limb.
Radiation therapy can precede or follow surgical treatment. Tumors that have
advanced locally or have metastasized can be treated with mono or
polychemotherapy, systemically or locally.[6]
However, chemotherapy and radiation therapy have not been shown to improve
survivorship significantly.[2]
Prognosis
Early detection is key. Untreated patients usually live 5 to 8 months after
diagnosis.[2]
- ^ James, William D.; Berger, Timothy G.; et al. (2006).
Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier.
ISBN 0-7216-2921-0. - ^ a b c Sharma, A; Schwartz, RA (June 2012). "Stewart-Treves
syndrome: Pathogenesis and management.". J Am Acad Dermatol. PMID 22682884. - ^ Kumar MBBS MD FRCPath, Vinay (2010). Robins and Cotran:
Pathologic Basis of Disease 8th Edition. Philadelphia, PA: Saunders
Elsevier. pp. 1093. ISBN 978-1-4160-3121-5. - ^ Pincus LB, Fox LP (August 2008). "Images in clinical medicine. The
Stewart-Treves syndrome". N. Engl. J. Med. 359 (9):
950. doi:10.1056/NEJMicm071344. PMID 18753651.
http://content.nejm.org/cgi/pmidlookup?view=short&pmid=18753651&promo=. - ^ Heitmann, C; Ingianni, G (January 2000).
"Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.". Ann Plast
Surg. 44 (1): 72-5. PMID 10651369. - ^ a b Wierzbicka-Hainaut, E; Guillet, G (December 2010).
"[Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of
lymphoedema]". Presse Med. 39 (12): 1305–8. PMID 20970956.