2005 Número: 1
STEWARD TREVES SYNDROME POST- MASTECTOMY
ECHENIQUE ELIZONDO, M., TUNEU VALLS, ZUBIZARRETA, J.
Universidad del País Vasco. Departamento de Cirugía
HospItal Donostia. Servicio de Dermatología.
is a rare, aggressive, vascular neoplasm arising in chronic congenital or
acquired lymphedema. Although it is most frequently associated with post-mastectomy
lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally
arise in congenital or pubertal hereditary lymphedema (Milroy's disease and
Meige's disease) and non-hereditary lymphedema (congenital, praecox
or forme tarde lymphoedemas) (1).Secondary lymphedema is
encountered more often. The most prevalent worldwide cause of lymphedema is
filariasis, which is particularly common in south-east Asia and Africa. In
Western countries postsurgical lymphedema of the extremity prevails.
Complications of chronic limb lymphedema include recurrent cellulitis and
lymphangiosarcoma albeit other tumors such squamous-cell carcinoma (2), B-cell
lymphoma (3) and angiosarcoma (4) have been reported.
of long-lasting or congenital lymphedema the finding of ulceration, violaceous
nodules or papules, or apparent traumatic ecchymoses should act as a
diagnostic beacon warning of dangers. A case is reported of a high-grade
lymphangiosarcoma developing in a patient with congenital hereditary
lymphedema (Milroy's disease) in a familial form . Hereditary lymphedemas that
are not associated with other malformations usually affect the lower limbs and
are inherited in an autosomal dominant fashion. These non-syndromic hereditary
lymphedemas are categorized by their age of onset, being either congenital (Milroy
disease) or having an onset in childhood or around puberty (Meige disease).
Development of lymphangiosarcoma
is usually associated with post-mastectomy
lymphedema, and has been
described in late-onset hereditary lymphedema. There is a high incidence of
multiple primary tumors in association with lymphangiosarcoma in the
literature.The risk of appearance of lymphangiosarcoma following mastectomy
and radiation therapy has been recently analyzed. The cumulative incidence of
sarcoma following irradiation of breast cancer was 0.2% (0.09-0.47) at 10
years. The standardized incidence ratio (SIR) of sarcoma (observed n# of cases
(Obs)/expected n# of cases (Exp) computed from the Danish Cancer Registry for
the same period) was 1.81 (CI 0.91-3.23). This is significantly higher than
one, with a p = 0.03 (One Tailed Exact Test). The mean annual excess (obs-exp)/100.000
person-years at risk during the same period/(100,000) was 9.92. This study
suggests that patients treated by radiation for breast cancer have a risk of
subsequent sarcomas that is higher than the general population.(5)
we emphasized the importance or regular clinical examination in all patients
affected by chronic lymphedema . In fact, although the prognosis of this
neoplasm is very poor, prompt
diagnosis and a rapid, ablative surgery associated with radiation therapy can
increase the possibility of survival of these patients. Chemotherapy with
intraarterial mitoxantrone and placitaxel with ex
vivo previous sensitivity test seems a current adequate complementary
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Lymphangiosarcoma in late-onset hereditary lymphedema: case report and
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RK, Black MM, Calonje E, Burnand KG.
Squamous cell carcinoma arising in chronic lymphoedema.
Dermatol. 1997 Mar;136(3):384-7.
3.- Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell
lymphoma of the leg in a chronic lymphedematous extremity.
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RM, Guerin DM, Verbov JLChronic lymphoedema and angiosarcoma.
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A, de Vathaire F, Terrier P, Le M, Auquier A, Mouriesse H, Grimaud E, Sarrazin
D, Tubiana M.Long-term risk of sarcoma following radiation treatment for
Int J Radiat Oncol Biol Phys. 1991 Jul;21(2):361-7.
6.- Breidenbach M, Rein D, Schmidt T, Heindel W,
Kolhagen H, Mallmann P, Kurbacher CM. Intra-arterial mitoxantrone and
paclitaxel in a patient with Stewart-Treves syndrome: selection of
chemotherapy by an ex vivo ATP-based chemosensitivity assay.
Anticancer Drugs. 2000 Apr;11(4):269-73.
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