2005   Número: 1









Carta al Director.    







Universidad del País Vasco. Departamento de Cirugía

HospItal Donostia. Servicio de Dermatología.


Lymphangiosarcoma 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.


In cases 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)


Moreover, 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 approach (6).






1.- Andersson HC, Parry DM, Mulvihill JJ. Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications.

Am J Med Genet. 1995 Mar 13;56(1):72-5.


2.-  Lister RK, Black MM, Calonje E, Burnand KG.     

Squamous cell carcinoma arising in chronic lymphoedema.

Br J 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.

Am J Dermatopathol. 2000 Jun;22(3):257-60.


4.-  Azurdia RM, Guerin DM, Verbov JLChronic lymphoedema and angiosarcoma.

Clin Exp Dermatol. 1999 Jul;24(4):270-2.


5.-  Taghian 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 breast cancer.

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.








Depósito Legal BI-8989-909
ISSN 1138-252X

Referencia: Miguel Echenique