2003   Número: 1








Carta al Director




SOTO, Jorge.,MD









Echenique-Elizondo, M., MD,FACS.

Associated Professor of Surgery. Basque Country University. School of Medicine.

P. Dr. Begiristain, 105

20010 San Sebastián. Spain


Phone. +34-943-017319

Fax.  +34-943-017330

E-mail. gepecelm@sc.ehu.es






Key Words





Milroy disease





Case report:


A 31 years old white male complaining of congenital – familial form-   non previously treated lymphoedema  - Milroy disease - was admitted with the aspect seen - Images  1,2,3 -. A biopsy  revealed the presence of lymphangiosarcoma – Images 4,5,6 -. No evidence of metastatic disease was clinically evident. High AK amputation was indicated and performed followed by prosthetic extremity replacement. No complementary treatment was done. Patient is free of the disease three years after operation and no evidence recurrence has been noticed so far..


Lymphoedema (1) may be primary or secondary to the presence of other disease and/or to the consequences of surgery or trauma (2). Primary lymphoedema may be congenital – Milroy´s disease – or may occur at any phase of life but it most commonly appears at puberty – Meig’s disease -. Secondary lymphoedema is encountered more often. The most prevalent worldwide cause of lymphoedema is filariasis, which is particularly common in south-east Asia and Africa. In Western countries postsurgical lymphoedema of the extremity prevails. Complications of chronic limb lymphoedema include recurrent cellulitis and lymphangiosarcoma albeit other tumors such squamous-cell carcinoma (3,4), b-cell lymphoma (5)  and angiosarcoma  (6,7,8,9) has been reported.


In cases of long-lasting or congenital lymphoedema 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 lymphoedema (Milroy's disease) in a familial form (10). Hereditary lymphoedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphoedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease) (11). Development of  lymphangiosarcoma is  usually associated with post-mastectomy lymphoedema, has  been described in late-onset hereditary lymphoedema. There is a high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (12).


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 hereditary lymphedema (11)(Milroy's syndrome and Meige's syndrome) and non-hereditary lymphoedema (congenital, praecox or forme tarde lymphoedemas) (13).







The risk of appearance of lymphangiosarcoma following mastectomy and radiation therapy has been recently analyzed. Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France) (14). Of these patients, 6919 were followed for at least 1 year. Out of these, 11 presented with sarcomas thought to be induced by irradiation, 2 of which were Steward-Treves Syndrome, and 9 of which were sarcomas within the irradiated fields. All histological slides were reviewed and a comparison with those of breast cancer was done. The sites of these sarcomas were: parietal wall, 1 case; second costal cartilage, 1 case; infraclavicular region, 1 case; supraclavicular region, 2 cases; internal third of the clavicle, 2 cases; axillary region 2 cases; and the internal side of the upper arm (Stewart-Treves syndrome), 2 cases. The median age of these 11 patients at the diagnosis of sarcomas was 65.8 (49-83). The mean latent period was 9.5 years (4-24). Three patients underwent radical mastectomy and nine modified radical mastectomy. Only one patient received chemotherapy. The radiation doses received at the site of the sarcoma were 45 Gy/18 fr. for 10 cases and 90-100 Gy for 1 case (due to overlapping between two fields). The histology was as follows: malignant fibrous histiocytoma, 5 cases; fibrosarcoma, 3 cases; lymphangiosarcoma, 2 cases; and osteochondrosarcoma, 1 case. The median survival following diagnosis of sarcoma was 2.4 years (4 months-9 years). Two patients are still alive: one with recurrence of her breast cancer, the other in complete remission, with 7 and 3 years follow-up, respectively. All other patients died from their sarcomas. 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. However, the benefit from adjuvant radiation therapy in the treatment of breast cancer exceeds the risk of second cancer; therefore, the potential of radiation-induced sarcomas should not be a factor in the selection of treatment for patients with breast cancer.


Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphoedema (15,16). In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients (17,18). Chemotherapy with intraarterial mitoxantrone and placitaxel with ex vivo previous sensitivity test seems a current adequate complementary approach (19).








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2.-  Laguerre B, Lefeuvre C, Kerbrat P, Hassel M . Stewart-Treves syndrome arising in post-traumatic lymphedema

Bull Cancer. 1999 Mar;86(3):279-82.


3.-  Dandurand M, Bernard F, Barneon G, Guilhou E, Guillot B, Guilhou JJ.Multiple spinocellular carcinomas on chronic lymphedema. Association with vitiligo.

Ann Dermatol Venereol. 1990;117(12):953-6.


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

Squamous cell carcinoma arising in chronic lymphoedema.

Br J Dermatol. 1997 Mar;136(3):384-7.


5.- 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.


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

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


7.-  Allan AE, Shoji T, Li N, Burlage A, Davis B, Bhawan J. Two cases of Kaposi's sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive.

Am J Dermatopathol. 2001 Oct;23(5):431-6.


 8.-  Bach AD, Walgenbach KJ, Horch RE.  Hemangiosarcoma of the left hand in a patient with the rare combination of Maffucci's and Stewart Treves syndrome.

Vasa. 2000 Feb;29(1):71-3.


9.-  Offori TW, Platt CC, Stephens M, Hopkinson GB. Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature.

Clin Exp Dermatol. 1993 Mar;18(2):174-7.


10.- 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.


11.- Brostrom LA, Nilsonne U, Kronberg M, Soderberg G.Lymphangiosarcoma in chronic hereditary oedema (Milroy's disease).

Ann Chir Gynaecol. 1989;78(4):320-3.


12.-  Devolder S, Breuillard F, Gross S. Guess What! Lymphangiosarcoma of Stewart-Treves.

Eur J Dermatol. 1998 Oct-Nov;8(7):527-8.


13.-  Cerri A, Gianni C, Corbellino M, Pizzuto M, Moneghini L, Crosti C. Lymphangiosarcoma of the pubic region: a rare complication arising in congenital non-hereditary lymphedema.

Eur J Dermatol. 1998 Oct-Nov;8(7):511-4.


14.-  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.


15.-  Chung KC, Kim HJ, Jeffers LL. Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients.

J Hand Surg [Am]. 2000 Nov;25(6):1163-8.


16.-  Le Bouedec G, Cure H, de Latour M, Dauplat J. [Stewart-Treves syndrome following mastectomy for breast cancer: a case report

Rev Med Interne. 2001 Aug;22(8):753-7.


17.-  Heitmann C, Ingianni G. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.

Ann Plast Surg. 2000.44(1).72-75


18.-  Aygit AC, Yildirim AM, Dervisoglu S. Lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome.

J Hand Surg . 1999 Feb;24(1):135-7.


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

Referencia: Miguel Echenique