ARTICLE
A 61-year-old woman was hospitalized for an acute bacterial cellulitis
of the left lower limb. She had bilateral lymphoedema of lower limbs which
had followed the treatment of a vulvar carcinoma ten years earlier, by
vulvectomy, lymph node dissection and radiotherapy. Physical examination
disclosed numerous violaceous hemorragic nodules on the left calf, extending
up to the groin (Fig. 1).
Treatment with intravenous penicillin G brought quick improvement of the
cellulitis. A biopsy specimen was taken from a nodule (Fig.
2). What is your diagnosis?
Lymphangiosarcoma
of Stewart-Treves
The biopsy specimen demonstrated a dermo-hypodermic proliferation of
spindle cells and multiple vascular spaces. Tumoral cells embolizing the
vascular lumen were frequently observed. Immunohistochemisty was positive
for endothelial marker vimentin, AML, and CD 34 (Fig.
3). Chest X-ray was normal. Abdominal ultrasonography and computered
tomography disclosed a left latero-aortic nodule suggesting a lymph node
metastasis. She received Bleomycin 15 mg every two weeks and topical nitrogen
mustard for one month without any improvement. Only symptomatic measures
were then taken and she died one month later.
Discussion
Lymphangiosarcoma is a malignant vascular tumor arising on a chronic
lymphoedema. Most of the patients are women and the lymphangiosarcoma
occurs at a mean age of 62. Stewart-Treves syndrome was originally reported
for lymphoedematous upper limbs following radical mastectomy for breast
cancer, and this is the most frequent occurrence. It occurs after a mean
lapse of ten years, in less than 1% of treated patients. Stewart-Treves
syndrome has been described less frequently on lymphoedema of the lower
limb, or on the upper limb without mastectomy. On the lower limb it usually
occurs either on congenital lymphoedema [1, 2] or on a post-infectious
lymphoedema. A few similar occurrences have already been described following
the treatment of pelvic cancers. Diagnosis is suggested when one or several
violaceous and hemorragic nodules arise on a chronic lymphoedema. Diagnosis
is finally histological. There is an admixture of spindle cells and vascular
spaces deformed by pleiomorphic endothelial cells. A metastasis of the
primary tumor is ruled out by electron microscopy (Weibel-palade granulations),
or, more currently, by immunohistochemistry. Lymphangiosarcoma cells express
positive endothelial markers (CD34, vimentin, keratine, VIII factor antigen).
Kaposi's sarcoma is excluded by epidemiological context and histologic
examination.
Rapid locoregional extension occurs via
coalescence of new and growing nodules. Subsequent systemic extension
occurs through the blood stream.
Because of the very low occurrence of lymphangiosarcoma,
the treatment is not well codified. There is a general agreement for considering
that a simple tumour excision is not sufficient. Most authors consider
that radical amputation of the limb provides the greatest chance of survival,
but this has obviously a negative psychological impact. External radiotherapy
has led to long survival in a few cases [3]. Interferon alpha [3], Bleomycine
[4], Vinblastin, combination chemotherapy [5] have not given lasting,
positive results.
REFERENCES
1. Andersson HC, Parry DM, Mulvihill JJ. Lymphangiosarcoma in late onset
hereditary lymphedema: case report and nosological implications. Am
J Med Genet 1995; 56: 72-5.
2. Laskas JJ, Shelley WB, Gray Wood M. Lymphangiosarcoma arising in
congenital lymphedema. Arch Dermatol 1975; 111: 86-9.
3. Perrot JL, Mazuy A, Levigne V, Claudy AL. Angiosarcome sur lymphœdème
congénital des membres inférieurs. Rev Eur Dermatol MST
1990; 2: 467-70.
4. Zylerberg VL, Picard C, Crickx B, Grossin M, Belaich S. Syndrome
de Stewart-Treves. Traitement par Bléomycine. Ann Dermatol Venereol
1992; 119: 913-5.
5. Chevrel JP, Briand O, Israel L. Le syndrome de Stewart-Treves. Nouv
Presse Med 1980; 9: 609-10.
|
Printable version
