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Ann Dermatol Venereol. 2018 Apr;145(4):266-269. doi: 10.1016/j.annder.2018.02.001. Epub 2018 Mar 9.

[Angiosarcoma in primary lymphoedema: A rare complication].

[Article in French]

Author information

1
Dermatologie, hôpital Saint-Vincent, Lille, France.
2
Dermatologie, hôpital Saint-Vincent, Lille, France; Université catholique de Lille, Lille, France. Electronic address: aurore.leguern@gmail.com.
3
Université catholique de Lille, Lille, France; Hématologie-oncologie, hôpital Saint-Vincent, Lille, France.
4
Dermatologie, hôpital Saint-Vincent, Lille, France; Université catholique de Lille, Lille, France.
5
Université catholique de Lille, Lille, France; Laboratoire d'anatomopathologie, hôpital Saint-Vincent, Lille, France.

Abstract

BACKGROUND:

Chronic lymphoedema is classically complicated by recurring episodes of cellulitis. Degeneration to the angiosarcoma form (Stewart-Treves syndrome) is much less common. It occurs mainly in the upper limbs following surgery or radiotherapy for mammary neoplasia. Herein we report a rare case of Stewart-Treves syndrome (STS) of the lower limb as a complication of congenital lymphoedema.

PATIENTS AND METHODS:

A 69-year-old woman treated for bilateral lower-limb oedema present for 30years developed painful necrotic lesions in her left lower limb. A diagnosis of angiosarcoma was made based on biopsy of a nodular lesion. Since surgical excision was precluded by the extent of the lesions, chemotherapy was initiated with paclitaxel 175mg/m2 every 21days. The outcome was rapidly unfavourable and the patient died at her home during the third course of treatment.

DISCUSSION:

Angiosarcoma is an extremely rare complication of primary lymphoedema; treatment is poorly codified and the prognosis is unfavourable.

KEYWORDS:

Angiosarcoma; Angiosarcome; Endothelial tumor; Lymphœdème primaire; Primary lymphedema; Stewart-Treves; Tumeur endothéliale

PMID:
29530502
DOI:
10.1016/j.annder.2018.02.001
[Indexed for MEDLINE]

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