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Case Reports
. 2018 Apr 14;6(4):663-665.
doi: 10.3889/oamjms.2018.191. eCollection 2018 Apr 15.

Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient!

Georgi Tchernev  1   2 , Irina Yungareva  3 , Hristo Mangarov  4 , Konstantin Stavrov  5 , Ilia Lozev  6 , Ivanka Temelkova  7 , Svetoslav Chernin  8 , Ivan Pidakev  9 , Michael Tronnier  10
Affiliations
Case Reports

Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient!

Georgi Tchernev et al. Open Access Maced J Med Sci. .

Abstract

Background: The Stewart-Treves syndrome with localisation in the region of the lower extremities is not something unusual as clinical pathology, but the clinical diagnostics is rather difficult, and it can be further complicated maximally because of: the similar locoregional findings in patients with other cutaneous malignancies.

Case report: Presented is a rare form of an epithelioid variant of the Stewart Treves syndrome in a woman, aged 81, localised in the region of the lower leg and significantly advanced only for 2 months. The diagnosis was confirmed histologically and immunohistochemically. Amputation of the affected extremity was planned. Discussed are important etiopathogenetic aspects regarding the approach in patients with lymphedema and possibility for development of the Stewart Treves syndrome.

Conclusion: Analyzing the evidence from the literature worldwide, we concluded that perhaps the only reliable (to some extent) therapeutic option in patients with Stewart Treves Syndrome is 1) the early diagnostics and 2) the following inevitable radical excision or amputation with the maximal field of surgical security in the proximal direction.

Keywords: (HHV-8); Amputation; Angiosarcoma; Epithelioid variant; Stewart Treves syndrome; Surgery.

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Figures

Figure 1
Figure 1
1a) multiple solitary nodules, 2-3.8 cm in diameter and tendency for confluence and endophyte growth resulting in the formation of a bigger endophyte tumour growth and forming a single tumour lesion with dimensions of about 22 to 16 cm. Perilesional area of erythema around the tumours. Presence of multiple satellite lesions in all directions around the tumour plaque; 1b) vessels with irregular branching consisting of large atypical endothelial cells. Mitotic figures and extravascular red blood cells, H&E x200; 1c) irregular acanthosis. In the upper dermis increased number of vessels. Extravasated red blood cells and oedema. H&E x25; 1d) atypical vessels expressing D2-40. Promontory sign, IHC x200; 1e) 3b CD31 in the atypical cells, IHC x200
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