open access

Vol 25, No 6 (2020)
Published online: 2020-11-01
Submitted: 2020-05-25
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Stewart-Treves syndrome: Case report and literature review

Radovan Vojtíšek, Emília Sukovská, Marika Kylarová, Denisa Kacerovská, Jan Baxa, Barbora Divišová, Jindřich Fínek
DOI: 10.1016/j.rpor.2020.09.006
·
Rep Pract Oncol Radiother 2020;25(6):934-938.

open access

Vol 25, No 6 (2020)
Published online: 2020-11-01
Submitted: 2020-05-25

Abstract

Lymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prognosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, including axillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account for approximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema. Lymphatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developing STS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema. Chronic lymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillary nodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction in the incidence of chronic lymphedema from 40% to 4%. The use of sentinel lymph node biopsy technique is also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STS decreased significantly with improved surgical and radiation techniques. The overall prognosis of STS patients is very poor. Only early radical surgical removal, including amputation or disarticulation of the affected limb, or wide excision at an early stage offers the greatest chance of long-term survival. Only a few case reports and series with a small number of patients with lymphangiosarcoma can be found in the literature. We present a case report of the first diagnosed STS at our department in an effort to highlight the need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.

Abstract

Lymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prognosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, including axillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account for approximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema. Lymphatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developing STS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema. Chronic lymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillary nodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction in the incidence of chronic lymphedema from 40% to 4%. The use of sentinel lymph node biopsy technique is also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STS decreased significantly with improved surgical and radiation techniques. The overall prognosis of STS patients is very poor. Only early radical surgical removal, including amputation or disarticulation of the affected limb, or wide excision at an early stage offers the greatest chance of long-term survival. Only a few case reports and series with a small number of patients with lymphangiosarcoma can be found in the literature. We present a case report of the first diagnosed STS at our department in an effort to highlight the need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.

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Keywords

Stewart-Treves syndrome; Lymphangiosarcoma; Angiosarcoma; Radiotherapy; Breast cancer

About this article
Title

Stewart-Treves syndrome: Case report and literature review

Journal

Reports of Practical Oncology and Radiotherapy

Issue

Vol 25, No 6 (2020)

Pages

934-938

Published online

2020-11-01

DOI

10.1016/j.rpor.2020.09.006

Bibliographic record

Rep Pract Oncol Radiother 2020;25(6):934-938.

Keywords

Stewart-Treves syndrome
Lymphangiosarcoma
Angiosarcoma
Radiotherapy
Breast cancer

Authors

Radovan Vojtíšek
Emília Sukovská
Marika Kylarová
Denisa Kacerovská
Jan Baxa
Barbora Divišová
Jindřich Fínek

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