Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome

Abstract

Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.

Skin‐Related complications of Klippel–Trenaunay Syndrome: a retrospective review of 410 patients

“…The cellulitis and bleeding rates noted are similar to our study (21% cellulitis; 17% bleeding) although we found a higher rate of ulceration (21%), potentially due to a greater proportion of adult patients in our cohort (mean age at presentation 23.8 years in our study compared to 14 years in the previous study). Documentation of skin cancer in the setting of KTS exists as case reports or survey studies only, and includes diagnoses of basal cell carcinoma, squamous cell carcinoma of the skin and angiosarcoma 7,8 . One prior study reported squamous cell carcinoma of the skin in conjunction with a large ulcer on the KTS‐affected lower limb 9 .…”

“…Documentation of skin cancer in the setting of KTS exists as case reports or survey studies only, and includes diagnoses of basal cell carcinoma, squamous cell carcinoma of the skin and angiosarcoma. 7,8 One prior study reported squamous cell carcinoma of the skin in conjunction with a large ulcer on the KTS-affected lower limb. 9 In our study, two patients had basal cell carcinoma and only one on a KTS-affected site.…”

“…This can be attributed to the propensity for skin breakdown/erosion in this region along with exposure to fecal matter or urine which may cause infection. In a case series of four KTS patients with Gram‐negative bacteremia, three of the four had a possible cutaneous portal of entry for bacteria due to involvement of the perineum in their VM 10 . Similarly, CM of the feet likely increases risk of infection due to wounds that may heal poorly and result in cellulitis, and potential for footwear to superficially traumatize the skin causing breakdown overlying the CM.…”

Case Report: Two cases of advanced primary cardiac angiosarcoma treated with anlotinib and a retrospective analysis of the literature

“…Targeted therapy has emerged as a current research focus. Presently, research has confirmed that tumor-targeting drugs such as sorafenib, sunitinib and pazopanib have certain therapeutic effects on angiosarcoma (7)(8)(9). However, due to the limited number of cases, designing clinical trials is challenging, and large-scale randomized controlled clinical trials are still lacking, resulting in the absence of a standard treatment plan.…”