Radiation-Induced Angiosarcoma in a Patient With Klippel-Trenaunay Syndrome: A Case Report.

Background

Klippel-Trenaunay syndrome (KTS) is a rare vascular disorder that typically presents in the lower limb with unilateral port-wine discoloration of the skin due to capillary malformations, varicose veins, and soft tissue and bone overgrowth. Radiation-induced angiosarcoma (RIAS) is a rare vascular malignancy that develops as a long-term complication secondary to radiation therapy. While there is no well-established direct relationship between KTS and development of angiosarcoma, there is literature that suggests a logical association between the 2 disorders. The present case report highlights the importance of further research into a potential connection between KTS and RIAS.

Methods

A 51-year-old female with a history of KTS was referred to the plastic surgery clinic for delayed breast reconstruction following RIAS of the left breast. It was decided that a 2-stage left latissimus dorsi musculocutaneous flap reconstruction with silicone implant would best achieve her goal of symmetry.

Results

The patient reported here had an unremarkable postoperative course. Of note, this patient experienced severe body dysmorphia after her mastectomy and RIAS excision due to the absence of her left breast juxtaposed with her prior large right breast augmentation. The choice of 2-stage combined autologous/implant reconstruction likely contributed to her satisfactory cosmetic outcome.

Conclusions

While RIAS and KTS are distinct conditions, a link may exist between the two. More research is needed to investigate this possible relationship. Aggressive treatment of RIAS is crucial for patient recovery, and a 2-stage combined autologous/implant reconstruction is an optimal choice for post-RIAS reconstruction, especially in patients hoping to achieve a large breast size.

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