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CC BY-NC 4.0 · Arch Plast Surg 2018; 45(06): 583-587
DOI: 10.5999/aps.2017.01032
Case Report

Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review

Dong Hwi Kim
Department of Plastic and Reconstructive Surgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
,
Ji Sung Lee
Department of Plastic and Reconstructive Surgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
,
Jong Weon Shin
Department of Plastic and Reconstructive Surgery, Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
,
Jean A Kim
Department of Clinical Pathology, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
,
Young Joon Jun
Department of Plastic and Reconstructive Surgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
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Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.

Keywords

Hemangioendothelioma - Neoplasms, vascular tissue - Neurofibromatoses - Skin manifestation - Neuralgia


Publication History

Received: 12 June 2017

Accepted: 01 December 2017

Article published online:
03 April 2022

© 2018. The Korean Society of Plastic and Reconstructive Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonCommercial License, permitting unrestricted noncommercial use, distribution, and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes. (https://creativecommons.org/licenses/by-nc/4.0/)

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