Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review.
- Author:
Dong Hwi KIM
1
;
Ji Sung LEE
;
Jong Weon SHIN
;
Jean A KIM
;
Young Joon JUN
Author Information
- Publication Type:Case Report
- Keywords: Hemangioendothelioma; Neoplasms, vascular tissue; Neurofibromatoses; Skin manifestation; Neuralgia
- MeSH: Adult; Child; Diagnosis; Extremities; Head; Hemangioendothelioma*; Humans; Lymph Nodes; Neck; Neoplasms, Vascular Tissue; Neuralgia; Neurofibromatoses*; Neurofibromatosis 1*; Skin; Skin Manifestations; Ulcer; Vascular Neoplasms
- From:Archives of Plastic Surgery 2018;45(6):583-587
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
