Arch Plast Surg.  2018 Nov;45(6):583-587. 10.5999/aps.2017.01032.

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

• Kim DH
• Lee JS
• Shin JW
• Kim JA
• Jun YJ

• KMID: 2427550
• DOI: http://doi.org/10.5999/aps.2017.01032

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.

Keyword

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

MeSH Terms

Adult
Child
Diagnosis
Extremities
Head
Hemangioendothelioma*
Humans
Lymph Nodes
Neck
Neoplasms, Vascular Tissue
Neuralgia
Neurofibromatoses*
Neurofibromatosis 1*
Skin
Skin Manifestations
Ulcer
Vascular Neoplasms