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Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review
Archives of Plastic Surgery ; : 583-587, 2018.
Article in English | WPRIM | ID: wpr-718053
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.
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Full text: Available Index: WPRIM (Western Pacific) Main subject: Skin / Skin Manifestations / Ulcer / Neurofibromatosis 1 / Neurofibromatoses / Vascular Neoplasms / Diagnosis / Extremities / Head / Hemangioendothelioma Type of study: Diagnostic study Limits: Adult / Child / Humans Language: English Journal: Archives of Plastic Surgery Year: 2018 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Skin / Skin Manifestations / Ulcer / Neurofibromatosis 1 / Neurofibromatoses / Vascular Neoplasms / Diagnosis / Extremities / Head / Hemangioendothelioma Type of study: Diagnostic study Limits: Adult / Child / Humans Language: English Journal: Archives of Plastic Surgery Year: 2018 Type: Article