Giant elephantiasis neuromatosa in the setting of neurofibromatosis type 1: A case report

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Giant elephantiasis neuromatosa in the setting of neurofibromatosis type 1: A case report.

Elephantiasis neuromatosa (EN) can arise from a plexiform neurofibroma of the superficial and deep nerves developing from a hyperproliferation of the perineural connective tissue infiltrating adjacent fat and muscles. To date, the clinical association between EN and neurofibromatosis type 1 (NF1) has been poorly defined, particularly with regard to the role of lymphatic alterations and the cons...

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Introduction:Neurofibromatosis is a genetic disease characterized by multifocal benign tumors of peripheral nerves, called neurofibromas, and pigmented spots on the skin which inherited as autosomal-dominant. The most common form of the disease is neurofibromatosis type 1, also known as von Recklinghausen's disease of the skin. When an individual has small number of lesions in a limited region ...

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ژورنال

عنوان ژورنال: Oncology Letters

سال: 2016

ISSN: 1792-1074,1792-1082

DOI: 10.3892/ol.2016.4469