Giant Pelvic Neurofibroma in Patient with Plexiform Sciatic Neurofibroma and Neurofibromatosis Type 1 | Zendy

Ivanka Temelkova | Zendy; Georgi Tchernev | Zendy

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Giant Pelvic Neurofibroma in Patient with Plexiform Sciatic Neurofibroma and Neurofibromatosis Type 1

Author(s) -

Ivanka Temelkova,

Georgi Tchernev

Publication year - 2019

Publication title -

open access macedonian journal of medical sciences

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.288

H-Index - 17

ISSN - 1857-9655

DOI - 10.3889/oamjms.2019.304

Subject(s) - medicine , plexiform neurofibroma , neurofibromatosis , neurofibroma , neurofibromatosis type i , thigh , anatomy , trunk , sacrum , pathology , ecology , biology

Neurofibromatosis is a genetic disease with an autosomal dominant type of inheritance. It is a multisystem disease in which, besides skin manifestations, there is a possibility for the involvement of other organs and systems, and an atypical variant of neurofibromatosis type 1 can also be observed- the so-called plexiform neurofibroma. In patients with this inherited disease, mortality is higher due to the existing risk for malignant transformation and development of malignant peripheral nerve sheath tumours (MPNSTs) or neurofibrosarcoma.

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