Neurofibromatosis Type 1 Presenting with Plexiform Neurofibromas in Two Patients: MRI Features

نویسنده

  • Ahmet Mesrur Halefoglu
چکیده

Neurofibromatosis type 1 (NF1), also known as peripheral neurofibromatosis or von Recklinghausen's disease, is one of the most common genetic disorders. It is inherited in an autosomal dominant pattern. Multiple cutaneous neurofibromas are hallmark lesions of NF1. Localized and plexiform neurofibromas of the paraspinal and sacral region are the most common abdominal neoplasms in NF1. Herein, we report two patients with a known history of NF1 presenting with multiple, extensive localized and plexiform neurofibromas. We describe the important distinguishing features of these tumors as seen on magnetic resonance imaging (MRI), including very bright signal intensity and target sign on T2 weighted images.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Unilateral and multiple nodular plexiform Neurofibromas: An uncommon presentation of Neurofibromatosis type 1

Plexiform neurofibroma is a rare variant but pathognomonic of von Recklinghausen disease. We report an uncommon presentation of neurofibromatosis type I in a girl. This 11-year old girl had multiple plexiform neurofibromas on her left hand, and also several Lisch’s nodules. Classification and clinical features are discussed briefly with emphasis on the possibility this condition may not be give...

متن کامل

Identification of Growth Hormone Receptor in Plexiform Neurofibromas of Patients with Neurofibromatosis Type 1

OBJECTIVE The aim of this study was to investigate the presence of growth hormone receptor in plexiform neurofibromas of neurofibromatosis type 1 patients. INTRODUCTION The development of multiple neurofibromas is one of the major features of neurofibromatosis type 1. Since neurofibromas commonly grow during periods of hormonal change, especially during puberty and pregnancy, it has been sugg...

متن کامل

Progesterone and Estrogen Receptors in Neurofibromas of Patients with NF1

Neurofibromatosis type 1 (NF1) or von Recklinghausen disease is a genetic disorder affecting the growth of cells in nervous system. One of the most remarkable characteristics of this disease is the development of benign tumors of the nervous system (neurofibromas).The purpose of this study was to test tissue samples taken from neurofibromas and plexiform neurofibromas of NF1 patients for the pr...

متن کامل

Giant plexiform neurofibroma causing asymptomatic cervical spinal cord compression in a child with neurofibromatosis type 1.

To cite: Gold JJ, Levy ML, Crawford JR. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013009799 DESCRIPTION A 12-year-old boy with neurofibromatosis type 1 (NF-1) presented with a giant mass involving the posterior head and neck. The mass was present since 6 months of age and showed considerable growth over the past 2 years that caused extensive disfigurement t...

متن کامل

Evaluation of Phenotypic Characteristics of 53 Cases of Neurofibroma in Hazrat Fatemeh Hospital during the Years 1994-2005

Background and Objective: Neurofibromas occur as solitary and multiple forms. The solitary ones are extraneural and more common, histologically exhibiting spindle cells with wavy nuclei, scattered among collagen fibers. Multiple neurofibromas are seen in neurofibromatosis (types I and II), including intr...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 2012  شماره 

صفحات  -

تاریخ انتشار 2012