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Everything you need to know about Computed Tomography (CT) & CT Scanning

Syndromes in CT: Neurofibromatosis Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Syndromes in CT ❯ Neurofibromatosis

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  • “Neurofibromatosis type 1 (NF1) is one of the most fascinating and common human mendelian disorders, affecting approximately one in 3000 persons. From the initial artist renderings of patients with NF1 in the 15th century and the earliest medical reports in 18th century, to the complex molecular genetic studies of the late 20th century, physicians and lay persons alike have been fascinated with this disease because of its diverse manifestations and the unusual and bizarre physical appearances associated with the disease.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
  • “NF1 belongs to a group of disorders referred to as phakomatoses. These disorders (NF1, neurofibromatosis type 2, tuberous sclerosis, Sturge-Weber syndrome, and neurocutaneous melano- sis) have selective involvement of tissues of ectodermal origin (central nervous system, eye, and skin). All of these disorders, with the exception of Sturge-Weber syndrome, have an autosomal dominant inheritance pattern.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
  • “The association of gastrointestinal stromal tumors with NF1 has been recently recognized in the medical literature. In all reports to date, the gastrointestinal stromal tumors occurring in patients with NF1 are located in the small intes- tine and are multiple or occur in association with other intestinal neoplasms. Abdominal pain and bleeding are the most common presenting symptoms.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
  • “Gastrointestinal stromal tumors associated with NF1 are histologically and immunophenotypically identical to those that occur in patients without NF1. The distinguishing features of gastrointestinal stromal tumors in NF1 are their predominant location in the small intestine and their tendency for multiplicity. Gastrointestinal stromal tumors arise in or near the muscularis propria of the gastrointestinal tract wall and may have intramural, intraluminal, or extraluminal growth.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
  • The biologic behavior of gastrointestinal stromal tumors in patients with NF1 does not appear to be different than that of such tumors in patients without NF1. The tumors may be benign, be malignant, or have uncertain malignant potential based on the current understanding of gastrointestinal stromal tumors.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
  • “On CT and MR images, the tumors are heterogeneously enhancing masses with focal areas of cystic change or evidence of hemorrhage. The tumors have an intramural location and may extend intraluminally, simulating a polypoid mass, or exophytically into the adjacent mes- entery.”
    Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation
    Angela D. Levy et al.
    RadioGraphics 2005; 25:455– 480
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