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Abdominal Manifestations of Neurofibromatosis

Abdominal Manifestations of Neurofibromatosis REVIEW ARTICLE Nicola Hartley, MBChB, MRCS,* Arumugam Rajesh, MBBS, FRCR,* Ratan Verma, MRCP, FRCR,Þ Rakesh Sinha, FRCR,* and Kumaresan Sandrasegaran, MDþ volvulus. Neurofibromatosis type 2 is a disorder predomi- Abstract: The purpose of this review is to illustrate, with examples, nantly involving the central nervous system. the abdominal manifestations of neurofibromatosis type 1 (NF1) on The association between malignancies such as lym- imaging, with emphasis on computed tomography. Mutations of the phoma and adenocarcinoma with NF1 is unclear. Although NF1 gene lead to abnormal tumor suppression. Consequently, NF1 is there have been reports of adenocarcinomas of the GI tract a complex disease, with patients having an increased prevalence of and pancreaticobiliary system, the presence of these tumors in benign and malignant neoplasms throughout the body. We present patients with NF1 may be unrelated. However, it is difficult to cases of the most common abdominal presentations: neurofibroma, dismiss the presence of adenocarcinoma of the small bowel in malignant peripheral nerve sheath tumor, pheochromocytoma, patients with NF1 as coincidental as these are uncommon carcinoid, gastrointestinal stromal tumor, and seminoma. neoplasms. Benign and malignant neoplasms may arise in the Key Words: neurofibromatosis, plexiform neurofibroma, abdomen in all age groups of NF patients. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Computer Assisted Tomography Wolters Kluwer Health

Abdominal Manifestations of Neurofibromatosis

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ISSN
0363-8715
eISSN
1532-3145
DOI
10.1097/rct.0b013e318054e1ca
pmid
18303281
Publisher site
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Abstract

REVIEW ARTICLE Nicola Hartley, MBChB, MRCS,* Arumugam Rajesh, MBBS, FRCR,* Ratan Verma, MRCP, FRCR,Þ Rakesh Sinha, FRCR,* and Kumaresan Sandrasegaran, MDþ volvulus. Neurofibromatosis type 2 is a disorder predomi- Abstract: The purpose of this review is to illustrate, with examples, nantly involving the central nervous system. the abdominal manifestations of neurofibromatosis type 1 (NF1) on The association between malignancies such as lym- imaging, with emphasis on computed tomography. Mutations of the phoma and adenocarcinoma with NF1 is unclear. Although NF1 gene lead to abnormal tumor suppression. Consequently, NF1 is there have been reports of adenocarcinomas of the GI tract a complex disease, with patients having an increased prevalence of and pancreaticobiliary system, the presence of these tumors in benign and malignant neoplasms throughout the body. We present patients with NF1 may be unrelated. However, it is difficult to cases of the most common abdominal presentations: neurofibroma, dismiss the presence of adenocarcinoma of the small bowel in malignant peripheral nerve sheath tumor, pheochromocytoma, patients with NF1 as coincidental as these are uncommon carcinoid, gastrointestinal stromal tumor, and seminoma. neoplasms. Benign and malignant neoplasms may arise in the Key Words: neurofibromatosis, plexiform neurofibroma, abdomen in all age groups of NF patients.

Journal

Journal of Computer Assisted Tomography – Wolters Kluwer Health

Published: Jan 1, 2008

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