| Literature DB >> 33211573 |
Maria Zulfiqar1, Michael Lin1, Kristy Ratkowski1, Marie-Helene Gagnon1, Christine Menias2, Cary Lynn Siegel1.
Abstract
OBJECTIVE. Loss of the neurofibromatosis type 1 (NF1) tumor suppressor protein causes uninhibited activation of the RAS oncogene, which leads to tumorigenesis in patients with NF1. This case-based review discusses imaging manifestations of NF1 in the abdomen and pelvis, highlighting key genetic associations and management to elucidate features different from the general population. CONCLUSION. The spectrum of pathologic findings includes gastrointestinal tumors such as gastrointestinal stromal tumors, genitourinary lesions including urogenital neurofibromas, vascular entities such as renal artery stenosis, and less common associations like lymphoma.Entities:
Keywords: lymphoma; malignant peripheral nerve sheath tumor; neurofibroma; neurofibromatosis type 1; paraganglioma; pheochromocytoma; renal artery stenosis
Mesh:
Year: 2020 PMID: 33211573 DOI: 10.2214/AJR.19.22283
Source DB: PubMed Journal: AJR Am J Roentgenol ISSN: 0361-803X Impact factor: 3.959