TY - JOUR
T1 - Imaging features of neurofibromatosis type 1 in the abdomen and pelvis
AU - Zulfiqar, Maria
AU - Lin, Michael
AU - Ratkowski, Kristy
AU - Gagnon, Marie Helene
AU - Menias, Christine
AU - Siegel, Cary Lynn
N1 - Publisher Copyright:
© American Roentgen Ray Society.
PY - 2021/1
Y1 - 2021/1
N2 - 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.
AB - 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.
KW - Lymphoma
KW - Malignant peripheral nerve sheath tumor
KW - Neurofibroma
KW - Neurofibromatosis type 1
KW - Paraganglioma
KW - Pheochromocytoma
KW - Renal artery stenosis
UR - http://www.scopus.com/inward/record.url?scp=85098712124&partnerID=8YFLogxK
U2 - 10.2214/AJR.19.22283
DO - 10.2214/AJR.19.22283
M3 - Article
C2 - 33211573
AN - SCOPUS:85098712124
SN - 0361-803X
VL - 216
SP - 241
EP - 251
JO - American Journal of Roentgenology
JF - American Journal of Roentgenology
IS - 1
ER -