Abstract:
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Purpose: To define the most prevalent patterns of involvement and MR imaging findings in abdominopelvic Neurofibromatosis type 1.
Methods and Materials: We reviewed the MR appearance of abdominopelvic lesions in 32 patients (18 males, 14 females) with type 1 neurofibromatosis. The patients were part of a multi-institutional study recruiting 300 patients. All imaging included coronal or sagittal, and contiguous axial short tau inversion recovery images (TR/TE 6000/35;inversion time,150msec; echo-train length 8). Axial images were used for volumetric measurements.
Results: The most common abdominopelvic involvement was in the lumbosacral plexus (n=25), which was combined lumbosacral (n=11), isolated sacral (n=10) or isolated lumbar (n=4). In 20, the involvement extended into the sciatic nerve, and in 20 there was sacral foraminal widening. Abdominal wall (n=23) and retroperitoneal (celiac [n=6] and para-aortic [n=13]) involvement were frequent. Lesions were less commonly intraperitoneal (P=0.001), being located in mesentery (n=4), spleen (n=2), and liver (n=1). Three males and 2 females had involvement of all pelvic structures, with evidence of prior urinary diversion (n=3) or current hydronephrosis (n=2). Genital involvement occurred similarly in males (31%) and females (26%), with involvement of the scrotum (n=7) and penis (n=4), uterus (n=4) and vulva (n=1). Spinal cord compression (n=5) and scoliosis (n=15) were also noted. Target appearance of plexiform lesions was noted in 19 patients.
Conclusion: Abdominopelvic involvement in neurofibromatosis type 1 is primarily extraperitoneal. Although lesions are most prevalent in the lumbosacral plexus and abdominal wall, retroperitoneal and pelvic involvement is common and usually affects important organs. The target MR appearance is present in more than half of the cases.
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Zacharia MD, T,
MR Imaging of Abdominopevlic Involvement in Neurofibromatosis Type 1: A Review of 32 Patients. Radiological Society of North America 2003 Scientific Assembly and Annual Meeting, November 30 - December 5, 2003 ,Chicago IL.
http://archive.rsna.org/2003/3102473.html
