10-year-old female with history of neurofibromatosis type I with large left sacral mass extending into the left leg.EXAMINATION: MRI pelvis and left femur without contrast 10/24/16 Extensive and multifocal T2 hyperintense masses throughout the lumbar spine, predominantly left paraspinal musculature, left lower quadrant, and extending into the left lower extremity, to the level of the mid thigh. These lesions are compatible with plexiform neurofibromata. The masses are relatively confluent, extending superiorly, out of the field of view, to the level of the superior kidneys, (seen on same day MRI lumbar spine). The greatest transverse diameter (in the left lower quadrant) measures greater than 13 cm and greatest AP dimension, also measures 13 cm. The neurofibromata cause significant mass effect in the pelvis, resulting in rightward deviation of the urinary bladder, rectum, as well as the aorta and iliac vasculature. Tumor also surrounds and splays the left internal and external iliac vessels, but flow voids remain patent.There is extensive involvement of the musculature with intramuscular neurofibromas in the left gluteal muscles, paraspinal musculature, psoas, obturator, as well as the majority of the musculature of the left thigh, with relative sparing of the vastus lateralis.Similar to that seen on prior radiographs, the osseous structures in the left pelvis and left femur are markedly abnormal, with heterogeneous marrow signal, thickened cortex, and multifocal areas of thinning.Additionally, there is extensive T2 signal abnormality involving the subcutaneous tissues of the vulva, buttocks, and lower back, compatible with cutaneous neurofibromata. Multiple cutaneous neurofibromata are also present in the posterior and lateral left thigh.Smaller neurofibromata can be seen more distally in the left thigh, which are separate from the confluent masses more proximally.There is extensive dural ectasia of the distal spinal canal with dextroscoliosis of the lumbar spine. Multiple neural foramina in the lower lumbar spine are widened secondary to tumor involvement of the exiting nerve roots.There is asymmetric bladder wall thickening along the anterior/right aspect of the bladder, measuring up to 1 cm in thickness, which may be related to chronic mass effect/obstruction by the massive plexiform neurofibromata.There is a small amount of free fluid in the pelvis, which is likely physiologic.
1.Extensive plexiform neurofibromata involving the lumbar spine, left hemipelvis, and left thigh which results in extensive mass effect on the pelvic organs and vasculature.2.Multiple intramuscular neurofibromata, particularly in the left gluteal, left psoas, and muscles of the left thigh. 3.Abnormal and bizarre configuration of the bones in the left hemipelvis, which likely reflects a combination of intrinsic bone abnormality as well as mass effect from adjacent and surrounding tumor.4.Extensive sheetlike cutaneous neurofibromata of the lower back, buttocks, vulva, and posterior left thigh. 5.Asymmetric wall thickening along the anterior/right aspect of the bladder, which suggests chronic mass effect/obstruction from adjacent tumor.6.Please refer to same-day MRI of the lumbar spine regarding additional findings including dural ectasia and multifocal neural foraminal widening.