Shonnard K M, Jelinek J S, Benedikt R A, Kransdorf M J
Department of Radiology, Walter Reed Army Medical Center, Washington, DC 20307-5001.
J Comput Assist Tomogr. 1992 May-Jun;16(3):433-8. doi: 10.1097/00004728-199205000-00017.
Neurofibromatosis of the genitourinary system is rare. We present the CT and MR findings of neurofibromas of the bladder in three patients with von Recklinghausen disease (neurofibromatosis, Type 1). In one case, genitourinary involvement was the primary presentation of the disease. Both CT and MR imaging revealed diffuse and nodular bladder wall involvement, along with pelvic sidewall and adjacent soft tissue abnormalities. The CT attenuation coefficients measured near soft tissue density. On T1-weighted spin echo MR images the tumors revealed signal intensity slightly greater than that of skeletal muscle. Neurofibromas showed markedly increased signal intensity on T2-weighted images relative to the surrounding soft tissues, with marked enhancement in two cases imaged following Gd-DTPA administration. Obstructive hydronephrosis was present in all cases, presumably due to neurofibromas involving the trigonal region. Pelvic sidewall tumors were visualized as rounded, nodular masses extending into the obturator foramina. In the evaluation of patients with von Recklinghausen disease, MR imaging, compared with CT, more clearly defined tumor extent within the bladder, pelvic sidewalls, and surrounding soft tissues.
泌尿生殖系统神经纤维瘤病较为罕见。我们展示了3例冯雷克林霍增氏病（神经纤维瘤病1型）患者膀胱神经纤维瘤的CT和MR表现。其中1例，泌尿生殖系统受累是该病的主要表现。CT和MR成像均显示膀胱壁弥漫性和结节状受累，同时伴有盆腔侧壁及邻近软组织异常。CT测量的软组织密度附近的衰减系数。在T1加权自旋回波MR图像上，肿瘤显示的信号强度略高于骨骼肌。相对于周围软组织，神经纤维瘤在T2加权图像上显示信号强度明显增加，在2例给予钆喷酸葡胺（Gd-DTPA）后成像的病例中显示明显强化。所有病例均存在梗阻性肾积水，推测是由于神经纤维瘤累及三角区所致。盆腔侧壁肿瘤表现为延伸至闭孔的圆形、结节状肿块。在评估冯雷克林霍增氏病患者时，与CT相比，MR成像能更清晰地显示膀胱、盆腔侧壁及周围软组织内肿瘤的范围。
