Tonsgard J H, Kwak S M, Short M P, Dachman A H
Department of Pediatrics, The University of Chicago Pritzker School of Medicine, Illinois 60637, USA.
Neurology. 1998 Jun;50(6):1755-60. doi: 10.1212/wnl.50.6.1755.
The authors examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors.
Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown.
CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1.
Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas.
The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions.
作者研究了1型神经纤维瘤病（NF-1）中丛状神经纤维瘤的发病率及放射学特征，以确定发生恶性神经鞘瘤风险最高的队列。
丛状神经纤维瘤是NF-1的常见并发症。它们可损害功能、导致毁容，并成为恶性神经鞘瘤的发生部位。丛状神经纤维瘤的发病率及自然病程尚不清楚。
对125例连续的成年NF-1患者（年龄≥16岁）中的91例进行了胸部、腹部和骨盆的CT成像检查。
20%的患者在胸段脊柱旁、纵隔或锁骨上区域有丛状神经纤维瘤。约40%的患者腹部/骨盆扫描异常。脊柱旁、骶丛、坐骨切迹和直肠周围区域是最常见的部位。大多数丛状神经纤维瘤无症状。影像学检查还发现了一些肿瘤，包括恶性神经鞘瘤、肾上腺肿瘤、类癌和神经鞘瘤。
NF-1中丛状病变及其他肿瘤的发生率表明临床医生应仔细监测青年成人；然而，仅凭影像学特征无法可靠地区分良性和恶性病变。
