A Large Intrathoracic Mass in a Patient with Neurofibromatosis-1: a case report

Abstracts: Dural ectasia is circumferential expansion or dilatation of the dural sac, and has been frequently reported in association with type 1 neurofibromatosis (NF1). It should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma because NF-1 has a high risk of tumor formation. In the spinal deformities of NF-1 patients, despite the scoliosis is the most frequent finding, several distinctive radiographic features, such as dural ectasia, defective pedicles, and spondylolisthesis, are relatively less common. Surgery may be performed for stabilization of the spine. Here we reported a case: A 62-year-old female known case of NF1 Whom was referred for unusual chest X-ray and Chest CT findings of thoracic mass, seen while she was hospitalized and under observation because of probable corona diagnosis befor the pandemic of this virus become known in Iran. The chest X-ray film showed homogenous opacity with a well defined margin in the right apex. Chest CT showed an enlarged intervertebral foramen and defect of the vertebral arch around the mass and scoliosis. Though preoperative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of Dura mater with spinal fluid out of the spinal canal. Part of the wall was excised and the residual opening was carefully repaired, surgical stabilizing was done. Retrospectively, MRI showed characteristic findings of a thoracic dural ecstasy and anterior meningocele.