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Information Journal Paper

Title

A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1

Pages

  107-112

Abstract

 Background and Importance: Dural ectasia is circumferential extension or dilatation of the dural sac, it is commonly associated with Neurofibromatosis type 1 (NF1). Because NF1 is associated with a high likelihood of tumor formation, it is critical to distinguish it from other posterior mediastinal cancers such as neurofibroma, neuroblastoma, and ganglioneuroma. Scoliosis is the most frequently observed feature in NF1 patients with spinal deformities. Moreover, there are several distinctive radiographic features, including Dural ectasia, defective pedicles, and spondylolisthesis, which are relatively less common in these patients. Surgery may be performed for stabilization of the spine. Case Presentation: The present study reports on a 62-year-old female with Neurofibromatosis type 1 who was referred for an unusual chest X-ray and chest Computed Tomography (CT) revealing a thoracic mass while she was hospitalized and under observation for dyspnea. The chest X-ray film showed homogenous opacity with a well-defined margin in the right apex. The chest CT showed an enlarged intervertebral foramen and defected vertebral arch around the mass and Scoliosis. Although the pre-operative 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, the residual opening was repaired, and surgical stabilization was performed. Conclusion: Retrospectively, magnetic resonance imaging showed the characteristics of thoracic Dural ectasia and anterior Meningocele, which, in an asymptomatic case, require regular radiographic follow-ups. Surgical intervention is an alternative for patients with spinal deformities or symptomatic patients due to the adverse effects of spinal cord compression and mediastinal structures.

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    APA: Copy

    ALIJANI, BABAK, Pakseresht Mogharab, Mahsa, & NASERI, AMIN. (2021). A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1. IRANIAN JOURNAL OF NEUROSURGERY, 7(2 (25)), 107-112. SID. https://sid.ir/paper/691292/en

    Vancouver: Copy

    ALIJANI BABAK, Pakseresht Mogharab Mahsa, NASERI AMIN. A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1. IRANIAN JOURNAL OF NEUROSURGERY[Internet]. 2021;7(2 (25)):107-112. Available from: https://sid.ir/paper/691292/en

    IEEE: Copy

    BABAK ALIJANI, Mahsa Pakseresht Mogharab, and AMIN NASERI, “A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1,” IRANIAN JOURNAL OF NEUROSURGERY, vol. 7, no. 2 (25), pp. 107–112, 2021, [Online]. Available: https://sid.ir/paper/691292/en

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