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Palliation of an Unusual Fixed Airway Obstruction
Description
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A3185 - Palliation of an Unusual Fixed Airway Obstruction
Author Block: J. Harvey1, G. E. Abraham2; 1Pulmonary and Critical Care Medicine, University of Mississippi Medical Center, Jackson, MS, United States, 2University of Mississippi Medical Center, Jackson, MS, United States.
Introduction
The differential diagnosis of a fixed airway obstruction in a patient presenting with dyspnea and hoarseness is broad but includes malignancy, both primary and metastatic. However, a malignant peripheral nerve sheath tumor is a very uncommon malignancy to cause this complication. We present a case of a malignant peripheral nerve sheath tumor causing a fixed airway obstruction, which was palliated with tracheal stent placement.
Case Report
A 39-year-old man with neurofibromatosis type 1 (NF1) presented with dyspnea and hoarseness. A flow-volume loop demonstrated flattening of the expiratory and inspiratory limbs, consistent with a fixed airway obstruction. Computed tomography of the thorax revealed a 16cm right apical mass encroaching on the mediastinum and causing extrinsic tracheal and superior vena caval compression. A flexible bronchoscopy with transbronchial needle aspirations revealed a high grade spindle cell sarcoma. In the setting of NF1, this is suspicious for a malignant peripheral nerve sheath tumor. A CRETM balloon dilator (Boston Scientific) was utilized to demonstrate that the trachea could be adequately dilated and could easily accommodate a tracheal stent without further worsening the obstruction. A 16 x 80mm covered metal stent (Merit Medical Endotek) was placed with immediate improvement. Repeat spirometry revealed complete resolution of the obstruction.
Discussion
A malignant peripheral nerve sheath tumor, or neurofibrosarcoma, is a rare soft tissue tumor occurring in only 10 percent of patients with NF1 and in only 0.001 percent of the general population. The most common location of such a tumor is an extremity, followed by the trunk, with intrathoracic or mediastinal tumors being very rare. The preferred treatment is wide local surgical resection and adjuvant radiation therapy. Unfortunately, even with aggressive treatment, the prognosis is poor with a 5 year survival as low as 16 percent. Tumor size greater than 5cm, higher tumor grade, association with NF1, and distant metastases portend a poor prognosis. Our patient presented with a rare tumor, poor prognostic signs, and a rare complication of fixed airway obstruction which was successfully palliated with placement of a covered metal stent.
Author Block: J. Harvey1, G. E. Abraham2; 1Pulmonary and Critical Care Medicine, University of Mississippi Medical Center, Jackson, MS, United States, 2University of Mississippi Medical Center, Jackson, MS, United States.
Introduction
The differential diagnosis of a fixed airway obstruction in a patient presenting with dyspnea and hoarseness is broad but includes malignancy, both primary and metastatic. However, a malignant peripheral nerve sheath tumor is a very uncommon malignancy to cause this complication. We present a case of a malignant peripheral nerve sheath tumor causing a fixed airway obstruction, which was palliated with tracheal stent placement.
Case Report
A 39-year-old man with neurofibromatosis type 1 (NF1) presented with dyspnea and hoarseness. A flow-volume loop demonstrated flattening of the expiratory and inspiratory limbs, consistent with a fixed airway obstruction. Computed tomography of the thorax revealed a 16cm right apical mass encroaching on the mediastinum and causing extrinsic tracheal and superior vena caval compression. A flexible bronchoscopy with transbronchial needle aspirations revealed a high grade spindle cell sarcoma. In the setting of NF1, this is suspicious for a malignant peripheral nerve sheath tumor. A CRETM balloon dilator (Boston Scientific) was utilized to demonstrate that the trachea could be adequately dilated and could easily accommodate a tracheal stent without further worsening the obstruction. A 16 x 80mm covered metal stent (Merit Medical Endotek) was placed with immediate improvement. Repeat spirometry revealed complete resolution of the obstruction.
Discussion
A malignant peripheral nerve sheath tumor, or neurofibrosarcoma, is a rare soft tissue tumor occurring in only 10 percent of patients with NF1 and in only 0.001 percent of the general population. The most common location of such a tumor is an extremity, followed by the trunk, with intrathoracic or mediastinal tumors being very rare. The preferred treatment is wide local surgical resection and adjuvant radiation therapy. Unfortunately, even with aggressive treatment, the prognosis is poor with a 5 year survival as low as 16 percent. Tumor size greater than 5cm, higher tumor grade, association with NF1, and distant metastases portend a poor prognosis. Our patient presented with a rare tumor, poor prognostic signs, and a rare complication of fixed airway obstruction which was successfully palliated with placement of a covered metal stent.
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