Ganglioneurofibroma Arising Within the Extralobar Pulmonary Sequestration

Abstract Background: Neurogenic tumor arising within the pulmonary sequestration (PS) is rare.Case Presentation: A 42-year-old asymptomatic female was referred to our hospital for work-up of extralobar PS. The independent feeding artery from the thoracic aorta was confirmed by three-dimensional computed tomography angiography (3D-CTA). Extrapulmonary lesions were excluded by whole-body radiographical examinations. Uniportal thoracoscopic resection of the sequestrated lung and mediastinal lymph node sampling was performed successfully. Finally, ganglioneurofibroma within the PS was confirmed by pathological staining. The patient was discharged from the hospital on the 3rd postoperative day without tumor recurrence a year after surgery.Conclusion: Preoperative 3D-CTA is useful to verify the aberrant vessels of PS; whereas an elaborate diagnostic work-up after a timely resection is necessary for possible subsequent management and follow-up plan.