Ganglioneurofibroma arising within the extralobar pulmonary sequestration
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(2020) 15:252
CASE REPORT
Open Access
Ganglioneurofibroma arising within the extralobar pulmonary sequestration Yuanyuan Liu1, Wenbin Wu2, Longbo Gong2 and Miao Zhang2*
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). Uniportal thoracoscopic resection of the sequestrated lung with mediastinal lymph node sampling was performed successfully. Ganglioneurofibroma within the PS was diagnosed as the specimen revealed positive expression of SRY-related HMG-box 10 protein, neuron-specific enolase, S-100, chromogranin A and synuclein. Tumor recurrence was not recorded 1 year after the surgery. Conclusion: Preoperative 3D-CTA is useful to identify the aberrant vessels of PS. An elaborate diagnostic work-up after a timely resection is necessary for subsequent management and follow-up plan. Keywords: Pulmonary sequestration (PS), Three-dimensional computed tomography angiography (3D-CTA), Uniportal, Single port, Video-assisted thoracoscopic surgery (VATS)
Background Ganglioneuroma is a rare, benign and well-differentiated neurogenic tumor, which is mostly localized in the posterior mediastinum [1]. It usually grows very slow and displaces the surrounding anatomical structures without infiltration [2]. To date, no specific serum biomarkers have been established for the diagnosis of neurogenic tumors. Pulmonary sequestration (PS) is mainly defined as a non-functioning lung tissue that has an unusual feeding artery mostly arising from the aorta, without certain pathological diagnostic criteria [3]. The etiology of PS is unknown. To our knowledge, neurogenic tumor originated in the sequestered lung is rare. Herein we presented a case of ganglioneurofibroma arising within an extralobar PS, followed by a brief literature review.
* Correspondence: [email protected] 2 Department of Thoracic Surgery, Xuzhou Central Hospital, 199 Jiefang South Road, Xuzhou 221009, China Full list of author information is available at the end of the article
Case presentation This report was approved by the Institutional Review Board of Xuzhou Central Hospital, and written informed consent was obtained from the patient. The clinical data was presented anonymously for privacy concern. A 42year-old asymptomatic female nonsmoker was admitted in June 2019 because the chest x-ray indicated a shadow in the left thorax (Fig. 1a). The blood tests showed that the tumor biomarkers such as carcinoembryonic antigen, alpha-fetoprotein, neuron-specific enolase, and cytokeratin-19 fragment were all in normal range. Further contrast-enhanced computed tomography (CT) revealed a homogenous mass located in the posterior mediastinum with a feeding artery from the thoracic aorta and an effluent vein into the left inferior pulmonary vein (Fig. 1b and c). Based on these findings, an e
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