Ganglioneuroma is a rare, benign and well-differentiated neurogenic tumor, which is mostly localized in the posterior mediastinum . It usually grows very slow and displaces the surrounding anatomical structures without infiltration . 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 . 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.
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 42-year-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 1C).
Based on these findings, an extralobular PS was empirically diagnosed. Uniportal thoracoscopic resection of the sequestrated lung was scheduled and performed after a multidisciplinary evaluation. A thorough preoperative work-up including abdomen CT, and whole-body bone emission CT was conducted, which excluded the other suspicious lesions. Meanwhile, three-dimensional CT angiography (3D-CTA) was utilized , which clearly demonstrated the anomalous vessels of the PS (Fig. 1D).
Fast-track surgery protocol was utilized. The single-incision surgery was performed successfully as the margin of the lesion was easily identified. However, frozen-section stain showed the diagnosis of ganglioneurofibroma (Fig. 1E). R0 resection was achieved while the sampled mediastinal lymph nodes were tumor-negative. The operation time was 70 minutes, while the estimated blood loss was about 20 mL. Ultrasound-guided serratus anterior plane block using a bolus of liposomal bupivacaine was used for analgesia. In addition, postoperative chest drainage was avoided.
The pathological staining confirmed the diagnosis of ganglioneurofibroma arising within the extralobar PS (Fig. 1F); whereas the immunohistochemistry tests of the specimen indicated positive expression of SRY-related HMG-box 10 protein, neuron-specific enolase, S-100, chromogranin A and synuclein. The patient displayed an uneventful course and was discharged from the hospital on postoperative day 3. During the one year follow up, the patient reported satisfactory quality of life while residual pleural effusion or tumor recurrence was not identified.