Clinical characteristics and therapeutic outcomes of mediastinal neuroblastoma with intraspinal extension: a retrospective study

Jue Tang; Dan Zhang; Ying-Yi Xu; Xin-Ke Xu; Feng-Hua Wang; Jia-Hang Zeng; Jiang-Hua Liang; Wei Liu; Le Li

doi:10.21037/tp-20-268

Clinical characteristics and therapeutic outcomes of mediastinal neuroblastoma with intraspinal extension: a retrospective study

Transl Pediatr. 2021 Apr;10(4):715-722. doi: 10.21037/tp-20-268.

Authors

Jue Tang¹, Dan Zhang², Ying-Yi Xu³, Xin-Ke Xu⁴, Feng-Hua Wang¹, Jia-Hang Zeng¹, Jiang-Hua Liang¹, Wei Liu¹, Le Li¹

Affiliations

¹ Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou, China.
² Reproductive Medicine Center, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
³ Department of Anesthesia, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou, China.
⁴ Department of Neurosurgery, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou, China.

Abstract

Background: Mediastinal neuroblastoma (NB) can invade the spinal canal and result in spinal cord compression. Some patients go on to develop severe spinal deformities after decompression of the spinal cord. The optimal therapeutic strategy for mediastinal NB with intraspinal extension is still unclear. Our study is to assess the therapeutic strategies for such patients.

Methods: A total of 77 patients suffered mediastinal tumors with intraspinal extension between March 2015 and Aug 2019 were enrolled in the study. According to the primary therapy, NB were classified into 4 groups: chemotherapy, video-assisted thoracoscopic surgery (VATS)/thoracotomy, neurosurgical decompression, and a combined thoracic-neurosurgical approach. Clinical features, including patient demographics, neurologic recovery and survival rate, were assessed.

Results: Among the 77 patients suffered mediastinal tumors with intraspinal extension, neurological symptoms were present in 44 patients. Neurological deficits improved in 76.5% of patients who underwent neurosurgical intervention and 50% of the other patients (P=0.094). Compression manifestations of ≤4 weeks duration showed an improved outcome compared to a longer compression time, with complete recovery of neurological function in 60% of patients versus 28.6% for patients with a longer symptom duration (P=0.04). NB constituted 49.4% of the 77 patients. An overall survival rate of 90.0%±9.5% was achieved for patients in the combined thoracic-neurosurgical group, 59.5%±15.0% in the thoracotomy group, 40.0%±29.7% in laminectomy group, and 37.0%±20.2% in the chemotherapy group. Complete regression of the tumor was demonstrated in 80% of combined group, which was greater than that of patients in the other groups (P=0.001).

Conclusions: Neurological recovery was correlated with the type of initial treatment and the duration of neurological symptoms. Mediastinal NB with intraspinal extension can be effectively managed with a combined neurosurgical and thoracic surgical approach.

Keywords: Neuroblastoma (NB); outcome; posterior mediastinal tumor; spinal cord compression; treatment.