Abstract
Objective:To explore the clinical manifestations, diagnosis, treatment, pathological types and prognosis of primary cervical neuroblastoma in children. Methods:The clinical data of 11 children with primary cervical neuroblastoma admitted to the Department of Otolaryngology and Head and Neck Surgery at Shanghai Children's Hospital from April 2015 to April 2022 were retrospectively analyzed. The clinical characteristics, imaging findings, pathological types, treatment methods, and outcomes of these 11 patients were examined in conjunction with a review of the literature. Results:The cohort of 11 neuroblastoma patients ranged in age from 28 days to 88 months （median age: 24 months）, including 3 males and 8 females. Among the 11 patients, 4 had tumors located in the carotid sheath area, 4 in the cervical space, and 3 in the parapharyngeal and retropharyngeal spaces. The clinical manifestations primarily included painless cervical masses and laryngeal stridor. There were 3 cases of primary cervical tumors with cervical lymph node metastasis and 1 case with bone marrow metastasis. Pathological findings revealed neuroblastoma in 8 cases and ganglionic neuroblastoma in 3 cases. In this group, 7 patients underwent surgery combined with chemotherapy, 2 patients received surgery combined with chemoradiotherapy, and 2 patients underwent surgery alone. Surgical resection was performed via a cervical approach in 7 cases, while 3 cases were treated using a transoral endoscopic approach. Additionally, one patient underwent a transoral endoscopic approach initially and a cervical approach subsequently. All patients completed their treatment and were followed up regularly, with follow-up durations ranging from 6 to 79 months （median: 34 months）. Nine patients achieved complete remission, 2 patients achieved partial remission, and none experienced disease progression. Conclusion:Primary cervical neuroblastoma exhibits a high degree of heterogeneity and presents at a younger age, primarily with cervical masses. Compared to external approaches, endoscopy-assisted transoral resection of parapharyngeal tumors offers advantages such as reduced damage to surrounding tissue and no visible neck scars, providing a new method for clinical treatment. Regular follow-up is essential for children with neuroblastoma, along with the establishment of specific disease management protocols and comprehensive care to improve survival quality.