Zhang Xin,Jin Qianya,Yuan Xiaojun.Challenges and strategies in the diagnosis and treatment of neuroblastoma in adolescents and adults: one case report with a literature review[J].Journal of Clinical Pediatric Surgery,,22():625-629.[doi:10.3760/cma.j.cn101785-202306028-005]
Challenges and strategies in the diagnosis and treatment of neuroblastoma in adolescents and adults: one case report with a literature review
- Keywords:
- Neuroblastoma; Molecular Targeted Therapy; Surgical Procedures; Operative; Diagnosis; Therapy
- Abstract:
- Objective To explore the clinical characteristics, diagnosis, treatment and prognosis of neuroblastoma (NB) in adolescents and adults.Methods Retrospective review was conducted for a 24-year-old NB patient initially misdiagnosed as chromaffin cell tumor treated and followed-up at Xinhua Hospital.Diagnostic process, anti-disialoganglioside (GD2) monoclonal antibody immunotherapy and targeted treatment of anaplastic lymphoma kinase (ALK) inhibitor lorlatinib were examined.We conducted a literature review was performed by searching the databases of Wanfang and China National Knowledge Infrastructure (CNKI) using such keywords of "adolescent and adult neuroblastoma." Additionally, PubMed was searched using the keywords of "adolescent and neuroblastoma" or "adult and neuroblastoma." The search was conducted until December 2021.The authors excluded cases of NB in patients aged under 14 years and cases of primary intracranial NB with limited information.Results This 24-year-old boy was admitted due to an abdominal mass detected during a physical examination.Primary tumor in right adrenal gland with lymph node and bone metastasis was surgically resected.After multiple pathological reviews, imaging examinations and comprehensive analysis of biological characteristics, a definite diagnosis of NB was confirmed.Genetic testing revealed the presence of ALK R1275Q mutation.Surgery, chemoradiotherapy and co-treatment of ALK inhibitor and GD2-targeted therapy resulted in disease control.A total of 69 relevant articles involving 142 cases of adolescent/adult NB were retrieved.There were 62 boys and 80 girls with an average diagnostic age of 26.5 years.At the time of diagnosis, primary tumor was located in abdomen in 82 cases (57.8%).Sixty-seven cases (47.2%) had distant metastasis.The 5-year survival rate for children with localized lesions was 64.4% versus 15.7% for those with metastasis.Among 139 treated children, only 3 cases received targeted therapy.The median follow-up period was 21.5 month and the overall survival rate 41.6%.Conclusion Neuroblastoma is rare in adolescents and adults and originates predominantly in abdomen.It exhibits higher diagnostic heterogeneity as compared to pediatric NB and has a worse prognosis.Distant metastasis is an independent factor affecting the prognosis of adolescent/adult NB patients.Third-generation ALK inhibitors and GD2-targeted immunotherapy may be effective in this population.
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Memo
收稿日期:2023-06-15。
基金项目:国家自然科学基金（82002918）
通讯作者:袁晓军,Email:yuanxiaojun@xinhuamed.com.cn