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Surgical Outcomes of Patients with Neuroblastoma in a Tertiary Centre in Hong Kong: A 12-year Experience

机译:香港第三专科医院神经母细胞瘤患者的手术结局:12年的经验

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Introduction: Neuroblastoma has a heterogeneous clinical course. The prognosis varies widely depending on the age of diagnosis, extent of disease and tumour biology. However, the specific clinical outcome of this disease in Hong Kong has not been well characterised thus far. Complete tumour excision has been demonstrated to confer survival benefit on patients with advanced disease even if there is metastasis. Since year 2004, we have adopted a revised, more aggressive surgical approach in managing these patients. Here, we aim to review our experience in the management of this disease. Methods: A retrospective review was performed for the past 12 years to include all patients who presented with neuroblastoma in our institution. Data such as the survival, age at diagnosis, MYCN amplification status, the extent of tumour excision, and stage of the disease were recorded and analysed. Results: 37 patients were included in this study. Overall survival of our patients was 67.6%. Patients with Stage 1, 2 and 4S have 100% survival whereas stage 4 patients only have 41.4% survival. Since our revised surgical approach in 2004, patients who had been operated had a better survival. Survival of stage 4 patients with operation after 2004 was 57.1% whereas the survival of patients at the same stage before 2004 was only 30%. Age at diagnosis, completeness of tumour excision and stage of disease are also correlated with overall prognosis. Further, patients with the presence of MYCN gene amplification have apparently poorer survival but it is not statistically significant due to the small sample size. Conclusion: The management of patients with neuroblastoma remains a challenge. Advanced stage of disease, incomplete tumour excision and increased age at diagnosis were all associated with poor survival. We demonstrated a better survival for those who underwent a more aggressive surgical approach, though this is a technically demanding and time consuming procedure. Thus, the management of advanced neuroblastoma should be centralised in a centre with combined surgical, oncological and paediatric intensive care expertise.
机译:简介:神经母细胞瘤具有不同的临床过程。预后因诊断年龄,疾病范围和肿瘤生物学而异。但是,到目前为止,该疾病在香港的具体临床结局尚未明确。事实证明,即使存在转移,完全切除肿瘤也可以使晚期疾病患者获得生存获益。自2004年以来,我们采用了经过修订的更具侵略性的手术方法来管理这些患者。在这里,我们旨在回顾我们在控制该疾病方面的经验。方法:回顾性回顾了过去12年中我们机构中所有出现神经母细胞瘤的患者。记录并分析诸如存活率,诊断时的年龄,MYCN扩增状态,肿瘤切除的程度以及疾病的阶段等数据。结果:37例患者被纳入本研究。我们患者的总生存率为67.6%。具有1、2和4S期的患者生存率为100%,而具有4S期的患者仅为41.4%。自2004年修订手术方法以来,手术患者的生存率更高。 2004年之后手术的4期患者的生存率为57.1%,而2004年之前同一阶段的患者的生存率仅为30%。诊断时的年龄,肿瘤切除的完整性和疾病阶段也与总体预后相关。此外,存在MYCN基因扩增的患者的生存期明显较差,但由于样本量小,因此在统计学上不显着。结论:神经母细胞瘤患者的治疗仍然是一个挑战。疾病晚期,肿瘤切除不完全和诊断时年龄增加均与不良的生存率相关。尽管这是一项技术要求高且耗时的过程,但我们证明了接受更积极的手术治疗的患者的生存率更高。因此,晚期神经母细胞瘤的管理应集中在具有外科,肿瘤学和儿科重症监护专业知识相结合的中心。

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