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成人转移性肾上腺神经母细胞瘤。

Metastatic adrenal neuroblastoma in an adult.

作者信息

Schalk Enrico, Mohren Martin, Koenigsmann Michael, Buhtz Peter, Franke Astrid, Jentsch-Ullrich Kathleen

机构信息

Klinik für Hämatologie/Onkologie, Germany.

出版信息

Onkologie. 2005 Jun;28(6-7):353-5. doi: 10.1159/000085526. Epub 2005 Jun 2.

Abstract

BACKGROUND

Neuroblastoma (NB) is a common malignancy in children, but rarely occurs in adults. Accepted unfavourable prognostic factors include age > 1 year, low histologic grade and advanced stage, MYCN amplification, chromosomal aberrations, elevations of neuron specific enolase and lactate dehydrogenase, and increased catecholamine metabolites in urine or serum. In adults, abdomen/retroperitoneum are the primary sites and in children the adrenal gland.

CASE REPORT

A 51- year-old man was admitted to our hospital with hypertension and a large right retroperitoneal mass. Clinically, phaeochromocytoma was suspected. Tumour resection revealed adrenal NB grade III. Chemotherapy according to the paediatric German Neuroblastoma Trial (NB97) was started. Follow-up computed tomography showed regression of the enlarged mediastinal and retroperitoneal lymph nodes. Because of local and systemic progression palliative radiochemotherapy was started. The patient died 9 months after diagnosis.

CONCLUSION

To the best of our knowledge this is the oldest NB patient registered so far in Germany. Currently there are no standard treatment guidelines for patients with NB in adulthood. Collection and evaluation of data in adult patients with this tumour are warranted in order to optimise treatment strategies.

摘要

背景

神经母细胞瘤(NB)是儿童常见的恶性肿瘤,但在成人中很少发生。公认的不良预后因素包括年龄大于1岁、低组织学分级、晚期、MYCN扩增、染色体畸变、神经元特异性烯醇化酶和乳酸脱氢酶升高,以及尿或血清中儿茶酚胺代谢产物增加。在成人中,腹部/腹膜后是主要发病部位,而在儿童中是肾上腺。

病例报告

一名51岁男性因高血压和右侧腹膜后巨大肿块入院。临床上怀疑为嗜铬细胞瘤。肿瘤切除显示为肾上腺III级NB。开始按照德国儿童神经母细胞瘤试验(NB97)进行化疗。随访计算机断层扫描显示肿大的纵隔和腹膜后淋巴结缩小。由于局部和全身进展,开始进行姑息性放化疗。患者在诊断后9个月死亡。

结论

据我们所知,这是德国迄今登记的年龄最大的NB患者。目前对于成年NB患者尚无标准治疗指南。有必要收集和评估成年NB患者的数据,以优化治疗策略。

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