Shah S, Ravindranath Y
Children's Hospital of Michigan, Department of Pediatric Hematology/Oncology, Detroit 48201, USA.
Indian J Pediatr. 1998 Sep-Oct;65(5):691-705. doi: 10.1007/BF02731044.
The neuroblastic tumours originate from primordial neural crest cells that normally develop into sympathetic nervous system, including the adrenal medulla. Neuroblastoma is the most intriguing pediatric neoplasm displaying diverse clinical and biologic characteristics and natural history. It has the highest rate of spontaneous regression of all human cancers, yet exhibits extremely malignant behaviour in older children with regional and disseminated disease. In the last 30 years, only a nominal improvement has occurred in the outlook of older children with metastatic disease at diagnosis. Tremendous gains in understanding of the biology of neuroblastoma in recent years have led to development of risk-related therapy based on age, stage and biological characteristics of neuroblastoma.
神经母细胞瘤起源于原始神经嵴细胞,这些细胞通常发育成交感神经系统,包括肾上腺髓质。神经母细胞瘤是最具吸引力的儿科肿瘤,具有多样的临床和生物学特征以及自然病程。它是所有人类癌症中自发消退率最高的,但在患有局部和播散性疾病的大龄儿童中却表现出极其恶性的行为。在过去30年里,诊断时患有转移性疾病的大龄儿童的预后仅出现了微不足道的改善。近年来对神经母细胞瘤生物学的深入了解促使基于神经母细胞瘤的年龄、分期和生物学特征开发了风险相关疗法。