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Journal of Pediatric Biochemistry 2015; 05(04): 131-138
DOI: 10.1055/s-0036-1572525
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Neuroblastoma: Diagnostic and Clinical Aspects

Silvia Marino
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Federica Puglisi
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Gaetano Magro
2   Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia,” Hospital Policlinico, University of Catania, Catania, Italy
,
Giuseppe Belfiore
3   Unit of Radiology, Hospital Policlinico, University of Catania, Catania, Italy
,
Vincenzo Di Benedetto
4   Unit of Paediatric Surgery, Hospital Policlinico, University of Catania, Catania, Italy
,
Maria Grazia Scuderi
3   Unit of Radiology, Hospital Policlinico, University of Catania, Catania, Italy
,
Anna Portale
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Salvatore D'Amico
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Vito Miraglia
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Maria Licciardello
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Luca Lo Nigro
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Milena La Spina
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Giovanna Russo
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
,
Andrea Di Cataldo
1   Unit of Paediatric Haematology and Oncology, Department of Clinical and Experimental Medicine, Hospital Policlinico, University of Catania, Catania, Italy
› Author Affiliations
Further Information

Publication History

21 December 2015

30 December 2015

Publication Date:
13 February 2016 (online)

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Abstract

Due to its many clinical and biologic characteristics, neuroblastoma (NB) is a polyhedric neoplasm. It is a very complex, extremely heterogeneous disease that can regress spontaneously even without therapy. However, it frequently shows a very aggressive behavior, refractory to current intensive multimodal therapy. The tumor, originating from primordial neural crest cells, is biochemically unique for its metabolic pathways of catecholamine synthesis and metabolism. Homovanillic acid (HVA), the main metabolite of dopamine, and vanillylmandelic acid (VMA), the main metabolite of adrenalin and noradrenalin, are sensitive and convenient markers of NB since they are found in large amounts in patient's urine. The rate of NB patients with positive VMA and/or HVA at the diagnosis varies with the stage of the disease, with high-stage tumors being more likely to have abnormal levels. Other less specific NB tumor serum markers are neuron-specific enolase, ferritin, and lactate dehydrogenase. NB is one of the few pediatric tumors in which tumor markers have been shown to have a role in the diagnosis, prognosis, and disease monitoring.

Keywords

neuroblastoma - homovanillic acid - vanillylmandelic acid - neuron-specific enolase - ferritin - lactate dehydrogenase
 

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