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Peripheral neuroblastic tumours in eastern Denmark 1972–2002

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Zimling ZG, Rechnitzer C, Rasmussen M, Petersen BL. Peripheral neuroblastic tumours in eastern Denmark 1972–2002. APMIS 2007;115:66–74.

The peripheral neuroblastic tumour group includes neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Neuroblastoma is the most common extracranial solid tumour of childhood. We have evaluated the histological presentation, MYCN gene status, and clinical course of peripheral neuroblastic tumours diagnosed and treated in eastern Denmark from 1972–2002. 125 patients were diagnosed with peripheral neuroblastic tumour during this 30-year period. The histological material was reviewed and classified into three categories in accordance with the Shimada system: unfavourable histology, favourable histology, and benign tumours. MYCN status was determined by fluorescent in situ hybridization (FISH) on paraffin sections from the primary tumour. Clinical information was obtained from hospital records. Diagnostic likelihood ratios in the two groups were calculated to compare the ability of MYCN status and histological classification to predict 5-year outcome. 41 tumours showed unfavourable histology, 30 tumours showed favourable histology, 11 were benign, and 43 were unclassifiable due to limited amounts of primary tumour, bad preservation or inaccessibility of the primary tumour necessitating metastatic tumour biopsy for diagnosis. Unfavourable histology was associated with widespread disease (p<0.001). The overall 5-year survival rate was 45%, which correlates well with the European survival rate reported for this time period. The 5-year survival rate in the unfavourable group was 30% as compared to 100% in the favourable histology group (p<0.001). The survival rate in the unclassifiable group was 13%. 26% of the neuroblastomas were MYCN amplified. MYCN amplification was associated with undifferentiated histology, a histological subtype of the unfavourable histology group (p<0.001). For unfavourable histology the positive diagnostic likelihood ratio was 2.9 as compared to 4.7 for MYCN amplification. The study has confirmed the prognostic significance of the Shimada system in the peripheral neuroblastic tumour group in a retrospective material, and has also demonstrated the prognostic superiority of MYCN compared to histological classification, thus reducing the necessary amounts of tumour tissue.
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Keywords: MYCN; Neuroblastoma; ganglioneuroblastoma; ganglioneuroma; prognosis; survival

Document Type: Research Article

Affiliations: 1: Pathology and 2: Paediatrics, University Hospital of Copenhagen, Copenhagen, Denmark

Publication date: 2007-01-01

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