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Open Access Research article

Absence of polysialylated NCAM is an unfavorable prognostic phenotype for advanced stage neuroblastoma

Miikka Korja1*, Anne Jokilammi1, Toivo T Salmi2, Hannu Kalimo3, Tarja-Terttu Pelliniemi4, Jorma Isola5, Immo Rantala6, Hannu Haapasalo6 and Jukka Finne1

Author Affiliations

1 Department of Medical Biochemistry and Genetics, University of Turku, Turku, Finland

2 Department of Pediatrics, Turku University Hospital, Turku, Turku, Finland

3 Department of Pathology, Turku University Hospital, Turku, Turku, Finland

4 Department of Clinical Chemistry, Turku University Hospital, Turku, Finland

5 Laboratory of Cancer Biology, Institute of Medical Technology, University of Tampere and Tampere University Hospital, Tampere, Finland

6 Department of Pathology, Tampere University Hospital, Tampere, Finland

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BMC Cancer 2009, 9:57  doi:10.1186/1471-2407-9-57

Published: 17 February 2009

Abstract

Background

The expression of a neural crest stem cell marker, polysialic acid (polySia), and its main carrier, neural cell adhesion molecule (NCAM), have been detected in some malignant tumors with high metastatic activity and unfavorable prognosis, but the diagnostic and prognostic value of polySia-NCAM in neuroblastoma is unclear.

Methods

A tumor tissue microarray (TMA) of 36 paraffin-embedded neuroblastoma samples was utilized to detect polySia-NCAM expression with a polySia-binding fluorescent fusion protein, and polySia-NCAM expression was compared with clinical stage, age, MYCN amplification status, histology (INPC), and proliferation index (PI).

Results

PolySia-NCAM-positive neuroblastoma patients had more often metastases at diagnosis, and polySia-NCAM expression associated with advanced disease (P = 0.047). Most interestingly, absence of polySia-NCAM-expressing tumor cells in TMA samples, however, was a strong unfavorable prognostic factor for overall survival in advanced disease (P = 0.0004), especially when MYCN was not amplified. PolySia-NCAM-expressing bone marrow metastases were easily detected in smears, aspirates and biopsies.

Conclusion

PolySia-NCAM appears to be a new clinically significant molecular marker in neuroblastoma, hopefully with additional value in neuroblastoma risk stratification.