Prognostic value of the International Neuroblastoma Pathology Classification in Neuroblastoma (Schwannian stroma-poor) and comparison with other prognostic factors: a study of 182 cases from the Spanish Neuroblastoma Registry |
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Authors: | Octavio Burgues Samuel Navarro Rosa Noguera Antonio Pellín Amparo Ruiz Victoria Castel Antonio Llombart-Bosch |
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Institution: | 1. Departamento de Patología, Facultad de Medicina de Valencia, Avda. Blasco Iba?ez, 17, E-46010, Valencia, Spain 2. Pediatric Oncology Unit, Hospital Universitario “La Fe”, Valencia, Spain
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Abstract: | In addition to clinical and biological factors, further valuable prognostic information in neuroblastoma (Schwannian stroma-poor)
(NB) patients is provided by the histopathologic analysis and the application of the International Neuroblastoma Pathology
Classification (INPC) system. The objective of this study was to assess the prognostic impact of the INPC classification in
a series of NB (Schwannian stroma-poor) and its relation with other prognostic factors. One hundred eighty-two cases of NB
were collected from the files of the Spanish Neuroblastoma Registry. Slides were reviewed, and NB cases were grouped into
favorable and unfavorable categories according to INPC criteria, taking into account morphological features (mitosis–karyorrhexis
index, histological subtype) and patient’s age at diagnosis. Other pathological presence of calcifications, tissular components,
and number of mitotic cells per 10 high-power field (HPF)], immunohistochemical (P-glycoprotein and Ki-67 protein expression)
and genetic (MYCN amplification and chromosome 1p deletion) features were also studied. Statistical analyses of overall survival
with Kaplan–Meier curves and a multivariate study using Cox regression were performed (40.3% of NBs were considered favorable
and 59.7% unfavorable). Unfavorable NB showed a mean survival time of 57 months compared with 89 months in favorable cases.
Advanced stage, more than ten mitoses per 10 HPF, Ki-67 expression in more than 30% of tumor cells, MYCN oncogene amplification
and chromosome 1p deletion were observed more frequently in unfavorable NB. The Cox regression analysis demonstrated that
clinical stage (International Neuroblastoma Staging System stage 4) and histological subtype (undifferentiated NB) were the
most important factors that influence the overall survival (p<0.001). INPC classification results are major prognostic indicators in NB and should be considered in the therapeutic stratification
of NB patients. |
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Keywords: | Neuroblastoma Neuroblastic tumors Pediatric tumors |
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