What is the Shimada histopathologic classification system of pediatric neuroblastoma?

Updated: Oct 09, 2017
  • Author: Norman J Lacayo, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

Shimada et al developed a histopathologic classification in patients with neuroblastoma. [3] This classification system was retrospectively evaluated and correlated with outcome in 295 patients with neuroblastoma who were treated by the Children's Cancer Group (CCG). Important features of the classification include (1) the degree of neuroblast differentiation, (2) the presence or absence of Schwannian stromal development (stroma-rich, stroma-poor), (3) the index of cellular proliferation (known as mitosis-karyorrhexis index [MKI]), (4) nodular pattern, and (5) age. Using these components, patients can be classified into the following histology groups:

Favorable histology group includes the following:

  • Patients of any age with stroma-rich tumors without a nodular pattern

  • Patients younger than 18 months with stroma-poor tumors, an MKI of less than 200/5000 (200 karyorrhectic cells per 5000 cells scanned), and differentiated or undifferentiated neuroblasts

  • Patients younger than 60 months with stroma-poor tumors, an MKI of less than 100/5000, and well-differentiated tumor cells

Unfavorable histology group includes the following:

  • Patients of any age with stroma-rich tumors and a nodular pattern

  • Patients of any age with stroma-poor tumors, undifferentiated or differentiated neuroblasts, and an MKI more than 200/5000

  • Patients older than 18 months with stroma-poor tumors, undifferentiated neuroblasts, and an MKI more than 100/5000

  • Patients older than 18 months with stroma-poor tumors, differentiated neuroblasts, and an MKI of 100-200/5000

  • Patients older than 60 months stroma-poor, differentiated neuroblasts, and an MKI less than 100

Shimada et al’s original classification was adopted and integrated into the International Neuroblastoma Pathology Classification (INPC). This was most recently revised. [4] The INPC system remains age-dependent.


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