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Pathology of peripheral neuroblastic tumors: Significance of prominent nucleoli in undifferentiated/poorly differentiated neuroblastoma

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Abstract

The presence of large cells having simultaneously increased cytoplasmic and nuclear volume accompanied by prominent nucleoli; i.e., differentiating neuroblasts and ganglion cells, is well documented in peripheral neuroblastic tumors (pNTs), and considered as one of the signs of tumor maturation and an indication of a better prognosis of the patients. On the other hand, in 2004 it was reported that large-cell neuroblastoma composed of neuroblastic cells with only nuclear enlargement without recognizable cytoplasmic maturation behaved poorly clinically. Here we are proposing a new pNT subtype in the neuroblastoma category, in addition to the undifferentiated, poorly differentiated and differentiating subtypes: that is large nucleolar neuroblastoma (LNN) characterized by large prominent nucleoli and no or very little amount of discernible cytoplasm. LNN, whose neuroblastic cells are often large in size due to nuclear enlargement, includes those tumors previously categorized into the large-cell neuroblastoma group. LNN tumors, regardless of the size of nuclei, seem to behave aggressively with a very poor prognosis of the patients. It is speculated that nucleolar enlargement without cytoplasmic maturation in LNN tumor cells can be a sign ofMYCN amplification.

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Abbreviations

LCN:

large cell neuroblastoma

LNN:

large nucleolar neuroblastoma

INPC:

International Neuroblastoma Pathology Committee〉

pNT:

peripheral neuroblastic tumors

NB:

neuroblastoma

NUD:

neuroblastoma, undifferentiated

NPD:

neuroblastoma, poorly differentiated

NDF:

neuroblastoma, differentiating

GNBi:

ganglioneuroblastoma, intermixed type

GNBn:

ganglioneuroblastoma, nodular type

GN:

ganglioneuroma

MKI:

mitosis-karyorrhexis index

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Correspondence to Tamás Tornóczky MD, PhD.

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Tornóczky, T., Semjén, D., Shimada, H. et al. Pathology of peripheral neuroblastic tumors: Significance of prominent nucleoli in undifferentiated/poorly differentiated neuroblastoma. Pathol. Oncol. Res. 13, 269–275 (2007). https://doi.org/10.1007/BF02940304

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Key words

  • LCN
  • LNN
  • neuroblastoma
  • nucleoli
  • INPC
  • MYCN
  • amplification
  • ganglion cell
  • maturation
  • Schwann cell