Pathology & Oncology Research

, Volume 13, Issue 4, pp 269–275 | Cite as

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

  • Tamás Tornóczky
  • Dávid Semjén
  • Hiroyuki Shimada
  • Inge M Ambros


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.

Key words

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



large cell neuroblastoma


large nucleolar neuroblastoma


International Neuroblastoma Pathology Committee〉


peripheral neuroblastic tumors




neuroblastoma, undifferentiated


neuroblastoma, poorly differentiated


neuroblastoma, differentiating


ganglioneuroblastoma, intermixed type


ganglioneuroblastoma, nodular type




mitosis-karyorrhexis index


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Copyright information

© Arányi Lajos Foundation 2007

Authors and Affiliations

  • Tamás Tornóczky
    • 1
  • Dávid Semjén
    • 1
  • Hiroyuki Shimada
    • 2
  • Inge M Ambros
    • 3
  1. 1.Department of PathologyPécs University, Medical SchoolPécsHungary
  2. 2.Department of Pathology and Laboratory Medicine, Children’s Hospital Los Angeles/Keck School of MedicineUniversity of Southern CaliforniaLos AngelesUSA
  3. 3.Children’s Cancer Research InstituteSt. Anna KinderkrebsforschungViennaAustria

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