Pathology of Peripheral Neuroblastic Tumors: Significance of Prominent Nucleoli in Undifferentiated/Poorly Differentiated Neuroblastoma

Tamás TORNÓCZKY1, Dávid SEMJÉN1, Hiroyuki SHIMADA2, Inge M AMBROS3

1Department of Pathology, Pécs University, Medical School, Pécs, Hungary
2Department of Pathology and Laboratory Medicine, Children’s Hospital Los Angeles/Keck School of Medicine, University of Southern California, Los Angeles, USA
3Children’s Cancer Research Institute, St. Anna Kinderkrebsforschung, Vienna, Austria


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 of MYCN amplification. Pathology & Oncology Research, Vol 13, Nr 4, 269-275, 2007

Key words: LCN; LNN; neuroblastoma; nucleoli; INPC; MYCN; amplification; ganglion cell; maturation; Schwann cell

Received: Sep 1, 2007; accepted: Nov 30, 2007
Correspondence: Tamás TORNÓCZKY, Department of Pathology, Pécs University, Medical School, Szigeti út 12. Pécs H-7643, Hungary; Tel: 36-72/324-122 / 5329, Fax: 36-72/336-621; E-mail:

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