Tornóczky Tamás, Semjén Dávid, Shimada Hiroyuki, Ambros Inge M
Department of Pathology, Pécs University, Medical School, Pécs, H-7643, Hungary.
Pathol Oncol Res. 2007;13(4):269-75. doi: 10.1007/BF02940304. Epub 2007 Dec 25.
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.
外周神经母细胞瘤（pNTs）中存在大细胞，其细胞质和细胞核体积同时增大，伴有明显的核仁，即分化中的神经母细胞和神经节细胞，这一点已有充分记录，并被视为肿瘤成熟的标志之一以及患者预后较好的指标。另一方面，2004年有报道称，由仅细胞核增大而无明显细胞质成熟的神经母细胞组成的大细胞神经母细胞瘤临床行为较差。除了未分化、低分化和分化型亚型外，我们在此提出神经母细胞瘤类别中的一种新的pNT亚型：即大核仁神经母细胞瘤（LNN），其特征是有大而明显的核仁且无或仅有极少量可辨别的细胞质。LNN的神经母细胞通常因细胞核增大而体积较大，包括那些先前归类为大细胞神经母细胞瘤组的肿瘤。LNN肿瘤，无论细胞核大小如何，似乎都具有侵袭性，患者预后极差。据推测，LNN肿瘤细胞中无细胞质成熟的核仁增大可能是MYCN扩增的标志。
