Pathology of peripheral neuroblastic tumors: Significance of prominent nucleoli in undifferentiated/poorly differentiated neuroblastoma
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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 wordsLCN 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, poorly differentiated
ganglioneuroblastoma, intermixed type
ganglioneuroblastoma, nodular type
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- 2.Peuchmaur M, d’Amore ES, Joshi VV, Roald B, Dehner LP, Gerbing RB, Stram DO, Lukens JN, Matthay KK.Shimada H: Revision of the International Neuroblastoma Pathology Classification: confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer 98:2274–2281, 2003PubMedCrossRefGoogle Scholar
- 4.Shimada H, Umehara S, Monobe Y, Hachitanda Y, Nakagawa A, Goto S, Gerbing RB, Stram DO, Lukens JN, Matthay KK: International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors. A report from the Children’s Cancer Group. Cancer 92:2451–2461, 2001PubMedCrossRefGoogle Scholar
- 5.Shimada H, Chatten J, Newton WA Jr.,Sachs N, Hamoudi AB, Chiba T, Marsden HB, Misugi K: Histopathologic prognostic factors in neuroblastic tumors: definition of subtypes of ganglioneuroblastoma and an age-linked classification of neuroblastomas. J Natl Cancer Inst 73:405–416, 1984PubMedGoogle Scholar
- 8.Goto S, Umehara S, Gerbing RB, Stram DO, Brodeur GM, Seeger RC, Lukens JN, Matthay KK, Shimada H: Histopathology (International Neuroblastoma Pathology Classification) and MYCN status in patients with peripheral neuroblastic tumors. A report from the Children’s Cancer Group. Cancer 92:2699–2708, 2001PubMedCrossRefGoogle Scholar