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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Cellular Classification

The International Neuroblastoma Pathologic Classification (INPC) system involves evaluation of tumor specimens obtained prior to therapy for the amount of stromal development, the degree of neuroblastic maturation, and the mitosis-karyorrhexis index of the neuroblastic cells.[1,2,3,4] Favorable and unfavorable prognoses are defined on the bases of these histologic parameters. The prognostic significance of this classification system, and of related systems using similar criteria, has been confirmed in several studies.[1,2,3] Neuroblastoma containing many differentiating cells, termed ganglioneuroblastoma, can have nodules of undifferentiated cells whose histology, along with MYCN amplification, determines prognosis.[4,5] About 25% of reported neuroblastomas diagnosed in the fetus and neonate are cystic; cystic neuroblastomas have lower stages and a higher incidence of favorable biology.[6]

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  1. Shimada H, Ambros IM, Dehner LP, et al.: The International Neuroblastoma Pathology Classification (the Shimada system). Cancer 86 (2): 364-72, 1999.
  2. Shimada H, Umehara S, Monobe Y, et al.: International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group. Cancer 92 (9): 2451-61, 2001.
  3. Goto S, Umehara S, Gerbing RB, et al.: Histopathology (International Neuroblastoma Pathology Classification) and MYCN status in patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group. Cancer 92 (10): 2699-708, 2001.
  4. Peuchmaur M, d'Amore ES, Joshi VV, et al.: Revision of the International Neuroblastoma Pathology Classification: confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer 98 (10): 2274-81, 2003.
  5. Kubota M, Suita S, Tajiri T, et al.: Analysis of the prognostic factors relating to better clinical outcome in ganglioneuroblastoma. J Pediatr Surg 35 (1): 92-5, 2000.
  6. Isaacs H Jr: Fetal and neonatal neuroblastoma: retrospective review of 271 cases. Fetal Pediatr Pathol 26 (4): 177-84, 2007 Jul-Aug.
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Last Updated: February 25, 2014
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