Pleomorphic anaplastic neuroblastoma

Pleomorphic anaplastic neuroblastoma (PAN) is a striking aspect of neuroblastoma first described by Cozzutto and Carbone in 1988.[1] Another case was thereafter reported by Cowan, et al. with cytogenetic and immunohistological analysis in a 28-year-old man.[2] The case described by Navarro, et al. showed MYCN amplification (more than 10 copies) and a 1p36 deletion as measured with FISH in 13% of cells.[3] Additionally there was a main cell population with a DNA index of 2 indicating a tetraploid DNA content and a high expression of MIBI (Ki-67), bel 2, p53, and P-glycoprotein, either correlated with rapid progression of disease.

Histopathology

Appearance of focal or diffuse presence of pleomorphic and anaplastic cells is the hallmark of this neuroblastoma variant.[1][4][5] Hyperchromatic and bizarre nuclei in neuroblasts and ganglion cells in undifferentiated or poorly differentiated foci are far too pathognomonic, although anaplastic expressions can be found in differentiating foci alike.[1][4][5] Undifferentiated neuroblasts may show enlarged and vesicular nuclei with thick nuclear membranes and prominent nucleoli, often a clue to forthcoming anaplastic change.

Controversy

Whether pleomorphism-anaplasia warrants a worse prognosis has not been fully assessed so far. Chatten argued that increasing anaplasia in stage IV in comparison with stage III found in her cases suggested a trend toward higher stage (and worse prognosis) with anaplasia.[6] However, within-stage differences were not significant, although cases without anaplasia fared better in each stage than those with. Abramowsky's et al. series of 7 children with anaplastic large cell neuroblastoma showed a better survival than previously reported cases.[7] Joshi, et al. did not find any meaningful difference in clinical outcome between cases with or without anaplasia.[8] Dehner pointed out the importance of recognizing this variant for differential diagnostic purposes.[9] Expressions of pleomorphism-anaplasia have been fully acknowledged among the multifarious features of neuroblastoma in the classification by the International Neuroblastoma Pathology Committee.[10] Recently Tornòczky et al. described 7 cases of large cell neuroblastoma characterized by larger nuclei with thick nuclear membranes and prominent nucleoli suggesting they might represent a distinct phenotype of neuroblastoma with aggressive clinical behavior.[11]

References
  1. Cozzutto C, Carbone A (1988). Pleomorphic (Anaplastic) neuroblastoma. Arch Pathol Lab Med 112:621-625.
  2. Cowan J, Dayal Y, Schaitzberg S, Tischler AS (1997). Cytogenetic and immunohistological analysis of an adult anaplastic neuroblastoma. Am J Surg Pathol 21(8):957-963.
  3. Navarro S, Noguera R, Pellin A, Mejia C, Ruiz A, Llombart-Bosch A (2000). Pleomorphic anaplastic neuroblastoma. Med Pediat Oncol 35:449-502.
  4. Fletcher CDM (2007). Diagnostic Histopatholoy of tumors. Churchill Livingstone, p. 1774. Rosai (2004).
  5. Rosai and Ackerman's Surgical Pathology. Philadelphia, Mosby, p.1128.
  6. Chatten J (1989). Anaplastic neuroblastomas. Letter to the editor. Arch Pathol Lab Med 113:9-10.
  7. Abramowsky CR, Katzenstein HM, Alvarado CS, Shehata BM (2009). Anaplastic large cell neuroblastoma. Pediat Devel Pathol 12(1):1-5.
  8. Joshi VV, Cantor AB, Altshuler G, Larkin EW et al. (1992). Age-linked prognostic categorization based on a new histologic grading system of neuroblastomas. A clinicopathologic study of 211 cases from the pediatric oncology group. Cancer 69:2197-2211.
  9. Dehner LP (1989). Anaplasia in solid malignant tumors of childhood. Arch Pathol Lab Med 113:11-12.
  10. Shimada H, Ambros IM, Dehner LP, Joshi VV, Roald B (1999). Terminology and morphologic criteria of neuroblastic tumors. Recommendations by the International Neuroblastoma Pathology Committee. Cancer 86(2):349-363.
  11. Tornòczky T, Kàlmàn E, Kajtàr PG, Nyàri T, Pearson AD, Tweddle DA, Board J, Shimada H (2004) Large cell neuroblastoma. A distinctive phenotype of neuroblastoma with aggressive clinical behavior. Cancer 100(2):390-397.
  • Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B (1999). Terminology and morphologic criteria of neuroblastic tumors. Recommendations by the International Neuroblastoma Pathology Committee. Cancer 86(2) 349–363.
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