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Belgorod State National Research University
Department: Pathological Anatomy.
Topic: Childhood Tumors.
Compiled by:
N. K. Gaur
Small,Round, Blue Cell Tumors in Childhood.
 Primitive appearance (not anaplastic or pleomorphic)
 Sheets of small,round,blue cells (with dark nuclei,scant
cytoplasm,indistinct borders.)
 May show features of organogenesis specific to the tissue
of origin.
Neuroblastoma
 Embryonal malignant tumour
 Neural crest origin
 Neoplastic neuroblasts
 Site: adrenal medulla &sympathetic ganglia
 7-10% of solid paediatric malignancies.
 Sporadic occurance.
 Rarely familial (bilateral,multifocal)
Pathology of Neuroblastoma.
Site :Paravertebral, Posterior mediastinum, abdomen ,Adrenal
1/3
Gross appearance:
Nodular, of varying size
May be encapsulated or infiltrative
Cut section: grey-tan, soft and friable
Varigated, necrosis, hemorrhage, calcification, cystic change
Microscopy of Neuroblastoma
Sheets of small, round, blue cells with dark nuclei, scant cytoplasm,
indistinct borders.
Mitosis++, Karyorrhectic debris +
Pleomorphism +/-
Homer-Wright rosettes, Neuropil.
Maturation: Schwann cell, stroma &ganglion cell differentiation.
Microscopy of Neuroblastoma.
Clinical features.
 Abdominal mass, fever
 Blueberry muffin
 Wide metastasis
 Secrete catecholamines
 Vanillylmandelic acid (VMA)/Homovanillic acid (HVA) screening.
Prognosis
 Stage
 spread to regional lymph nodes,liver,lungs,bones etc
 Age :< 1 yr.
 Morphology –gangliocytic differentiation better
 MYCN (N myc) gene amplification-worse.
Retinoblastoma
 Malignant tumour of the eye in childhood
 Neuroepithelial origin –posterior retina
 Familial,- 60-70%, associated with germ line mutation, heritable.
 Sporadic:30-40%,somatic gene mutation.
 Associated with Rb 1 gene
 Secondary malignancy –osteosarcoma
RB gene
 RB gene is on chromosome 13
 RB gene function is the most critical checkpoint in the cell cycle and
allows the cell to enter from
G1 to S
 Tumour supressor gene
 If both RB genes are abnormal i.e. mutated or have a missing allele, it
permits unregulated cell proliferation.
 Knudson’s two-hit hypothesis
 People with RB mutations are susceptibe to malignancies especially
osteosarcoma
Morphology of retinoblastoma.
 Gross: occular masses.
 Microscopy: Sheets of small,round,blue cells with dark nuclei,scant
cytoplasm,indistinct borders
 Flexner-Wintersteiner rosettes.
Morphology of retinoblastoma.
Behaviour .
 Spread through optic nerve or to subarachnoid space to CNS, bone,
lymph nodes.
 Cure with treatment.
 Spontaneous cure.
 Second malignancy.
Childhood cancers

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Childhood cancers

  • 1. Belgorod State National Research University Department: Pathological Anatomy. Topic: Childhood Tumors. Compiled by: N. K. Gaur
  • 2. Small,Round, Blue Cell Tumors in Childhood.  Primitive appearance (not anaplastic or pleomorphic)  Sheets of small,round,blue cells (with dark nuclei,scant cytoplasm,indistinct borders.)  May show features of organogenesis specific to the tissue of origin.
  • 3.
  • 4. Neuroblastoma  Embryonal malignant tumour  Neural crest origin  Neoplastic neuroblasts  Site: adrenal medulla &sympathetic ganglia  7-10% of solid paediatric malignancies.  Sporadic occurance.  Rarely familial (bilateral,multifocal)
  • 5.
  • 6.
  • 7. Pathology of Neuroblastoma. Site :Paravertebral, Posterior mediastinum, abdomen ,Adrenal 1/3 Gross appearance: Nodular, of varying size May be encapsulated or infiltrative Cut section: grey-tan, soft and friable Varigated, necrosis, hemorrhage, calcification, cystic change
  • 8. Microscopy of Neuroblastoma Sheets of small, round, blue cells with dark nuclei, scant cytoplasm, indistinct borders. Mitosis++, Karyorrhectic debris + Pleomorphism +/- Homer-Wright rosettes, Neuropil. Maturation: Schwann cell, stroma &ganglion cell differentiation.
  • 10. Clinical features.  Abdominal mass, fever  Blueberry muffin  Wide metastasis  Secrete catecholamines  Vanillylmandelic acid (VMA)/Homovanillic acid (HVA) screening.
  • 11. Prognosis  Stage  spread to regional lymph nodes,liver,lungs,bones etc  Age :< 1 yr.  Morphology –gangliocytic differentiation better  MYCN (N myc) gene amplification-worse.
  • 12. Retinoblastoma  Malignant tumour of the eye in childhood  Neuroepithelial origin –posterior retina  Familial,- 60-70%, associated with germ line mutation, heritable.  Sporadic:30-40%,somatic gene mutation.  Associated with Rb 1 gene  Secondary malignancy –osteosarcoma
  • 13. RB gene  RB gene is on chromosome 13  RB gene function is the most critical checkpoint in the cell cycle and allows the cell to enter from G1 to S  Tumour supressor gene  If both RB genes are abnormal i.e. mutated or have a missing allele, it permits unregulated cell proliferation.  Knudson’s two-hit hypothesis  People with RB mutations are susceptibe to malignancies especially osteosarcoma
  • 14.
  • 15. Morphology of retinoblastoma.  Gross: occular masses.  Microscopy: Sheets of small,round,blue cells with dark nuclei,scant cytoplasm,indistinct borders  Flexner-Wintersteiner rosettes.
  • 17. Behaviour .  Spread through optic nerve or to subarachnoid space to CNS, bone, lymph nodes.  Cure with treatment.  Spontaneous cure.  Second malignancy.