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Neoplasms of infancy and childhood

Neoplasms of infancy and childhood. Neoplasms of infancy and childhood. Benign>malignant Incidence of malignancy:1-15 yrs - 1.3 /10,000 /year but leading cause of death after accidents in the West. (developing countries??)

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Neoplasms of infancy and childhood

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  1. Neoplasms of infancy and childhood

  2. Neoplasms of infancy and childhood • Benign>malignant • Incidence of malignancy:1-15 yrs - 1.3 /10,000 /year but leading cause of death after accidents in the West. (developing countries??) • Most malignant tumours in children arise from hematopoietic,nervous and soft tissues (adults –epithelial)

  3. Difference between adult & Paed tumours • Association between abnormal development (teratogenesis) & tumour induction. • Prevalence of constitutional genetic abnormalities or syndromes that predispose to cancer • Tendency of malignancy to undergo differentiation • Improved survival

  4. Benign tumours • Hemangiomas “port wine stain” • Lymphangiomas (cystic hygroma) • Sacrococcygeal teratoma • Naevi

  5. Sacrococcygeal teratomas • Germ cell neoplasm • 1:40,000 live births • Mass in the sacrum and buttocks • Composed of elements of > 1 germ cell layer.mixture of elements. • Neural origin determines the behaviour • < 2 months-benign.

  6. Small,round, blue cell tumours • 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.

  7. 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)

  8. 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, calcificaton,cystic change

  9. Gross appearence of Neuroblastoma.

  10. 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

  11. Microscopy of neuroblastoma

  12. Clinical features • Abdominal mass, fever • Blueberry muffin • Wide metastasis • Secrete catecholamines • Vanillylmandelic acid (VMA)/Homovanillic acid (HVA) screening.

  13. Prognosis • Stage • spread to regional lymph nodes,liver,lungs,bones etc • Age :< 1 yr. • Morphology –gangliocytic differentiation better • MYCN (N myc) gene amplification-worse

  14. 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

  15. 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

  16. Morphology of retinoblastoma

  17. Morphology of retinoblastoma • Gross: occular masses. • Microscopy: Sheets of small,round,blue cells with dark nuclei,scant cytoplasm,indistinct borders • Flexner-Wintersteiner rosettes.

  18. Behaviour • Spread through optic nerve or to subarachnoid space to CNS, bone, lymph nodes. • Cure with treatment • Spontaneous cure • Second malignancy

  19. Wilms’ tumour (Nephroblastoma) • Malignant neoplasm of embryonal nephrogenic elements • Composed of embryonal elements • Prevalence :1:10,000 • 2-5 yrs • Good prognosis • Associated with congenital malformations • Tumour resembles developing kidney

  20. Associated syndromes • WAGR –Wilms tumour, Aniridia, Genitourinary anomalies,mental Retardation WT 1 gene • Denys-Drash syndrome: Wilms tumour,intersexual disorders,glomerulopathy. WT1 gene • Beckwith –Wiedemann syndrome(BWS) :Wilms tumour, overgrowth, visceromegaly,macroglossia. WT2

  21. Cut surface :bulging,pale tan

  22. Histopathology of Wilms tumour • Components of Wilms tumour (triphasic) • Blastema • Immature epithelial- abortive tubules ,glomeruli • Immature stroma (mesenchymal)

  23. Clinical features • 1-3 yrs • Unilateral (sporadic),bilateral (familial) • Large abdominal mass • Hematuria • Pain abdomen • Hypertension • Intestinal obstruction • Pulmonary metastasis

  24. In conclusion • Childhood tumors are different from adult • Small blue round cell tumors • Associated with genetic abnormalities.

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