1 / 29

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??)

myron
Télécharger la présentation

Neoplasms of infancy and childhood

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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.

More Related