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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)
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
Benign tumours
Hemangiomas port wine stain Lymphangiomas (cystic hygroma) Sacrococcygeal teratoma Naevi
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.
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, calcificaton,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 amplificationworse
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. Knudsons two-hit hypothesis People with RB mutations are susceptibe to malignancies especially osteosarcoma
Morphology of retinoblastoma
Morphology of retinoblastoma
Gross: occular masses. Microscopy: Sheets of small,round,blue cells with dark nuclei,scant cytoplasm,indistinct borders Flexner-Wintersteiner rosettes.
Behaviour
Spread through optic nerve or to subarachnoid space to CNS, bone, lymph nodes. Cure with treatment Spontaneous cure Second malignancy
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
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
Components of Wilms tumour (triphasic) Blastema Immature epithelial- abortive tubules ,glomeruli Immature stroma (mesenchymal)
Clinical features
1-3 yrs Unilateral (sporadic),bilateral (familial) Large abdominal mass Hematuria Pain abdomen Hypertension Intestinal obstruction Pulmonary metastasis
In conclusion
Childhood tumors are different from adult Small blue round cell tumors Associated with genetic abnormalities.