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Neoplasms of infancy and childhood. Benign>malignant Benign>malignant Incidence of malignancy:1-15 yrs - 1.3 /10,000 /year but leading cause of death.

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Presentation on theme: "Neoplasms of infancy and childhood. Benign>malignant Benign>malignant Incidence of malignancy:1-15 yrs - 1.3 /10,000 /year but leading cause of death."— Presentation transcript:

1 Neoplasms of infancy and childhood

2 Benign>malignant Benign>malignant Incidence of malignancy:1-15 yrs /10,000 /year but leading cause of death after accidents in the West. (developing countries??) Incidence of malignancy:1-15 yrs /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) 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. Association between abnormal development (teratogenesis) & tumour induction. Prevalence of constitutional genetic abnormalities or syndromes that predispose to cancer Prevalence of constitutional genetic abnormalities or syndromes that predispose to cancer Tendency of malignancy to undergo differentiation Tendency of malignancy to undergo differentiation Improved survival Improved survival

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

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

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8 Small,round, blue cell tumours Primitive appearance (not anaplastic or pleomorphic) Primitive appearance (not anaplastic or pleomorphic) Sheets of small,round,blue cells (with dark nuclei,scant cytoplasm,indistinct borders.) Sheets of small,round,blue cells (with dark nuclei,scant cytoplasm,indistinct borders.) May show features of organogenesis specific to the tissue of origin. May show features of organogenesis specific to the tissue of origin.

9 Neuroblastoma Embryonal malignant tumour Embryonal malignant tumour Neural crest origin Neural crest origin Neoplastic neuroblasts Neoplastic neuroblasts Site: adrenal medulla &sympathetic ganglia Site: adrenal medulla &sympathetic ganglia 7-10% of solid paediatric malignancies. 7-10% of solid paediatric malignancies. Sporadic occurance. Sporadic occurance. Rarely familial (bilateral,multifocal) Rarely familial (bilateral,multifocal)

10 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

11 Gross appearence of Neuroblastoma.

12 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

13 Microscopy of neuroblastoma

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

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

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17 Retinoblastoma Malignant tumour of the eye in childhood Malignant tumour of the eye in childhood Neuroepithelial origin –posterior retina Neuroepithelial origin –posterior retina Familial, %, associated with germ line mutation, heritable. Familial, %, associated with germ line mutation, heritable. Sporadic:30-40%,somatic gene mutation. Sporadic:30-40%,somatic gene mutation. Associated with Rb 1 gene Associated with Rb 1 gene Secondary malignancy –osteosarcoma Secondary malignancy –osteosarcoma

18 RB gene RB gene is on chromosome 13 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 RB gene function is the most critical checkpoint in the cell cycle and allows the cell to enter from G1 to S G1 to S Tumour supressor gene Tumour supressor gene If both RB genes are abnormal i.e. mutated or have a missing allele, it permits unregulated cell proliferation. If both RB genes are abnormal i.e. mutated or have a missing allele, it permits unregulated cell proliferation. Knudson’s two-hit hypothesis Knudson’s two-hit hypothesis People with RB mutations are susceptibe to malignancies especially osteosarcoma People with RB mutations are susceptibe to malignancies especially osteosarcoma

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20 Morphology of retinoblastoma

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

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

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

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

26 Cut surface :bulging,pale tan

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

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

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


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