neoplasms of infancy and childhood. benign>malignant benign>malignant incidence of...
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Neoplasms of infancy Neoplasms of infancy and childhoodand childhood
Neoplasms of infancy and Neoplasms of infancy and childhoodchildhood
Benign>malignant Benign>malignant Incidence of malignancy:1-15 yrs - Incidence of malignancy:1-15 yrs -
1.3 /10,000 /year but leading 1.3 /10,000 /year but leading cause of death after accidents in cause of death after accidents in the West. (developing countries??)the West. (developing countries??)
Most malignant tumours in Most malignant tumours in children arise from children arise from hematopoietic,nervous and soft hematopoietic,nervous and soft tissues (adults –epithelial) tissues (adults –epithelial)
Difference between adult & Paed Difference between adult & Paed tumourstumours
Association between abnormal Association between abnormal development (teratogenesis) & development (teratogenesis) & tumour induction.tumour induction.
Prevalence of constitutional genetic Prevalence of constitutional genetic abnormalities or syndromes that abnormalities or syndromes that predispose to cancerpredispose to cancer
Tendency of malignancy to undergo Tendency of malignancy to undergo differentiationdifferentiation
Improved survival Improved survival
Benign tumoursBenign tumours
Hemangiomas “port wine stain”Hemangiomas “port wine stain” Lymphangiomas (cystic hygroma)Lymphangiomas (cystic hygroma) Sacrococcygeal teratomaSacrococcygeal teratoma NaeviNaevi
Sacrococcygeal teratomasSacrococcygeal teratomas
Germ cell neoplasmGerm cell neoplasm 1:40,000 live births1:40,000 live births Mass in the sacrum and buttocksMass in the sacrum and buttocks Composed of elements of > 1 germ Composed of elements of > 1 germ
cell layer.mixture of elements.cell layer.mixture of elements. Neural origin determines the Neural origin determines the
behaviourbehaviour < 2 months-benign.< 2 months-benign.
Small,round, blue cell tumoursSmall,round, blue cell tumours
Primitive appearance (not anaplastic Primitive appearance (not anaplastic or pleomorphic)or pleomorphic)
Sheets of small,round,blue cells (with Sheets of small,round,blue cells (with dark nuclei,scant dark nuclei,scant cytoplasm,indistinct borders.)cytoplasm,indistinct borders.)
May show features of organogenesis May show features of organogenesis specific to the tissue of origin.specific to the tissue of origin.
NeuroblastomaNeuroblastoma
Embryonal malignant tumour Embryonal malignant tumour Neural crest origin Neural crest origin Neoplastic neuroblastsNeoplastic neuroblasts Site: adrenal medulla &sympathetic Site: adrenal medulla &sympathetic
gangliaganglia 7-10% of solid paediatric 7-10% of solid paediatric
malignancies.malignancies. Sporadic occurance.Sporadic occurance. Rarely familial (bilateral,multifocal)Rarely familial (bilateral,multifocal)
Pathology of neuroblastomaPathology of neuroblastoma
Site :Paravertebral, Posterior Site :Paravertebral, Posterior mediastinum, abdomen ,Adrenal 1/3mediastinum, abdomen ,Adrenal 1/3
Gross appearance:Gross appearance:
Nodular, of varying sizeNodular, of varying size
May be encapsulated or infiltrativeMay be encapsulated or infiltrative
Cut section: grey-tan, soft and friable Cut section: grey-tan, soft and friable
Varigated,necrosis,hemorrhage, Varigated,necrosis,hemorrhage, calcificaton,cystic changecalcificaton,cystic change
Gross appearence of Gross appearence of Neuroblastoma.Neuroblastoma.
Microscopy of neuroblastomaMicroscopy of neuroblastoma
Sheets of small,round,blue cells with Sheets of small,round,blue cells with dark nuclei,scant dark nuclei,scant cytoplasm,indistinct borders.cytoplasm,indistinct borders.
Mitosis++, Karyorrhectic debris +Mitosis++, Karyorrhectic debris +
Pleomorphism +/-Pleomorphism +/-
Homer-Wright rosettesHomer-Wright rosettes, Neuropil., Neuropil.
Maturation: Schwann cell, stroma Maturation: Schwann cell, stroma &ganglion cell differentiation&ganglion cell differentiation
Microscopy of neuroblastomaMicroscopy of neuroblastoma
Clinical featuresClinical features
Abdominal mass, feverAbdominal mass, fever Blueberry muffinBlueberry muffin Wide metastasisWide metastasis Secrete catecholaminesSecrete catecholamines Vanillylmandelic acid Vanillylmandelic acid
(VMA)/Homovanillic acid (HVA) (VMA)/Homovanillic acid (HVA) screening.screening.
PrognosisPrognosis
Stage Stage spread to regional lymph spread to regional lymph
nodes,liver,lungs,bones etcnodes,liver,lungs,bones etc Age :< 1 yr.Age :< 1 yr. Morphology –gangliocytic Morphology –gangliocytic
differentiation better differentiation better MYCN (N myc) gene amplification-MYCN (N myc) gene amplification-
worseworse
RetinoblastomaRetinoblastoma
Malignant tumour of the eye in childhoodMalignant tumour of the eye in childhood Neuroepithelial origin –posterior retinaNeuroepithelial origin –posterior retina Familial,- 60-70%, associated with germ Familial,- 60-70%, associated with germ
line mutation, heritable.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
RB geneRB gene RB gene is on chromosome 13RB gene is on chromosome 13 RB gene function is the most critical RB gene function is the most critical
checkpoint in the cell cycle and allows checkpoint in the cell cycle and allows the cell to enter from the cell to enter from
G1 to SG1 to S Tumour supressor geneTumour supressor gene If both RB genes are abnormal i.e. If both RB genes are abnormal i.e.
mutated or have a missing allele, it mutated or have a missing allele, it permits unregulated cell proliferation. permits unregulated cell proliferation.
Knudson’s two-hit hypothesisKnudson’s two-hit hypothesis People with RB mutations are People with RB mutations are
susceptibe to malignancies especially susceptibe to malignancies especially osteosarcoma osteosarcoma
Morphology of retinoblastomaMorphology of retinoblastoma
Morphology of retinoblastomaMorphology of retinoblastoma
Gross: occular masses.Gross: occular masses. Microscopy: Sheets of Microscopy: Sheets of
small,round,blue cells with dark small,round,blue cells with dark nuclei,scant cytoplasm,indistinct nuclei,scant cytoplasm,indistinct bordersborders
Flexner-Wintersteiner rosettes.Flexner-Wintersteiner rosettes.
Behaviour Behaviour
Spread through optic nerve or to Spread through optic nerve or to subarachnoid space to CNS, bone, subarachnoid space to CNS, bone, lymph nodes.lymph nodes.
Cure with treatmentCure with treatment Spontaneous cureSpontaneous cure Second malignancy Second malignancy
Wilms’ tumour (Nephroblastoma)Wilms’ tumour (Nephroblastoma) Malignant neoplasm of embryonal Malignant neoplasm of embryonal
nephrogenic elements nephrogenic elements Composed of embryonal elementsComposed of embryonal elements Prevalence :1:10,000 Prevalence :1:10,000 2-5 yrs2-5 yrs Good prognosisGood prognosis Associated with congenital Associated with congenital
malformationsmalformations Tumour resembles developing kidneyTumour resembles developing kidney
Associated syndromesAssociated syndromes WAGR –Wilms tumour, Aniridia, WAGR –Wilms tumour, Aniridia,
Genitourinary anomalies,mental Genitourinary anomalies,mental RetardationRetardation
WT 1 geneWT 1 gene Denys-Drash syndrome: Wilms Denys-Drash syndrome: Wilms
tumour,intersexual tumour,intersexual disorders,glomerulopathy. WT1 genedisorders,glomerulopathy. WT1 gene
Beckwith –Wiedemann Beckwith –Wiedemann syndrome(BWS) :Wilms tumour, syndrome(BWS) :Wilms tumour, overgrowth, overgrowth, visceromegaly,macroglossia. WT2visceromegaly,macroglossia. WT2
Cut surface :bulging,pale tanCut surface :bulging,pale tan
Histopathology of Wilms tumourHistopathology of Wilms tumour
Components of Components of Wilms tumour Wilms tumour (triphasic)(triphasic)
BlastemaBlastema Immature Immature
epithelial- abortive epithelial- abortive tubules ,glomerulitubules ,glomeruli
Immature stroma Immature stroma (mesenchymal)(mesenchymal)
Clinical featuresClinical features 1-3 yrs1-3 yrs Unilateral (sporadic),bilateral Unilateral (sporadic),bilateral
(familial)(familial) Large abdominal massLarge abdominal mass HematuriaHematuria Pain abdomenPain abdomen HypertensionHypertension Intestinal obstructionIntestinal obstruction Pulmonary metastasisPulmonary metastasis
In conclusionIn conclusion
Childhood tumors are different from Childhood tumors are different from adultadult
Small blue round cell tumorsSmall blue round cell tumors Associated with genetic Associated with genetic
abnormalities.abnormalities.
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