Upload
gary-wells
View
223
Download
0
Embed Size (px)
Citation preview
Belgorod State National Research University
Department: Pathological Anatomy.
Topic: Childhood Tumors.
Compiled by:
N. K. Gaur
Small,Round, Blue Cell Tumors in Childhood.
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 1/3Gross appearance:Nodular, of varying sizeMay be encapsulated or infiltrativeCut section: grey-tan, soft and friable Varigated, necrosis, hemorrhage, calcification, 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 amplification-worse.
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. Knudson’s two-hit hypothesis People with RB mutations are susceptibe to malignancies especially
osteosarcoma
Morphology of retinoblastoma.
Gross: occular masses. Microscopy: Sheets of small,round,blue cells with dark nuclei,scant
cytoplasm,indistinct borders Flexner-Wintersteiner rosettes.
Morphology of retinoblastoma.
Behaviour .
Spread through optic nerve or to subarachnoid space to CNS, bone, lymph nodes.
Cure with treatment. Spontaneous cure. Second malignancy.