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??)
Most malignant tumours in
children arise from
hematopoietic,nervous and soft
tissues (adults epithelial)
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
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.
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.

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
Gross appearence of
Neuroblastoma.
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.
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

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

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
Cut surface :bulging,pale tan

Histopathology of Wilms tumour
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.