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Important cause of death in young children
Malignancies Need to distinguish from benign lesions
Heterotopia / Choristoma
Early diagnosis is the aim
Treatment is better tolerated
Contains the tissues derived from all ectoderm,
endoderm and mesoderm.
Skin is most commonly seen: hence the name dermoid
Any tissues can be seen: cartilage, bone, tooth, hair,
sebaceous glands, brain tissues…..etc.
Most commonly seen in the midline structures.
Most common solid tumor of childhood next to CNS
Most occur below 5 yrs of age
Tendency to regress spontaneously
Head to toe Sympathetic chain
75% arise within abdomen Adrenals
Can arise in brain, Head & neck
Range in size
Advanced tumors may invade the renal vein
Grey-White, Soft, Friable, Large areas of hemorrhage,
Tendency to metastasize early and invade adjacent
Some areas of differentiation seen
Some ganglion like cells Maturation
Ganglioneuroma Better differentiated
Presence of Schwann cell stroma
Protuberant abdomen, fever, weight loss
Hepatomegaly, metastasis, bone pain
Elevated blood levels of catecholamines: VMA
Staging of the tumors & age of patient decides the prognosis
Del (1p36) & amplification of N-myc oncogene Bad
Mc Malignant tumor of eye in childhood
Multifocal or Bilateral
Spontaneous regression is noted, secondary primary
Familial & Sporadic
Rb gene mutation
1 in 20000 infants and children
60% Familial; 40% Sporadic
Autosomal dominant trait
Gene is present on chromosome 13q14,
Loss of both alleles leads to Retinoblastoma
Arise from neuroepithelial cell
Some differentiated structures may be present
Flexner- Wintersteiner rosettes
Dissemination by Optic nerve CNS. Skull bones, LN.
Median age at presentation is 2 yrs
Poor vision, strabismus, Leukocoria (Cat’s eye reflex)
Spontaneous regression, with or without Rx is also noted
Secondary tumors like OS & Soft tissue sarcomas.
Most common renal tumour in children
10 / million, under 15 yrs,
Common age – 2 to 5 yrs,
5 to 10% bilateral – synchronous or metachronous