Pathology of retinoblastoma Dr ajit nambiar objectives What

Pathology of retinoblastoma Dr ajit nambiar

objectives • What is retinoblastoma!!! • Cell of origin • Eitopathogenesis • Pathology • prognosis

retino/ blast / oma!!!! WHAT IS RETINOBLASTOMA

Remember blast and age of patient!!!! Most common intraocular tumor in eye

CELL OF ORIGIN Although the name retinoblastoma might suggest origin from a primitive retinal cell, it is now clear that the cell of origin of retinoblastoma is a neuronal progenitor.

All tumors are caused by ……. And these r brought about by……. . , ………. And GOK

TUMORS CAN BE SPORADIC somatic mutation HEREDITARY Germline mutation bilateral

MOST COMMON GENE INVOLVED Rb GENE

Genetics autosomal dominant inheritance Heriditary Retinoblastoma arises when the retinal progenitor suffers a second, somatic mutation and the RB gene function is lost. In the sporadic cases, both RB alleles are lost by somatic mutations.

How do gene mutations cause cancer What is function of normal Rb gene

EXPRESSION OF CYCLIN DEPENDENT KINASES DURING CELL CYCLE

TUMOR SUPRESSOR GENES ARE INHIBITORS IN THE CELL CYCLE(CYCLIN DERIVED KINASE INHIBITORS)

When cells divide uncontrolled U get mass of cells

PATHOLOGY : GROSS & MICROSCOPY

Layers of the eye

What gives the white reflex c. Mixed a. Shows growth into the vitreous(arrow marks the retina) b. Growth below the retina d. Diffuse c. Mixed a. Endophytic b. Exophytic d. Growth along retina with deposits along ciliary body(CD)

MICROSCOPY KEY WORDS BLASTS : UNDIFFERENTIATED ROSSETTES Flexner-Wintersteiner rosettes reflecting photoreceptor differentiation.

Rossette FRENCH FOR ROSE

PROGNOSIS depends on spread(STAGING) • Retinoblastoma tends to spread to the brain and bone marrow • Prognosis is adversely affected by extraocular extension and invasion along the optic nerve, and by choroidal invasion. • 5 YR SURVIVAL IN most cases is above 90% • Hereditary with bilateral tumors generally have bad prognosis

A DOCTOR’S DILEMMA • My colleague’s daughter had retinoblastoma treated when she was 2 yr old. • Now aged 13 yr she has complaining of pain in the knee. • WHAT WAS THE MOTHER FEARING …… • THANK GOD HER WORST FEARS WEREN’T TRUE

Second cancers after retinoblastoma Survivors of the hereditary form of retinoblastoma have a much higher risk for developing other types of cancer throughout their lives. This is because each cell in the body has an abnormal RB 1 tumor suppressor gene Osteogenic sarcoma, soft tissue sarcoma, malanomas, lung , breast, uterine

sometimes we force things to happen change tracks let go sit back and enjoy the present!!! Ajit nambiar THANK U DEAR FRIENDS HAVE A NICE DAY!!!