Retinoblastoma

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Retinoblastoma : 

Retinoblastoma Dr Gyanendra Lamichhane Lumbini Eye Institute Bhairahwa,Nepal

Introduction : 

Introduction It is the most common , primary,malignant intraocular tumor of children. Second to uveal melanoma in the general population. Seen in 1:14000 to 20000 live births. Age- usually becomes apparent by 3 yrs. No sex predeliction.

Pathogenesis : 

Pathogenesis Genetic predisposition Malignant transformation of primitive retinal cells. Due to defect or loss of the retinoblastoma gene (13q14)- suppressor gene. Somatic or germinal mutation. Knudson’s two hit hypothesis.

Genetics : 

Genetics Heritable and Non-heritable types. Non-heritable :60% 85% are unilateral(U/L) Somatic mutation Single tumor Average age- 2 yrs Affected pts will not pass on disease to offspring.

Heritable type : 

Heritable type Seen in 40% Germline mutation with autosomal dominance Positive family history-6% 85% are B/L, 15% U/L cases fall into this group. Age- earlier presentation- 18 months. Risk of transmission- 50% May develop secondary malignancies.

Presentation : 

Presentation Leucocoria- 60% Strabismus- 20% (eso- 50%, exo- 50% ) Sec. Glaucoma Acute red eye Uveitis Pseudohypopyon, hyphema, NVI Orbital inflammation Proptosis Metastasis to L.node and brain.

LEUCORIA : 

LEUCORIA

RED EYE : 

RED EYE

PSEUDOHYPOPYON : 

PSEUDOHYPOPYON

PROPTOSIS : 

PROPTOSIS

WITH VITREOUS SEEDING : 

WITH VITREOUS SEEDING

RETINOBLASTOMA : 

RETINOBLASTOMA

OPTIC NERVE INVOLVEMENT : 

OPTIC NERVE INVOLVEMENT

Findings : 

Findings Intraretinal Endophytic Exophytic Histology- flexner-wintersteiner rosettes Homer wright rosettes Fleurettes.

INTRARETINAL TUMOR : 

INTRARETINAL TUMOR

ENDOPHYTIC TUMOR : 

ENDOPHYTIC TUMOR

EXOPHYTIC TUMOR : 

EXOPHYTIC TUMOR

Investigations : 

Investigations CBC Serum LDH USG X-ray chest, skull CT scan MRI BM biopsy.

Systemic association : 

Systemic association Other tumors like – Pineoblastoma (trilateral retinoblastoma) Osteogrnic sarcoma Soft tissue sarcoma 13q14 deletion syndrome

Reese-Ellsworth classification : 

Reese-Ellsworth classification Group-1- very favourable prognosis 1A- single tumor < 4 DD, at or post to equator. 1B- multiple tumor <4 DD, at or post to equator. 2A- single tumor 4-10 DD, at or post to equator 2B- multiple tumor 4-10 DD.

Contd….. : 

Contd….. 3A- any tumor ant to the equator 3B- single tumor 10 DD post to equator. 4A- multiple tumor, 10 DD or >. 4B- any tumor ant to ora serrata 5A- tumor > ½ of retina 5B- vitreous seeding.

Treatment : 

Treatment Chemotherapy: Drugs- vincristine, etoposide, carboplatin 3-4 weekly cycles Indications- Chemoreduction esp if B/L Extraocular involvement. Orbital recurrence after enucleation Metastasis Intracranial involvment.

Surgery : 

Surgery Enucleation: good if tumor is only intraocular- must attempt to get a long stump of optic nerve (10- 15 mm). U/L cases IN B/L cases if assymetric involvement. Rarely exenteration.

External beam radiation : 

External beam radiation 40 –50 Gy in divided doses. Uses- tumors not responding to chemo or local T/T Tumor of optic disc. Vitreous seeding. Side effects- cataract, sec tumor, def bone growth.

Episcleral plaque radiation : 

Episcleral plaque radiation Iodine-125 , Ruthenium-106 Uses-small tumors(12 by 6 mm), solitary, anterior , with minimal vitreous seeding.

Photocoagulation : 

Photocoagulation Xenon arc Smaller tumors- 2-3 mm thick, post to equator.( 7 mm diameter). Needs clear media. Not to be used in vitreous seeding.

Laser : 

Laser Transpupillary thermotherapy Diode laser Small tumors (4mm diameter, 2mm thick) Laser given around base of tumor. Repeated after 3 –4 weeks.

Cryotherapy : 

Cryotherapy Small to medium tumors ant to equator Triple freeze –thaw . Repeat in 2-3 weeks. C/I in vitreous seeding.

Prognostic factors : 

Prognostic factors Optic nerve : if cut end is free of tumor-mortality- 8% If cut end is involved-mortality-65% If L.cribrosa- 15% Choroid affected- poor prognosis Tumor size and location Histology Age of onset, B/L.,heritable.

Regression : 

Regression Type 1- calcific ,avascular mound. Type 2- tan, fish flesh , attenuated vessels. Type 3- both.

Follow up : 

Follow up During T/T- every 2-4 weeks If T/T effective: FU 3 mnthly for 2 yrs 6 mnthly till 6 yrs of age Yearly.

D/D : 

D/D Coats disease Toxocariasis ROP PHPV Coloboma of post segment Org. vit hmg. Astrocytoma Medullated nerve fibre

COATS DISEASE : 

COATS DISEASE

TOXOCARA GRANULOMA : 

TOXOCARA GRANULOMA

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