Retinoblastoma Flashcards

1
Q

What is retinoblastoma?

A

Malignant tumor of embryonic neural retina.

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2
Q

Age range for RtB?

A

<5 years

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3
Q

Pathophysiology of RtB?

A

Intraocular growth, prior to invasion of structures within the globe or spread to metastatic sites.

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4
Q

Symptoms of RtB?

A

Decreased visual acuity
Inflammation

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5
Q

Signs of RtB?

A

Leukocoria
Strabismus
Hyphema
Virtreous hemorrhage- black pupil
Proptosis

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6
Q

How do you classify RtB?

A
  1. laterality: unilateral( 2 yrs)
    Bilateral (1 year)
    Trilateral ( <5 years)
  2. Focality: Unifocal / multifocal
  3. Genetics: hereditary - 40%
    non hereditary - 60%
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7
Q

What is trilateral RtB?

A

Consists of bilateral hereditary + intracranial neuroblastic tumor of the pineal gland
Occurs in 5- 15 % of children with familial, multifocal or bilateral RtB.

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8
Q

How do I diagnose RtB?

A

Elicit red relex
Siblings of patients should be screened at regular interval at least through age 3
Siblings of patients should be screened for mutations in RB1 genetic
CT or MRI of brain and orbit
No surgical biopsy is performed

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9
Q

How do I investigate?

A

FBC
BUE and Creatinine
Lumbar puncture if there’s suspicion of CNS disease
Bone marrow biopsy in abnormal blood counts
Histopathologic evaluation if enucleation is performed.

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10
Q

Treatment modalities?

A

Systemic chemotherapy
External beam radiotherapy
Local ophthalmic therapy ( laser Ry)
Intra arterial chemotherapy, using and interventional neuro-radiology approach
Enucleation

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11
Q

Secondary malignacies

A

Osteocarcoma
Soft tissue sarcomas
Melanoma
Leukemia
Lymphoma
Breast cancer

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