Retinoblastoma Flashcards

1
Q
A
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2
Q

What is retinoblastoma?

A

Primarily a pediatric malignancy that develops in cells of the retina

It is the most common intraocular tumor in young children.

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3
Q

Where is the retinoblastoma gene (RB gene) located?

A

Located on chromosome 13

It is a tumor suppressor gene.

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4
Q

What type of cells does retinoblastoma originate from?

A

Neuroectoderm

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5
Q

What characterizes retinoblastoma under a microscope?

A

Contains deep blue cells with little cytoplasm following a specific staining procedure

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6
Q

What is a common complication of retinoblastoma tumors?

A

Necrosis and hemorrhage are common within the tumor

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7
Q

What percentage of all primary tumors of the eye does retinoblastoma account for?

A

25%

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8
Q

At what age are 95% of retinoblastoma cases diagnosed?

A

Before the age of 5

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9
Q

What is the incidence rate of retinoblastoma in live births?

A

1 in 15,000 live births will be diagnosed

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10
Q

What are the two forms of retinoblastoma?

A

Inheritable and Non-inheritable

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11
Q

What is a characteristic of the inheritable form of retinoblastoma?

A

History of disease in the family, tends to be bilateral tumors

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12
Q

What is a characteristic of the non-inheritable form of retinoblastoma?

A

No history of disease in family, roughly 65% are unilateral

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13
Q

What are potential parental factors linked to retinoblastoma?

A

Diets low in fruits and vegetables, exposure to chemicals, smoking, excessive radiation exposure, older age among fathers

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14
Q

What is leukochoria?

A

An abnormal retinal light reflex often seen in retinoblastoma

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15
Q

What imaging modality is the choice for pre-treatment staging of retinoblastoma?

A

MRI

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16
Q

What is the Reese Ellsworth Classification used for?

A

To correlate tumor size and location with visual preservation

17
Q

What characterizes Group A in the International Classification for Intraocular Retinoblastoma?

A

Small tumors (no more than 3 mm) that are only in the retina

18
Q

What are poor prognostic factors in retinoblastoma?

A

Orbital invasion, involvement of optic nerve, CNS dissemination, inherited bilateral tumors

19
Q

What is the goal of therapy in retinoblastoma?

A

Cure and preservation of vision

20
Q

What are focal conservative treatments for retinoblastoma?

A
  • Cryotherapy
  • Laser photocoagulation
  • Plaque radiotherapy
21
Q

What is enucleation?

A

Surgery to remove the eye and part of the optic nerve

22
Q

What chemotherapy agents show good response rates for retinoblastoma?

A
  • Vincristine
  • Cyclophosphamide
  • Platinols
23
Q

What is the typical radiation therapy dose for retinoblastoma?

A

4000-5000 cGy / 180-200 cGy if using EBRT alone

24
Q

What is the purpose of brachytherapy in retinoblastoma treatment?

A

Local control for a small mass

25
Q

What are common acute side effects of retinoblastoma treatment?

A
  • Skin changes
  • Loss of eyebrows or eyelashes
  • Irritation of eye
  • Dryness
  • Redness
26
Q

What are chronic side effects of retinoblastoma treatment?

A
  • Cataract
  • Dry eye
  • Secondary malignancies
  • Blind spots
  • Retinopathy
  • Optic neuropathy
27
Q

What is the cure rate for iodine 125 plaque therapy?

A

80% cure rate and visual preservation