Tumors of the Eye and Orbit Flashcards by Mario Luna

Tumors of the Eye and Orbit

What is the most common and next most common primary intraocular malignancy in adults?

most common is melanoma followed by lymphoma

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Tumors of the Eye and Orbit

What is the most common and next most common tumor malignancy in children?

most common is retinoblastoma followed by medulloepithelioma

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Tumors of the Eye and Orbit

What are the two most common primary tumors that metastasize to the intraocular areas?

breast

lung

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Tumors of the Eye and Orbit
Radiation Tolerance

Loss of eyelashes may occur at doses as low as ___ using standard fractionation?

20 Gy

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Tumors of the Eye and Orbit
Radiation Tolerance

Xerophthalima may occur at doses as low as ___ ?

24 to 26 Gy

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Tumors of the Eye and Orbit
Radiation Tolerance

Acute conjunctivitis is common with doses ≥____

30 Gy

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Tumors of the Eye and Orbit
Radiation Tolerance

Early dry eye syndrome (9 to 10 months) is usually caused by doses >____.

57 Gy

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Tumors of the Eye and Orbit
Radiation Tolerance

Late dry eye syndrome (4 to 11 years) is usually caused by doses >____.

30 to 45 Gy

Moderate-dose orbital RT (30 to 45 Gy) can cause dry eye syndrome 4 to 11 years after treatment, whereas higher doses (>57 Gy) can produce it in 9 to 10 months.

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Tumors of the Eye and Orbit
Radiation Tolerance

If possible, treating with the eyes closed reduces the risk of RT-induced conjuctivitis.

TRUE or FALSE?

False.
***
Conjunctivitis can be
reduced by treating with an open eye with megavoltage equipment if the clinical
situation permits.

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Tumors of the Eye and Orbit
Radiation Tolerance

I. For the cornea, what are the doses associated with
punctate epithelial erosions,
corneal edema, and
perforation, respectively?

II. These radiation doses are mainly responsible for most acute corneal toxicities.
TRUE or FALSE?

30 to 50

40 to 50

60 Gy

False.
***
Although RT can directly injure the cornea, most acute corneal toxicity results
from loss of the tear film with secondary keratitis sicca.

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Tumors of the Eye and Orbit
Radiation Tolerance

The iris is relatively radioresistant, and thus, acute iritis is rare.
However,
persistent iritis, with symptoms such as pain, red eye, and blurred vision, has
been observed after hypofractionated RT doses of __ to __Gy and after doses
≥__ Gy given with conventional fractionation.

30 to 40 (hf)

70 (cf)

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Tumors of the Eye and Orbit
Radiation Tolerance

I. The estimated risk of cataract for 1-Gy exposure to the lens during childhood is 50%.

II. In adults, after 2.5 to 6.5 Gy, there is a 33% of progressive cataract with latent period of 4 years, whereas after 6.51 to 11.5 Gy, there is a 66% risk with a latent period of 8 years.

Which is TRUE?

I.
***
Hall estimated 50% risk of cataract for 1-Gy exposure to the lens during childhood.
In adults, higher doses are associated with radiation-induced cataract:
After 2.5 to 6.5 Gy, there is a 33% of progressive cataract with latent period of 8 years, whereas after 6.51 to 11.5 Gy, there is a 66% risk with a latent period of 4 years.

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Tumors of the Eye and Orbit
Radiation Tolerance

What is the he threshold dose for retinal damage?

30 to 35 Gy

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Tumors of the Eye and Orbit
Radiation Tolerance

In a review by investigators from the Mayo
Clinic, the risk of RION was almost zero with conventionally fractionated doses
≤50 Gy and still rare with maximum dose <55 Gy.

Fraction size was of primary importance: In cases where >60 Gy was
received, fraction size was more important than total dose in producing RION.

TRUE or FALSE?

True.
***
The 15-year actuarial risk was 11% when fraction size was <1.9 Gy compared
with 47% when fraction size was >1.9 Gy.

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Tumors of the Eye and Orbit

What are the RT fractionations used in the treatment of pterygium?
from benign disease chapter

3 Gy x 10 to 6 Gy x 10

30 Gy/3fractions (Nakamatsu et al.)

25 Gy × 1

low fractionation (2 Gy in 10 fractions) to high fractionation dose (5 Gy in 7 fractions) beta radiotherapy (Viena et al.)

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Tumors of the Eye and Orbit

Choroidal hemangiomas are benign vascular tumors of the choroid, characterized as either circumscribed or
diffuse.

Which is usually diagnosed in young patients wither because of fundus examination prompted by a facial hemangiomaassociated with Sturg-Weber syndrome?

diffuse

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Tumors of the Eye and Orbit

I. The clinical course of both form of choroidal
hemangioma is highly variable, with visual impairment ranging from none to total blindness.

II. Neither variety of choroidal hemangioma metastasizes or
transforms to malignancy.

Which is/are TRUE?

Both.
***
Therefore, the primary indication for treatment is to
prevent loss of visual acuity.

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Tumors of the Eye and Orbit

What is the usual treatment for circumscribed lesions (eye chapter) and for CH that are not near the central visual structures such as macula and papilla (benign disease chapter)

photodynamic therapy (eye chapter)

```
PDT
and/or
Photocoagulation
and/or
transpupillary thermotherapy
benign diseases chapter
~~~

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Tumors of the Eye and Orbit

Dose range for EBRT (photon) that can result in flattening of the hemangioma, resorption of subretinal fluid, and reattachment of the retina within 6 to 12 months.

18 to 30 in 10-18fx

In very advanced cases with retinal detachment, a higher dose of
36 Gy in fractions of 1.8 Gy appear to be efficacious

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Tumors of the Eye and Orbit

Brachytherapy can be considered for CH particularly for circumscribed CH.

TRUE or FALSE?

True.

This treatment usually
achieves resolution of subretinal fluid and reattachment of the retina with
preservation of pretreatment visual acuity

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Tumors of the Eye and Orbit

Brachytherapy dose for CH varies with isotope, but in a study using 125-I, what is the dose to the apex of the lesion that caused tumor regression in 100% of cases treated?

48 Gy

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Tumors of the Eye and Orbit

At the Institut Curie series, what dose was used for CH that resulted in retinal reattachment occurred in all
cases, and a completely flat scar was obtained in 91.5%?

20 CGyE.

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Tumors of the Eye and Orbit
(from benign disease chapter)

As reported by Schilling, what is the 3DCRT dose for circumscribed CH?
for diffuse CH?

Retinal reattachment occurred in 64% of the cases with improved vision in 50% and stable vision in 50%.

18 to 20 Gy for circumscribed CH

30 Gy for diffuse CH given in 1.8- to 2-Gy daily fractions.

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Tumors of the Eye and Orbit
(from benign disease chapter)

Fractionated proton radiotherapy doses range from _________.

In the study by Zografos et al., all 54 cases experienced retinal reattachment and visual acuity was improved in 70%.
Levy-Gabriel et al. demonstrated a 100% rate of retinal reattachment and substantial improvement in visual acuity using proton beam therapy.

16.4 to 30 Gy in four fractions.

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Tumors of the Eye and Orbit Capillary hemangiomas are benign endothelial cell neoplasms that rarely occur on the eyelids or skin of the orbit. I. Retinal capillary hemangiomas may represent component of the Beckwith-Wiedemman syndrome, and lesions of the face that occupy the distribution of the trigeminal nerve can be a component of Sturge-Weber syndrome. II. The natural history of these lesions is usually spontaneous regression over 3 to 4 years, making conservative management the treatment of choice. III. Lesions that are large enough to obstruct vision and amblyopia may occur. Treatment options then include corticosteroids, interferon alfa-2a, laser therapy, embolization, immunomodulators, surgery, and systemic propranolol. IV. RT has been historically used in the management of capillary hemangiomas with original studies showing that doses in the 16 to 20 Gy range provide effective local control. Which is FALSE?

I. von Hippel-Lindau syndrome (VHL) - not Beckwith-Wiedemann :P oo na. pero... ganyan magpatest board examiners.

Tumors of the Eye and Orbit What are the characteristic imaging features in orbital pseudotumor?

extraocular muscle enlargement, optic nerve thickening, and inflammation of retrobulbar adipose tissue. (additional from benign disease chapter: enhances with contrast)

Tumors of the Eye and Orbit (from benign disease chapter) Orbital pseudotumor presents as a chronic inflammation of the orbital soft tissues. TRUE or FALSE?

False. symptoms present acutely.

Tumors of the Eye and Orbit RT dose for orbital pseudotumor as reported by Mathiesen. (both from this chapter and benign disease chapter)

20 Gy/10 fx *** additional info from benign disease chapter: Prabhu et al. reported on 26 orbits affected with OP in 20 patients treated with radiation therapy (25.2 to 30.6 Gy) between 2002 and 2011. The majority (85%) demonstrated a response to RT, and 70% of the patients initially on steroids were completely off steroids by the last follow-up visit. Long-term complications occurred in seven patients, the majority of which were cataracts.

Tumors of the Eye and Orbit RT dose. TO/GO

20 Gy/10fx

Tumors of the Eye and Orbit Most common improvement in symptoms after RT for TO/GO

motility impairment (Mourits) (Prummel)

Tumors of the Eye and Orbit (from benign disease chapter) ``` Graves ophthalmopathy (GO), also referred to as Graves orbitopathy or thyroid eye disease, is an autoimmune disorder affecting the musculature of the orbits. The presence of activated __ lymphocytes leads to an inflammatory reaction secondary to the release of cytokines. ```

Tumors of the Eye and Orbit (from benign disease chapter) What are the parameters of SPECS, a scoring system for TO/GO.

Soft tissue involvement, Proptosis, Extraocular movements, Corneal involvement, and Sight (visual acuity)

Tumors of the Eye and Orbit (from benign disease chapter) What are the most commonly involved muscles in GO/TO?

inferior and medial rectus muscles.

Tumors of the Eye and Orbit Metastases to the uvea is more common than primary ocular cancers. Within the uvea, what is the most common site of metastasis?

choroid (88%) followed by iris (9%) and ciliary body (2%). (Shields and colleagues)

Tumors of the Eye and Orbit What is the most common primary site of tumors metastatic to the uvea? (males)

lung (40%) | followed by gastrointestinal (9%) and kidney (8%).

Tumors of the Eye and Orbit What is the most common primary site of tumors metastatic to the uvea? (females)

breast (68%) lung (12%) others (4%)

Tumors of the Eye and Orbit fluorescein fundoscopic finding in choroidal metastases from NSCLC?

hyperfluorescence from the surface of the choroidal | tumor in its late phase with the accumulation of subretinal fluid.

Tumors of the Eye and Orbit Although unilateral RT for metastatic disease can be achieved with new modern techniques, current recommendation is to treat bilaterally. TRUE or FALSE?

True

Tumors of the Eye and Orbit For solitary metastases in patients with limited survival expectancy, as well as lesions distant to the optic nerve, this treatment can be considered as primary and salvage treatment after EBRT.

Plaque brachytherapy

Tumors of the Eye and Orbit What is the primary and salvage doses using plaque brachytherapy?

68. 8 Gy | 235. 6 Gy

Tumors of the Eye and Orbit What are the factors predictive of local recurrence in uveal melanomas?

epithelioid cell type large tumor size posterior tumor extension

Tumors of the Eye and Orbit In uveal melanoma, he probability of malignancy can be estimated according to what parameters?

tumor thickness serous retinal detachment orange pigment and symptoms

Tumors of the Eye and Orbit The desire to improve survival and preserve vision stimulated the development of alternative, organ-preserving therapies for uveal melanoma. Still, enucleation is required in a subset of patients, either because tumor is too extensive at presentation or if vision loss were likely to occur with conservative treatment. General guidelines for enucleation include poor general health of the patient and what other tumor characteristics?

tumor diameter >17 mm; thickness >6 to 7 mm; involvement of the optic disc; invasion of more than 30% of the iris, ciliary body, or angle; retinal perforation;

Tumors of the Eye and Orbit Plaque Brachytherapy Differentiate Iodine-125 and Ruthenium-106.

Iodine 125 - emits gamma rays - range 8 to 10 mm thick Ruthenium-106 - beta particles - <5 mm thick

Tumors of the Eye and Orbit Plaque Brachytherapy Historically, the general objective with all plaques is to deliver approximately 80 Gy to the tumor apex by fixing the plaque in the exact location of the tumor. However, recent data suggest lower doses may be sufficient for tumor control with less toxicity: Local control and overall survival did not differ according to dose quartile (<69 Gy, 69 to 81 Gy, 81 to 89 Gy, >89 Gy). However, visual acuity and radiation complications increased with increasing dose and the authors concluded that doses as low as __ Gy may be appropriate for uveal melanoma.

Tumors of the Eye and Orbit Plaque Brachytherapy The American Brachytherapy Society updated their consensus guidelines on plaque therapy for uveal melanoma in 2014 and determined that brachytherapy is appropriate for the majority of uveal melanomas. What are the exceptions?

Exceptions include tumors with >5 mm of extraocular extension basal diameters that exceed the limits of brachytherapy applicators patients with blind and painful eyes, and patients with no light perception.

Tumors of the Eye and Orbit What type of RT has distinct advantage for parapapillary and peripapillary tumors due to it's dosimetric characteristic of sharp field edge?

Proton therapy

Tumors of the Eye and Orbit RT dose for uveal melanoma (proton therapy)

total dose of 70 Cobalt Gray Equivalents (CGE), which is delivered in five equal fractions over 7 to 10 days (63.6 proton Gy × 1.1 relative biologic effectiveness = 70 CGE)

Tumors of the Eye and Orbit RT dose for uveal melanoma using single fraction SRS.

50 Gy to the 50% IDL

Tumors of the Eye and Orbit RT dose for uveal melanoma using fractionated SRT.

50 Gy/10 Gy/5 fx

Tumors of the Eye and Orbit Retinoblastoma has been associated with poor socioeconomic status and HPV. TRUE or FALSE?

True

Tumors of the Eye and Orbit Retinoblastoma can be heritable and nonheritable. Bilateral retinoblastoma, is labeled nonheritable in the absence of family history. TRUE or FALSE?

False *** Rb has a heritable form and nonheritable form, with approximately 55% of children having the nonheritable form. If there is no family history, the disease is labeled “sporadic,” but this does not necessarily indicate that it is the nonheritable form, because bilateral cases, most of which are heritable, also have no family history.

Tumors of the Eye and Orbit ``` Rb can present with unilateral disease (two-thirds of cases), with bilateral disease, or rarely trilateral disease. ``` What organ involvement constitutes the third organ in trilateral disease?

pineal gland

Tumors of the Eye and Orbit Bilateral Rb is diagnosed later than unilateral disease. TRUE or FALSE?

False. *** Approximately 80% of children in Rb are diagnosed before the age of 3, with unilateral cases diagnosed at an earlier age (14 to 16 months) than those with bilateral presentations (29 to 30 months)

Tumors of the Eye and Orbit Which of the following is/are TRUE? I. Macroscopically, zones of necrosis are found near blood vessels, whereas viable tumor cells are found in relatively avascular areas. II. Microscopically, only differentiated elements are present. Undifferentiated elements are diagnosed by IHC staining. III. Differentiated elements appear as collections of small, round cells with hyperchromatic nuclei; undifferentiated elements include Flexner-Wintersteiner rosettes, Homer-Wright rosettes, and fleurettes from photoreceptor differentiation.

``` NONE. *** I - the other way around II - both are present III - the other way around ```

Tumors of the Eye and Orbit Where is the RB gene located?

long arm of chromosome 13 (13q14)

Tumors of the Eye and Orbit Rb What is the imaging of choice to detect intraocular calcifications?

Tumors of the Eye and Orbit Rb What is the imaging of choice to detect extraocular involvement?

MRI of the brain and orbits

Tumors of the Eye and Orbit Rb What is the indication to do brain and spinal cord MRI and CSF examination?

gross invasion of the optic nerve by imaging studies or evidence of microscopic involvement beyond the lamina cribrosa on histopathologic examination of the enucleated eye.

Tumors of the Eye and Orbit Rb Most children with unilateral Rb present with advanced disease and require enucleation. TRUE or FALSE?

True

Tumors of the Eye and Orbit Rb Aside from unilateral disease in general, what are other indications for enucleation?

- bilateral disease where the eye with more advanced disease does not respond to chemotherapy or other treatments, - when active tumor is present in an eye with no vision, - when glaucoma is present as a result of neovascularization of the iris - tumor invasion into the anterior chamber, - when direct visualization of an active tumor is obstructed by conditions including hemorrhage, corneal opacity, or cataract.

Tumors of the Eye and Orbit Rb Identify the Reese-Elseworth classification: Vitreous seeding

Group 5B (very unfavorable)

Tumors of the Eye and Orbit Rb Identify the Reese-Elseworth classification: Lesions extending anteriorly to the ora serrata

Group 4B (unfavorable)

Tumors of the Eye and Orbit Rb Identify the Reese-Elseworth classification: Multiple tumors, none larger than 4 DD, all at or behind the equator

``` Group 1B (very favorable) group 1a is solitary ```

Tumors of the Eye and Orbit Rb Identify the Reese-Elseworth classification: Multiple tumors, none larger than 4-10 DD behind the equator

``` Group 2B (favorable) group 2a is solitary ```

Tumors of the Eye and Orbit Rb Identify the Reese-Elseworth classification: Any lesion anterior to the equator

Group 3A (possible maintenance) 3B is solitary tumor larger than 10 DD behind the equator

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: ≤3 mm in size (smaller tumor)

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: ``` larger tumor (>3 mm) or macular location (<3 mm to fovoela) or juxtapapillary location (≤1.5 mm to disc) or clear subretinal fluid ≤3 mm from margin ```

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: Extensive (occupying >50% of globe)

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: Extensive (occupying >50% of globe) neovascular glaucoma opaque media from hemorrhage in anterior chamber, vitreous, or subretinal space invasion of postlaminar optic nerve, sclera, orbit, anterior chamber, choroid (>2 mm)

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: Diffuse seeds - subretinal seeds >3mm from rb - vitreous seeds >3mm from rb - both retinal and vitreous >3mm from rb

D D1, 2, 3 respectively "D"iffuse seeds

Tumors of the Eye and Orbit Rb Identify the ICR subgroup: Focal seeds - subretinal seeds ≤3mm from rb - vitreous seeds ≤3mm from rb - both subretinal and vitreous >3mm from rb

C C1, 2, 3, respectively Fo"C"al seeds

Tumors of the Eye and Orbit Rb Dose range (RT)

40-50 in 1.5-1.8 | some report 42-44/20-22 fx

Tumors of the Eye and Orbit Rb Coverage of the entire retina is necessary in RT for Rb. TRUE or FALSE?

True.

Tumors of the Eye and Orbit Primary intraocular lymphoma (PIOL), formally known as ocular reticulum cell sarcoma, is an uncommon clinical manifestation of non-Hodgkin lymphoma, which arises in the retina or the vitreous humor. It is now treated initially with chemotherapy. If RT is used, what is the dose?

35 to 45 Gy

Tumors of the Eye and Orbit Most common histology of optic pathway glioma?

pilocytic or LGA

Tumors of the Eye and Orbit OPG. What is the main indication for RT?

progressive disease

Tumors of the Eye and Orbit OPG. RT doses?

45 to 60 Gy in 1.8 to 2.0 Gy fractions

Tumors of the Eye and Orbit OPG. What hereditary condition is associated with poor prognosis even after treatment?

Tumors of the Eye and Orbit OPG. Age __ at the time of treatment and RT dose ___ were associated with significantly improved PFS and improved endocrinologic control. (Grabenbauer)

>10 | >45

Tumors of the Eye and Orbit OPG. BONUS: Please read CNS in children chapter

BONUS: Please read CNS in children chapter

Tumors of the Eye and Orbit Lymphoma Usual RT dose for orbital lymphoma (ocular adnexal lymphoma) to achieve control.

30-36 Gy

Tumors of the Eye and Orbit What syndrome is usually associated with sebaceous carcinoma of the eyelid?

Muir-Torre syndrome

Tumors of the Eye and Orbit Historically, regional node metastasis occurred in about 30% of sebaceous carcinoma, but metastases have become less frequent in recent years. Where do tumors that originate in the upper eyelid tend to metastasize?

preauricular and parotid

submandibular and cervical