A 4 year old male is brought in by his mother stating that the child complains of left eye pain, constant squinting and inability to see out of the left eye. There is no significant past history. Family history reveals a grandfather diagnosed at the age of 49 with a ìmalignant eye tumor. On physical exam, the child does exhibit an erythematous left eye with mild periorbital edema, periodic nystagmus and strabismus. He also exhibits leukocoria (white pupil). A crude visual exam does reveal decreased visual acuity in the left eye compared to the right eye.

You refer the patient to an ophthalmologist who orders a CT scan which is seen below.

1. Based on the history, physical findings, and CT scan, the patient’s most likely diagnosis is:

A. optic nerve glioma

B. intraocular melanoma

C. intraocular amelanotic melanoma

D. ciliary body melanoma

E. retinoblastoma

F. periorbital abscess

2. The following statement(s) is/are true regarding this patient’s diagnosis:

A. the majority of the patients with this disease process are

diagnosed as adults.

B. the tumor arises from melanocytes

C. the majority of the patients with this disease process are

diagnosed before the age of 6-8 years.

D. the risk of this disease process is 8-10 times greater in whites

compared to blacks.

E. the most common etiology of this process is Staphylococcus

aureus.

3. The following statement(s) is/are true regarding ocular melanomas:

A. Represent the most common malignant ocular tumors in whites.

B. They may arise from the uvea, retina, ciliary body, or iris of the eye.

C. Intraocular melanoma, spindle cell types, have a better prognosis than

ones with an epithelioid component and melanomas with prominent necrosis.

D. Intraocular melanomas do not have the same pattern of metastases as cutaneous melanomas. When intraocular melanomas metastasize they usually metastasize to the liver first.

E. All of the above.

4. Mutation of the Rb gene predisposes individuals to the following tumors:

A. Retinoblastoma

B. Osteosarcoma

C. Glioblastoma multiforme

D. All of the above.

5. The following statement(s) is/are true regarding hereditary retinoblastoma:

A. Patients typically have bilateral tumors.

C. Patients are at risk of trilateral retinoblastoma.

D. Most patients have no previous family history of retinoblastoma.

E. All of the above.

6. Leukocoria in this patient is due to:

A. Gross appearance of the tumor which is white.

B. The lack of melanin production by the tumor

C. The inflammatory response

D. The reduction of vascularity to the retina and iris

E. None of the above

7.The following statement(s) is/are true regarding retinoblastoma:

A. They are considered as one of the "small blue cell tumors" of children

B. If detected early, they have favorable prognosis with a 5 year survival rate approaching 90%

C. Retinocytomas are considered a benign variant of retinoblastoma

D. None of the above

E. All of the above.