Devita, Hellman, and Rosenberg's Cancer

LWBK1006-29 LWW-Govindan-Review December 9, 2011 15:36

398 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Review

Question 29.27. Which of the following statements about the genetics of retinoblastoma

transmission is NOT true?

A. It is transmitted as a highly penetrant autosomal-dominant trait and

is the most common pediatric ocular tumor.

B. The majority of retinoblastomas are hereditary, bilateral, and caused

by germ line mutations of 13q14; only 10% to 15% are sporadic and

unilateral.

C. All sporadic bilateral retinoblastomas also have a germ line mutation

transmitted in an identical manner as familial retinoblastoma.

D. The parents and siblings of all patients with retinoblastoma should

have a thorough ophthalmoscopic evaluation whether or not there is

family history of the disease.

Questions

29.28.–29.31.

An 11-month-old boy who has just learned to walk is noticed to consis-

tently bump into objects in his path. There is no history of major medical

illness in this healthy child. The parents report a “white-looking” eye on

the right side that was not observed during a regular pediatric checkup

at 6 months of age. There is no family history of ophthalmic disease or

tumors. On examination, he has a white pupillary reflex on the right side.

In addition, mild proptosis and a sixth nerve palsy are noticed in the

right eye. The remainder of the physical examination is normal. A biopsy

confirms the presence of retinoblastoma.

Question 29.28. All patients with retinoblastoma should undergo the following staging

studies, EXCEPT:

A. CT of the orbit

B. Magnetic resonance imaging (MRI) of the orbit and head to include

the optic nerve

C. Meticulous inspection under general anesthesia of both eyes

D. Lumbar puncture and cerebrospinal fluid (CSF) cytology

Question 29.29. Further staging studies reveal complete visual loss in the affected eye and

tumor involving the right orbit and optic nerve. The left eye is normal.

CSF, bone, and bone marrow studies are normal. Therapy should include:

A. Cryotherapy or hyperthermia

B. Plaque radiotherapy and chemotherapy

C. Enucleation with a sufficient length of the affected optic nerve

D. Enucleation with a sufficient length of the affected optic nerve and

local radiation

Question 29.30. Enucleation instead of vision-sparing interventions is indicated for all of

the following, EXCEPT:

A. Bilateral neuroblastoma with multiple lesions in both eyes

B. Unilateral retinoblastoma that completely fills the globe

C. Painful glaucoma and loss of vision in one eye

D. Bilateral retinoblastoma with no useful vision in either eye