Radiation Oncology Study Guide

Celine Bicquart Ord, Eric K. Hansen and Charles R. Thomas, Jr. (eds.)Radiation Oncology Study Guide201310.1007/978-1-4614-6400-6_1© Springer Science+Business Media New York 2013

1. Central Nervous System Tumors: Adult and Pediatric

Celine Bicquart Ord¹   and Charlotte Dai Kubicky²  

(1)

Department of Radiation Oncology, Scott & White Memorial Hospital, Temple, TX 76508, USA

(2)

Department of Radiation Medicine, Oregon Health Science University, 3181 SW Sam Jackson Park Road, KPV4, Portland, OR 97239, USA

Celine Bicquart Ord (Corresponding author)

Email: cord@sw.org

Charlotte Dai Kubicky

Email: charlottedai@gmail.com

Abstract

Neurofibromatosis type 1, von Recklinghausen’s disease, is most commonly associated with what type of intracranial tumor?

Questions

1.

Neurofibromatosis type 1, von Recklinghausen’s disease, is most commonly associated with what type of intracranial tumor?

A.

Meningioma

B.

Astrocytoma

C.

Glioblastoma

D.

Oligodendroglioma

2.

What type of intracranial tumor is most commonly seen with neurofibromatosis type 2?

A.

Schwannoma

B.

Meningioma

C.

Astrocytoma

D.

Oligodendroglioma

3.

All of the following are correct regarding Turcot’s syndrome except:

A.

There is an association with familial polyposis of the colon.

B.

There is an association with medulloblastoma.

C.

There is an association with high-grade glioma.

D.

There is an association with low-grade glioma.

4.

All of the following are tumors associated with tuberous sclerosis except:

A.

Hemangioblastoma

B.

Angiofibromas

C.

Hamartomas

D.

Subependymal giant cell tumors

5.

Cerebellar, brainstem, and spinal cord hemangioblastomas are frequently seen in which disorder?

A.

Cowden’s syndrome

B.

Turcot’s syndrome

C.

Von Hippel-Lindau disease

D.

Gorlin’s syndrome

6.

In which of the following tumors does survival increase with increasing age?

A.

Glioblastoma multiforme

B.

Oligodendroglioma

C.

Anaplastic astrocytoma

D.

Brainstem glioma

7.

MDM2 amplification/overexpression is a genetic event frequently seen in what tumor?

A.

Secondary glioblastoma multiforme

B.

Primary glioblastoma multiforme

C.

Anaplastic astrocytoma

D.

Oligodendroglioma

8.

Deletion of 1p and 19q in oligodendroglioma is associated with:

A.

Better response to therapy but similar progression-free and overall survival

B.

Better response to therapy with improved progression-free but similar overall survival

C.

Similar response to therapy with improved progression-free and overall survival

D.

Better response to therapy with improved progression-free and overall survival

9.

Methylation of the O⁶-methylguanine-DNA methyltransferase, MGMT, promoter is associated with:

A.

Decreased MGMT expression and improved overall survival

B.

Decreased MGMT expression and decreased overall survival

C.

Increased MGMT expression and improved overall survival

D.

Increased MGMT expression with no effects on overall survival

10.

The most common presenting symptom of low-grade astrocytoma is:

A.

Headache

B.

Weakness

C.

Personality change

D.

Seizure

11.

Which one of the following is not a negative risk factor in the prognostic ­scoring system of low-grade gliomas developed by Pignatti and colleagues?

A.

Astrocytoma histology subtype

B.

Tumor crossing the midline

C.

Age 40 years or less

D.

Presence of neurological deficit before surgery

12.

All of the following are true regarding the EORTC 22844 Believer’s Trial in low-grade glioma except:

A.

Only 25 % of patients underwent gross total resection.

B.

45 Gy was the radiation dose of one arm in the study.

C.

50.4 Gy was the radiation dose of one arm in the study.

D.

Overall survival did not improve with dose escalation.

13.

According to the long-term results of EORTC 22845 in which early versus delayed radiotherapy for resected low-grade gliomas was compared, which of the following is not true?

A.

Progression-free survival improved in the early radiotherapy group.

B.

Overall survival was not different between the early versus delayed groups.

C.

Seizures were better controlled at 1 year in the delayed radiotherapy group.

D.

Total dose of radiotherapy given was 54 Gy.

14.

In the EORTC 26951 trial, patients with low-grade glioma were randomized after surgery and radiation to +/− chemotherapy. Which statement regarding this trial is false?

A.

Chemotherapy consisted of PCV (procarbazine, lomustine, and vincristine).

B.

The initial published results showed only a progression-free survival benefit.

C.

The most recent results still show no overall survival benefit.

D.

Median survival has not yet been reached in those with 1p19q deletion that received adjuvant PCV chemotherapy.

15.

Regarding magnetic resonance spectroscopy (MRS), which of the following is NOT true?

A.

Brain tumors are characterized by an elevated choline to creatine ratio.

B.

Radiation change on MRS is characterized by decreased choline levels.

C.

The MR spectrum of brain tumors is characterized by an increasing Hunter’s angle from left to right.

D.

Radiation change on MRS is characterized by decreased N-acetylaspartate.

16.

In a four-arm ECOG/RTOG trial for high-grade gliomas comparing a standard 60 Gy to 3 experimental arms, which is NOT true?

A.

A 10 Gy boost in addition to the standard 60 Gy WBRT improved survival.

B.

40–60 year olds in the 60 Gy + BCNU arm had significantly increased survival.

C.

The 60 Gy + methyl-CCNU and DTIC arm was more toxic than the 60 Gy + BCNU arm.

D.

Age was found to be the most important prognostic factor.

17.

In a dose escalation study of malignant gliomas performed by Lee et al. at the University of Michigan, which of the following is NOT true?

A.

Dose-limiting toxicity was not seen.

B.

There was no trend in improved overall survival despite dose escalation.

C.

Despite dose escalation, the majority of tumors progressed in-field.

D.

In addition to local progression, there was extensive distant progression.

18.

Regarding RTOG 90-05, a radiosurgery dose-finding study, which of the following is true?

A.

Dose-limiting toxicity was defined as irreversible grade 2 neurotoxicity.

B.

The maximum tolerated dose for tumors 2–3 cm was 15 Gy.

C.

Patients with gliomas are more likely to fail locally compared to those with metastases.

D.

Approximately two-thirds of patients failed locally.

19.

Regarding brachytherapy in the brain for malignant glioma, all of the following are true except:

A.

In a Brain Tumor Cooperative Group study, there was no survival benefit seen with the addition of a brachytherapy boost.

B.

The GliaSite RT system uses an intracavitary device filled with an ¹⁹²Ir solution.

C.

In using an intracavitary balloon for brachytherapy, there is not a true point source.

D.

Smaller balloon fill volumes may result in higher doses at the balloon surface, possibly leading to increased radiation necrosis.

20.

All of the following regarding temozolomide are true except:

A.

It is an alkylating agent with a half-life of minutes.

B.

Hypermethylation of the MGMT promoter leads to increased sensitivity to temozolomide.

C.

It was first approved as a single agent in recurrent glioma.

D.

Concurrent with radiation for newly diagnosed glioblastoma, it is given daily at 150mg/m².

21.

What were the 5-year overall survivals with and without temozolomide, respectively, reported in the latest (2009) update of the Stupp trial?

A.

10 %, 2 %

B.

2 %, 10 %

C.

27 %, 10 %

D.

10 %, 27 %

22.

According to the 2009 update of the Stupp trial, radiation +/− temozolomide for glioblastoma, what was the strongest predictor of outcome?

A.

Extent of resection

B.

Age

C.

Performance status

D.

MGMT status

23.

Which of the following targets/therapies for glioblastoma multiforme is incorrect?

A.

EGFR/erlotinib

B.

Farnesyl transferase/tipifarnib

C.

EGFR/trastuzumab

D.

VEGF/bevacizumab

24.

What was the median survival in those elderly patients treated with radiotherapy as opposed to best supportive care, as reported by Bauman et al.?

A.

4 months

B.

6 months

C.

8 months

D.

10 months

25.

Comparing standard fractionation to hypofractionated radiotherapy for poor prognosis GBM patients aged 60 years or older, Roa et al. found:

A.

Improved survival in the standard fractionation arm.

B.

Improved survival in the hypofractionated arm.

C.

Those receiving standard fractionation were more likely to require an increase in steroids post-treatment.

D.

More patients in the hypofractionated arm did not complete their planned treatment course.

26.

What was the prescribed dose of radiotherapy in the randomized glioblastoma multiforme in the elderly study, as reported by Keime-Guibert et al.?

A.

30 Gy

B.

45 Gy

C.

50.4 Gy

D.

60 Gy

27.

All of the following tumors have a propensity for distant dissemination except:

A.

CNS germ cell tumors

B.

Ependymoma

C.

Choroid plexus tumors

D.

Meningioma

28.

Maximal resection is an important initial component in therapy in all of the following except:

A.

Glioblastoma multiforme

B.

Meningioma

C.

CNS germinoma

D.

Chondrosarcoma

29.

All of the following are risk factors for the development of meningioma except:

A.

Female sex

B.

Neurofibromatosis type 2

C.

Prior exposure to ionizing radiation

D.

Asian race

30.

What pathological feature is frequently present in meningioma?

A.

Psammoma bodies

B.

Rosenthal fibers

C.

Pseudorosettes

D.

Gemistocytes

31.

Which of the following pathways of spread of meningioma is least common?

A.

Growth along the base of skull

B.

Growth along meningeal surfaces

C.

Growth with invasion into the brain

D.

Growth with invasion of adjacent dural or superior sagittal sinuses

32.

Which statement is incorrect regarding 5-year progression-free survival (5-year PFS) of meningioma, as reported by Goldsmith et al.?

A.

Gross totally resected benign meningioma has a 5-year PFS of >80 %.

B.

Subtotally resected benign meningioma has a 5-year PFS of approximately 50 %.

C.

The addition of radiation after subtotally resected benign meningioma raises progression-free survival to >85 %.

D.

The addition of radiation after resection of malignant meningioma raises progression-­free survival to >70 %.

33.

Which is the most typical appearance of meningioma on magnetic resonance imaging?

A.

Isointense on pre-contrast T1, no enhancement with gadolinium contrast

B.

Isointense on pre-contrast T1, uniform enhancement with gadolinium contrast

C.

Hyperintense on pre-contrast T1, no enhancement with gadolinium contrast

D.

Hyperintense on pre-contrast T1, uniform enhancement with gadolinium contrast

34.

In a low-grade oligodendroglioma, which of the following radiographic patterns would most typically be noted on magnetic resonance imaging?

A.

Enhances on T1 after gadolinium administration, hypointense on T1 pre-contrast

B.

Non-enhancing on T1 after gadolinium administration, hypointense on T1 pre-contrast

C.

Non-enhancing on T1 after gadolinium administration, hyperintense on T1 pre-contrast

D.

Enhances on T1 after gadolinium administration, hyperintense on T1 pre-contrast

35.

All of the following are rationales for the use of stereotactic radiosurgery to treat meningioma except:

A.

Lesions are typically well circumscribed.

B.

Alpha/beta ratio is similar to that of late-reacting tissue.

C.

Improvement in local control of subtotally resected lesions.

D.

Treatment side effects are independent of tumor location.

36.

In treating parasellar meningiomas with SRS, what dose limit to the optic apparatus is suggested to reduce the risk of optic neuropathy?

A.

12 Gy

B.

8 Gy

C.

6 Gy

D.

5 Gy

37.

Rosenthal fibers are a pathognomic feature of which tumor type?

A.

Pleomorphic xanthoastrocytomas

B.

Oligodendrogliomas

C.

Germinomas

D.

Pilocytic astrocytomas

38.

Which one of the following tumor/pathologic feature pairings is incorrect?

A.

Glioblastoma/necrosis

B.

Medulloblastoma/pseudorosettes

C.

Craniopharygioma/cystic areas

D.

Oligodendroglioma/fried egg appearance

39.

Comparing the incidence in adults and children, ependymomas tend to be:

A.

Intracranial in adults and supratentorial in children

B.

Spinal in adults and infratentorial in children

C.

Spinal in adults and supratentorial in children

D.

Intracranial in adults and infratentorial in children

40.

Which of the following subtypes of ependymoma has the worst prognosis?

A.

Myxopapillary

B.

Anaplastic

C.

Solid squamous papillary

D.

Cellular

41.

Which of the following statements about ependymoma is false?

A.

Gross total resection is achieved more commonly in infratentorial compared to supratentorial tumors.

B.

The predominant pattern of failure is local.

C.

Extent of resection is the most important prognostic factor.

D.

Radiotherapy dose is typically 59.4 Gy.

42.

Which of the following is not a common site of presentation of chordoma?

A.

Base of skull

B.

Lower cervical spine

C.

Lumbar spine

D.

Sacrum

43.

Protons are used in place of photons for some chordomas due to all but which of the following:

A.

Steep dose gradients of protons

B.

Excellent 5-year local control rates

C.

Higher RBE of protons

D.

Proximity of tumor to critical structures

44.

All of the following are prognostic factors of the RTOG RPA classification for high-grade glioma developed by Curran et al. except:

A.

Age >60

B.

Histology

C.

Performance status

D.

Extent of resection

45.

All are true regarding the new RTOG RPA analysis for glioblastoma multiforme developed by Li et al. except:

A.

It includes only 4 variables, compared to the prior RPA analysis, which included 6.

B.

Classes V and VI are combined due to indistinguishable survival.

C.

Extent of resection is no longer one of the four variables in the new RPA analysis.

D.

This new RPA analysis will be used for future RTOG glioblastoma multiforme trials.

46.

All of the following are true regarding the RTOG study 94-02 (Cairncross et al.) looking at outcomes in pure and mixed oligodendroglioma receiving postoperative radiotherapy +/− PCV chemotherapy, except:

A.

Progression-free survival was improved with the addition of PCV chemotherapy.

B.

Overall survival was not improved with the addition of PCV chemotherapy.

C.

Those tumors with 1p and 19q allelic loss had median survival times > 7 years.

D.

There was no increased toxicity with the addition of PCV chemotherapy to radiotherapy.

47.

How many mitoses per 10 high-powered fields constitute anaplastic meningioma?

A.

5

B.

10

C.

15

D.

20

48.

What is the grading system used to describe completeness of resection of meningiomas?

A.

Simpson

B.

Brooker

C.

Bloom-Richardson

D.

Kadish

49.

What is the most common site for a CNS germ cell tumor?

A.

Anterior pituitary

B.

Posterior pituitary

C.

Pineal gland

D.

Suprasellar region

50.

What syndrome is commonly seen with pineoblastoma?

A.

Eaton-Lambert

B.

Parinaud’s

C.

Limbic encephalitis

D.

Cushing’s

51.

Which of the following pineal tumors has the LEAST propensity for craniospinal dissemination?

A.

Pineoblastoma

B.

Germinoma

C.

Non-germinomatous germ cell tumor

D.

Pineocytoma

52.

All of the following statements regarding hemangioblastomas and hemangiopericytomas are true except:

A.

While hemangioblastomas are indolent and slow growing, hemangiopericytomas are locally aggressive.

B.

Hemangioblastomas and hemangiopericytomas are both associated with Von Hippel-Lindau.

C.

Hemangiopericytoma, in contrast to hemangioblastoma, shows a propensity for local recurrence after resection.

D.

Surgical resection is the primary therapy for both tumors.

53.

Regarding craniopharyngiomas, all of the following are true except:

A.

They are derived from remnants of Rathke’s pouch.

B.

There is a bimodal incidence.

C.

Calcification is infrequently seen.

D.

Diabetes insipidus is a frequent complication of surgery.

54.

What percent of pituitary adenomas are nonsecretory?

A.

30 %

B.

40 %

C.

50 %

D.

60 %

55.

Which of the following hormones is secreted by the neurohypophysis?

A.

ADH

B.

ACTH

C.

GH

D.

TSH

56.

Which one of the following hormones secreted by a pituitary adenoma has no effective medical therapy?

A.

Prolactin

B.

Thyroid-stimulating hormone

C.

Adrenocorticotropic hormone

D.

Growth hormone

57.

After radiation to the pituitary axis, which is most likely to be the first hormone to be affected?

A.

Gonadotropin-releasing hormone

B.

Thyroid-stimulating hormone

C.

Adrenocorticotropic hormone

D.

Growth hormone

58.

What is the most caudal structure transmitted through the cavernous sinus?

A.

CN III

B.

CN V1 (V one)

C.

CN V2

D.

CN VI (six)

59.

What is the most frequent primary ocular tumor?

A.

Melanoma

B.

Lymphoma

C.

Retinoblastoma

D.

Optic nerve sheath meningioma

60.

Which of the following primary ocular tumors occurs more frequently in women than men?

A.

Melanoma

B.

Lymphoma

C.

Retinoblastoma

D.

Optic nerve sheath meningioma

61.

Which is the most common tumor type to metastasize to the orbit?

A.

Non-small-cell lung cancer

B.

Hepatocellular carcinoma

C.

Breast cancer

D.

Small-cell lung cancer

62.

Which of the following Collaborative Ocular Melanoma Study (COMS) ­classifications is incorrect?

A.

Small melanoma: 1–3 mm thick

B.

Small melanoma: < 10 mm in largest tumor dimension

C.

Medium melanoma: 3–8 mm thick

D.

Large melanoma: > 16 mm in largest tumor dimension

63.

Which of the following is true regarding the COMS study for large choroidal melanomas?

A.

Patients were randomized to enucleation versus plaque brachytherapy.

B.

Patients were randomized to enucleation versus external beam radiotherapy.

C.

Patients were randomized to enucleation +/− plaque brachytherapy.

D.

Patients were randomized to enucleation +/− preoperative external beam radiotherapy.

64.

Acoustic neuromas most commonly affect which cranial nerve?

A.

Cranial nerve V

B.

Cranial nerve VII

C.

Cranial nerve VIII

D.

Cranial nerve IX

65.

Which of the following is not an embryonal CNS tumor?

A.

Medulloblastoma

B.

Atypical teratoid/rhabdoid tumor

C.

Germinoma

D.

Pineoblastoma

66.

What is the typical radiotherapy regimen for embryonal CNS tumors?

A.

None

B.

Craniospinal irradiation

C.

Focal radiation

D.

Whole ventricular radiation

67.

Which of the following molecular subtypes of medulloblastoma has the best prognosis?

A.

Wnt-pathway-associated subtype

B.

Hh-pathway-associated subtype

C.

c-MYC-pathway-associated subtype

D.

Other subtypes

68.

What is the superior border of the posterior fossa?

A.

Clivus

B.

Tentorium cerebelli

C.

Calvarium

D.

Occipital bone

69.

What would be the recommended radiotherapy regimen for standard-risk medulloblastoma?

A.

Craniospinal radiation to 36 Gy, followed by a boost to the posterior fossa to a total of 54 Gy in combination with multiagent chemotherapy

B.

Craniospinal radiation to 54 Gy in combination with multiagent chemotherapy

C.

Craniospinal radiation to 23.4 Gy, followed by a boost to the posterior fossa to a total of 54 Gy in combination with multiagent chemotherapy

D.

Craniospinal radiation to 23.4 Gy in combination with multiagent chemotherapy

70.

In a St. Jude Children’s Research trial prospectively evaluating focal treatment of the tumor bed, as opposed to treatment of the entire posterior fossa, all of the following are true except:

A.

Dose to the entire posterior fossa was 36 Gy, followed by a conformal tumor bed boost to 54 Gy.

B.

3-year posterior fossa failure was 6.3 %.

C.

Chemotherapy was given after radiation.

D.

Dose reduction to the posterior fossa was over 10 %.

71.

Which is not a component of therapy for children < 3 years with medulloblastoma?

A.

Surgery

B.

Chemotherapy

C.

Craniospinal irradiation

D.

Posterior fossa irradiation

72.

Which is the least common site of ependymoma in children?

A.

Posterior fossa arising from roof of fourth ventricle

B.

Parenchyma of cerebral hemispheres

C.

Cerebellopontine angle

D.

Spinal cord

73.

All of the following are true regarding ependymomas except:

A.

Most recurrences are local.

B.

The most common histology in the cauda equina is myxopapillary.

C.

More ependymomas occur supratentorially, rather than infratentorially.

D.

Extent of surgery is the most important predictor of outcome.

74.

What is the most appropriate radiotherapy dose after gross total resection of a localized ependymoma?

A.

50.4 Gy

B.

54 Gy

C.

55.8 Gy

D.

59.4 Gy

75.

What is the most appropriate next step in management of a completely resected low-grade astrocytoma of the cerebellum in children and young adults?

A.

Focal radiotherapy

B.

Chemotherapy

C.

Observation

D.

Posterior fossa radiotherapy

76.

All of the following are true regarding germinomas except:

A.

Diagnosis does not require a biopsy.

B.

Germinomas are radiosensitive.

C.

Germinomas are chemosensitive.

D.

Whole ventricular radiation is appropriate for localized germinoma.

77.

All of the following are true regarding craniopharyngiomas except:

A.

Endocrine abnormalities are infrequent.

B.

Derived from embryonic remnant of the anterior pituitary.

C.

Commonly present with mixed solid/cystic components.

D.

Possesses a bimodal age of incidence.

78.

All of the following are true regarding management of craniopharyngiomas except:

A.

Subtotal resection + radiation and gross total resection alone have equivalent outcomes.

B.

Radical surgery has been shown to be associated with loss of more IQ points compared to subtotal surgery.

C.

There is no radiation dose–response in terms of local control of craniopharyngioma.

D.

Hypopituitarism at presentation predicts for treatment-related morbidity.

79.

What is the next best step in management of an asymptomatic optic glioma in a 7-year-old child?

A.

Close observation

B.

Surgery

C.

Fractionated radiation

D.

Chemotherapy

80.

What gene mutation is seen in over 80 % of atypical teratoid/rhabdoid tumors?

A.

INI-1

B.

Wnt

C.

Hh

D.

c-MYC

81.

Impingement of what structure results in Parinaud’s syndrome, commonly seen in pineoblastoma?

A.

Optic nerve

B.

Optic chiasm

C.

Superior colliculi

D.

Inferior colliculi

82.

What other tumor can be seen in children that have bilateral retinoblastomas?

A.

Pineocytoma

B.

Pituitary adenoma

C.

Pineoblastoma

D.

Anaplastic ependymoma

Answers and Rationales

1.

The correct answer is B. Intracranial astrocytomas of any grade can be seen. Pilocytic astrocytomas (WHO I) are particularly common. Brown PD, Shaw EG, van den Bendt MJ. Low-grade gliomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 443–59.

2.

The correct answer is A. Although meningiomas and astrocytomas can be seen with NF2, schwannomas, particularly bilateral acoustic neuromas are most commonly seen. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

3.

The correct answer is D. Turcot’s is an inherited syndrome associated with the biallelic DNA mismatch repair mutations, with an association between familial polyposis of the colon and brain tumors (medulloblastoma, malignant glioma). Turcot J et al. Malignant tumors of the central nervous system associated with familial polyposis of the colon and bright green urine which may be related to vegetables in the diet: report of two cases. Dis Colon Rectum. 1959;2:465–8.

4.

The correct answer is A. Tuberous sclerosis is associated with angiofibromas, hamartomas, and subependymal giant cell tumors, but not hemangioblastomas. The latter are associated with Von Hippel-Lindau.

5.

The correct answer is C. Von Hippel-Lindau is an autosomal dominant disorder, caused by a tumor suppressor gene on chromosome 3. Cerebellar, brainstem, and spinal cord hemangioblastomas are associated with VHL. Cowden’s syndrome (multiple hamartoma syndrome) is an autosomal dominant inherited syndrome resulting from mutation in the PTEN gene on arm 10q that causes hamartomatous neoplasms of the skin, mucosa, GI tract, bones, CNS, eyes, and genitourinary tract. The skin is involved in 90–100 % of cases; the thyroid is involved in 66 % of cases. Turcot’s is an inherited syndrome associated with the biallelic DNA mismatch repair mutations, with an association between familial polyposis of the colon and brain tumors (medulloblastoma, malignant glioma). Gorlin’s is an autosomal dominantly inherited disorder also known as nevoid basal cell carcinoma syndrome, caused by mutations in the PTCH gene on chromosome 9q. In addition to the development of basal cell carcinomas, benign odontogenic keratocysts of the jaw are seen in 75 % of patients, usually occurring at a young age. Less commonly seen are ovarian or cardiac fibromas, and/or medulloblastomas (seen in children ≤ 2 years). Liaw D et al. Germline mutations of the PTEN gene in Cowden disease, an inherited breast and thyroid cancer syndrome. Nat Genet. 1997;16(1):64–7. Lloyd KM II, Dennis M. Cowden’s disease. A possible new symptoms complex with multiple system involvement. Ann Intern Med. 1963;58:136–42. Turcot J et al. Malignant tumors of the central nervous system associated with familial polyposis of the colon and bright green urine which may be related to vegetables in the diet: report of two cases. Dis Colon Rectum. 1959;2:465–8. Gorlin R, Goltz R. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med. 1960;262(18):908–12.

6.

The correct answer is D. Whereas survival decreases with increasing age in glioblastoma multiforme, oligodendroglioma, and anaplastic astrocytoma, it improves in brainstem glioma with increasing age. Freeman CR, Farmer JP. Pediatric brain stem gliomas: a review. IJROBP. 1998;40:265–71. Guillamo JS, Monjour A, Taillandier L, et al. Brainstem gliomas in adults: prognostic factors and classification. Brain 2001;124:2528–39.

7.

The correct answer is B. MDM2 overexpression is frequently seen in primary glioblastoma, while loss of 19q and IDH1 positivity is associated with secondary glioblastoma. Genetics of glioma progression and the definition of primary and secondary glioblastoma. Brain Pathol. 1997;7:1131–36. The next generation of glioma biomarkers: MGMT methylation, BRAF mutations, and IDH1 mutations. Von Diemling A, Korshunov A, Hartmann C. Brain Pathol. 2011;1:74–87.

8.

The correct answer is D. 1p and 19q deletion is an established prognostic factor for anaplastic oligodendrogliomas. Hoang-Xuan et al. described their prospective trial results, which showed loss of 1p correlated with objective tumor response. In analyzing 1p19 status of 100 low-grade glioma patients enrolled on two North Central Cancer Treatment Group Protocols, Buckner et al. found that 1p and 19q deletions were associated with superior 5-year progression-free and overall survival. Hoang-Xuan K, Capelle L, et al. Temozolomide as initial treatment for adults with low-grade oligodendrogliomas or oligoastrocytomas and correlation with chromosome 1p deletions. J Clin Oncol. 2004;22:3133–8. Bucker JC, Ballman KV, et al. Diagnostic and prognostic significance of 1p and 19q deletions in patients (pts) with low-grade oligodendroglioma and astrocytoma. J Clin Oncol. 2005;23:1145.

9.

The correct answer is A. MGMT is a DNA repair enzyme that repairs damage induced by alkylating agents, such as temozolomide. Methylation of the MGMT promoters results in epigenetic silencing and thus decreased MGMT expression. After determining the methylation status of glioblastoma patients randomized to radiation +/− temozolomide, Hegi et al. found MGMT promoter methylation to be associated with improved survival. Hegi ME et al. MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med. 2005;352:997–1003.

10.

The correct answer is D. Seizure is the most common presenting symptom, occurring in approximately two-thirds of patients. Brown PD, Shaw EG, van den Bendt MJ. Low-grade gliomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 443–59.

11.

The correct answer is C. Pignatti et al. used the databases of low-grade glioma patients from EORTC trials 22844 and 22845 to develop a prognostic scoring system using imaging, patient, and tumor characteristics. The following were found by Cox regression to be negative risk factors: age 40 years or more, astrocytoma histology subtype, largest diameter of the tumor ≥6 cm, tumor crossing the midline, and presence of neurological deficit before surgery. Two or more risk factors comprised the low-risk group, with a median survival of 7.7 years. Three or more risk factors defined a high-risk group with a median survival of 3.2 years. Pignatti F, van den Bent M, et al. Prognostic factors for survival in adult patients with cerebral low-grade glioma. J Clin Oncol. 2002;20:2076–84.

12.

The correct answer is B. In EORTC 22844, the Believer’s Trial, patients with low-grade glioma were randomized to immediate postoperative RT with either 45 Gy or 59.4 Gy. Only 25 % underwent gross total resection prior to radiation. Dose escalation did not result in improved survival, nor progression-free survival. Karim AB et al., A randomized trial on dose–response in radiation therapy of low-grade cerebral glioma: European Organization for Research and Treatment of Cancer (EORTC) Study 22844. Int J Radiat Oncol Biol Phys. 1996;36(3):549–56.

13.

The correct answer is C. Long-term results of the EORTC 22845 randomized trial that compared early versus delayed radiotherapy for low-grade gliomas were published in the Lancet in 2005. While progression-free survival improved in the early radiotherapy arm (5.3 years vs. 3.4 years), there was no difference in overall survival (7.4 vs. 7.2 years). There was better seizure control in the early radiotherapy group at 1 year (25 % with seizures in early group vs. 41 %, p = 0.03). Median dose delivered was 54 Gy in 30 fractions. Van de Bent MJ. et al. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Lancet. 2005;355:985–90.

14.

The correct answer is C. The EORTC 26951 trial randomized 368 patients with anaplastic oligodendroglioma after surgery and radiation to either observation or adjuvant PCV chemotherapy (procarbazine, lomustine, vincristine). The initial published 5-year results in 2006 showed only a progression-free survival benefit (1.9 years vs. 1.1 years). 1p19q deletion was subsequently evaluated, and those with 1p19q deletion had a 5-year overall survival of 74 % versus 30 % without the deletion. With 12 years of follow-up, the addition of PCV chemotherapy not only improves progression-free survival, but also improves overall survival. Median survival is significantly prolonged (42.3 months vs. 30.6 months) in those that received adjuvant PCV. In those with 1p19q deletion that received adjuvant PCV, median survival has not been reached. Van den Bent MJ et al. First analysis of EORTC 26951, a randomised phase III study of adjuvant PCV chemotherapy in patients with highly anaplastic oligodendroglioma. J Clin Oncol. 2006;24(18):2715–22. Van den Bent MJ et al. Long-term follow-up results of EORTC 26951 a randomised phase III study of adjuvant PCV chemotherapy in anaplastic oligodendroglial tumors (AOD). J Clin Oncol. 2012;(Suppl;abstr 2).

15.

The correct answer is C. Magnetic resonance spectroscopy is a method to monitor biochemical changes in the brain. The most commonly used brain metabolites in MRS are choline, a measure of increased cell turnover, which is elevated in tumors and inflammatory processes; creatine, a measure of energy stores, decreased in tumors; and N-acetylaspartate (NAA), a neuronal marker which decreases in processes that adversely affect neuron integrity. The MR spectrum shows various peaks of these metabolites, displayed from left to right—choline, creatine, and NAA. Hunter’s angle is a line drawn along the peaks of the white matter metabolites and is generally downward in brain tumors (denoting an increased choline to creatine ratio). Radiation necrosis on MRS is usually denoted by low levels of NAA, creatine, and choline. Nelson SJ et al. Characterization of untreated gliomas by magnetic resonance imaging. Neuroimag Clin. 2002;12:599–613. Graves EE et al. Serial proton MR spectroscopic imaging of recurrent malignant gliomas after gamma knife radiosurgery. AJNR. 2001;22:613–24.

16.

The correct answer is A. To attempt to determine an optimal radiation dose for malignant gliomas, ECOG/RTOG performed a four-arm study, comparing 3 experimental arms:(1) 60 Gy WBRT + 10 Gy partial brain boost, (2) 60 Gy WBRT + BCNU, and (3) 60 Gy WBRT + CCNU + dacarbazine (DTIC) against a standard 60 Gy WBRT arm. There was no survival benefit seen with the 10 Gy boost. Age was found to be the most important prognostic factor, with 18-month survival 64 % in those <40 years, versus 20 % in those 40–60 years, and 8 % in those >60 years. Among the 40–60 year-old group, the addition of BCNU did improve survival compared to 60 Gy WBRT alone. Not unexpectedly, the combination of CCNU + DTIC was more toxic than BCNU, producing a higher incidence of thrombocytopenia. Chang CH et al. Comparison of postoperative radiotherapy and combined postoperative radiotherapy and chemotherapy in the multidisciplinary management of malignant gliomas. A Joint Radiation Therapy Oncology Group and Eastern Cooperative Oncology Group Study. Cancer. 1983;(6):997–1007.

17.

The correct answer is D. Lee et al. reported on the patterns of failure following high-dose 3-D conformal radiotherapy of high-grade astrocytomas. All patients were treated to either 70 or 80 Gy. An in-field recurrence was defined as 80 % or more of the volume of the recurrence lying within the 95 % isodose region. It was termed as outside when 20 % or less of the volume of the recurrence was inside the 95 % isodose region. Of the 36 evaluable patients, 89 % failed in-­field, with only 1 failing outside the high-dose region. Despite the dose escalation, there was no improvement in overall survival. No dose-limiting toxicity was seen. Lee SW et al. Patterns of failure following high-dose 3-D conformal radiotherapy for high-grade astrocytomas: a quantitative dosimetric study. Int J Radiat Oncol Biol Phys. 1999;43:79–88.

18.

The correct answer is C. RTOG 90-05 sought to define optimal doses for radiosurgery in patients with recurrence malignant brain tumors, including both gliomas and metastases. Patients were stratified by tumor volume: <2 cm, 2–3 cm, and 3.1–4 cm; dose-limiting toxicity was defined as irreversible grade 3 neurotoxicity or any grade 4/5 neurotoxicity seen within 3 months of SRS. While the maximum tolerated dose was not reached in tumors <2 cm, dose was not escalated beyond 24 Gy. Maximum tolerated dose for the 2–3 cm and 3.1–4 cm tumors was 18 Gy and 15 Gy, respectively. Approximately half (48 %) of patients failed locally, with local failure more likely in those with gliomas compared to those with metastases. Shaw EG et al. Single dose radiosurgical treatment of recurrent previously irradiated primary brain tumors and brain metastases: final report of RTOG protocol 90-05. Int J Radiat Oncol Biol Phys. 1996;34:647–54.

19.

The correct answer is B. The Brain Tumor Cooperative Group study 8701 randomized patients with malignant glioma post resection to temporary ¹²⁵I brachytherapy followed by EBRT + BCNU or EBRT and BCNU alone. No survival benefit was seen in the brachytherapy boost group. The GliaSite RT system uses an ¹²⁵I solution injected into a closed catheter balloon. With a balloon, there is not a true point source, and with smaller fill volumes, the surface dose can be much higher. This can theoretically lead to higher rates of radiation necrosis. Selker RG et al. The brain tumor cooperative group NIH trial 87-01: a randomized comparison of surgery, external radiotherapy, and carmustine versus ­surgery, interstitial radiotherapy boost, external radiation therapy, and carmustine. Neurosurgery. 2002;51:343–55.

20.

The correct answer is D. Temozolomide is an alkylating agent with a half-­life of minutes that leads to methylation of the O-6 position of guanine. Unrepaired, this will lead to double-strand breaks. Although now the standard of care in newly diagnosed glioblastoma multiforme due to the overall survival benefit seen in Stupp’s landmark trial, it was first studied and FDA approved in recurrent glioma. Yung and colleagues randomized patients with recurrent glioma to either procarbazine or temozolomide and found that those on the temozolomide arm were more likely to objectively respond and remain progression-free at 6 months. In Stupp’s study, temozolomide was given concurrently with radiation (60 Gy), at a daily dose of 75 mg/m², and adjuvantly for 6 cycles at 150–200 mg/m². The latest update published in 2009 in The Lancet showed that the overall survival benefit persisted: 5-year survival 9.8 % versus 1.9 %. MGMT is a DNA repair enzyme that repairs this damage induced by temozolomide. Methylation of the MGMT promoters results in epigenetic silencing and thus decreased MGMT expression. After determining the methylation status of glioblastoma patients randomized to radiation +/− temozolomide, Hegi et al. found MGMT promoter methylation to be associated with improved survival. Hegi ME et al. MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med. 2005;352:997–1003. Yung WK, Albright RE, et al. A phase II study of temozolomide vs. procarbazine in patients with glioblastoma multiforme at first relapse. Br J Cancer. 2000;83:588–93. Stupp R et al. Effects of radiotherapy with concomitant and adjuvant temozolomide versus radiotherapy alone on survival in glioblastoma in a randomised phase III study: 5-year analysis of the EORTC-NCIC trial. The Lancet Oncol. 2009;10(5):459–66.

21.

The correct answer is A. In Stupp’s study, temozolomide was given concurrently with radiation (60 Gy), at a daily dose of 75 mg/m², and adjuvantly for 6 cycles at 150–200 mg/m². The initial report in 2005 showed an improvement in median survival with the addition of temozolomide: 14.6 versus 12.1 m. Two-­year survival was also improved (26.5 % vs. 10.4 %). The latest update published in 2009 in The Lancet showed that the overall survival benefit persisted:5-year survival 9.8 % versus 1.9 %. Stupp R et al. Effects of radiotherapy with concomitant and adjuvant temozolomide versus radiotherapy alone on survival in glioblastoma in a randomised phase III study: 5-year analysis of the EORTC-NCIC trial. The Lancet Oncol. 2009;10(5):459–66.

22.

The correct answer is D. The latest update published in 2009 in The Lancet showed that the overall survival benefit persisted: 5-year survival 9.8 % versus 1.9 %. MGMT status was retrospectively determined and found to be the strongest predictor of outcome. The benefit of combined modality therapy extended to all clinical subgroups, even those aged 60–70 years. Stupp R et al. Effects of radiotherapy with concomitant and adjuvant temozolomide versus radiotherapy alone on survival in glioblastoma in a randomised phase III study: 5-year analysis of the EORTC-NCIC trial. The Lancet Oncol. 2009;10(5):459–66.

23.

The correct answer is C. EGFR is overexpressed in 50 % of GBMs. Erlotinib and gefitinib, small molecule inhibitors of EGFR tyrosine kinase activity, are currently being tested in combination with radiation for glioblastoma. The farnesyl transferase inhibitor, tipifarnib, has been studied in phase I and II trials of recurrent malignant glioma. Trastuzumab is a monoclonal antibody that interferes with the HER-2 receptor, most commonly used in breast cancer. Bevacizumab has been studied in phase I/II trials in the setting of recurrent glioblastoma. Wong AJ et al. Increased expression of the epidermal growth factor receptor gene in malignant gliomas is invariably associated with gene amplification. Proc Natl Acad Sci USA. 1987;84:6899–903. Volgelbaum M et al. Initial Experience with the EGFR tyrosine Tarceva (OSI-774) for single agent therapy of recurrent/progressive glioblastoma multiforme. Neuro-oncology 2003;5:356. Chakravarti A et al. An update of phase I data from RTOG 0211: a phase I/II clinical study of ZD 1839 (Gefitinib) + radiation for newly diagnosed glioblastoma patients [abstract]. Neuro-oncology. 2004;6:372. Cloughsey TF et al. Two phase II trials of R115777 (Zarnestra) in patients with recurrent glioblastoma multiforme (GBM): a comparison of patients on enzyme-inducing anti-epileptic drugs (EIAED) and not on EIAED at maximum tolerated dose respectively: a North American Brain Tumor Consortium (NABTC) report [abstract]. Neuro-­oncology. 2004;5:349.

24.

The correct answer is D. In this single-arm prospective trial, elderly patients ≥ 60 years and KPS ≤50 were treated with short-course radiotherapy (30 Gy/10 fractions). When comparing these patients with historical controls of those receiving only best supportive care, median survival was improved from 1 to 10 m. Bauman GS et al. A prospective study of short-course radiotherapy in poor prognosis glioblastoma multiforme. Int J Radiat Oncol Biol Phys. 1994 29(4):835–9.

25.

The correct answer is C. To evaluate different fractionations in poor prognosis glioma patients, Roa and colleagues randomized patients to standard fractionation (60 Gy/30 fx) versus hypofractionation (40 Gy/15 fx). No survival difference was found, with median survival approximately 5 months in both arms. Those in the standard fractionation arm were less likely to complete the entire planned course (74 % vs. 90 % in the hypofractionated arm) and were also more likely to require an increase in steroids post-treatment (49 % vs. 23 % in the hypofractionated arm). Roa W et al. Abbreviated course of radiation therapy in older patients with glioblastoma multiforme: a prospective randomized clinical trial. J Clin Oncol. 2004;22:1583–88.

26.

The correct answer is C. In this randomized study, 85 elderly patients (≥ 70 years) with a KPS of ≥ 70 were randomized to either best supportive care or 50.4 Gy in 1.8 Gy fractions. At the first planned analysis, the efficacy of radiotherapy exceeded the preset boundary, closing the study. At a median follow-up of 21 weeks, median survival with RT was 29.1 weeks compared to 16.9 weeks without radiotherapy. Quality of life did not differ between the two groups. Keime-Guibert F et al. Radiotherapy for glioblastoma in the elderly. N Engl J Med. 2007;356(15):1527–35.

27.

The correct answer is D. While CNS germ cell tumors, ependymomas, and choroid plexus tumors have a propensity for distant dissemination, meningiomas tend to be more locally aggressive in nature. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

28.

The correct answer is C. Due to its histology and chemosensitivity, maximal resection is generally NOT an important component of therapy for CNS germinoma. Merchant TE. Central nervous system tumors in children. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 1409–23.

29.

The correct answer is D. The incidence of meningiomas in females is double that of males. Prior exposure to ionizing radiation is a risk factor for the development of meningiomas, as is neurofibromatosis type 2. Individuals with NF2 are also at risk for development of schwannomas and ependymomas. There is no known difference in incidence of meningiomas according to race. Mack EE et al. Meningiomas induced by high-dose cranial irradiation. J Neurosurg. 1993;79:28. Louis DN et al. Meningiomas. In: Kleihues P, Cavenee WK, editors. Pathology and genetics: tumors of the nervous system. Lyon: IARC Press; 2000. p. 176.

30.

The correct answer is A. Psammoma bodies are frequently found in meningioma. Rosenthal fibers and gemistocytes are seen in astrocytomas. Pseudorosettes are a hallmark pathological feature of ependymomas. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

31.

The correct answer is C. Meningiomas most commonly grow along meningeal surfaces. They can grow along the base of skull and even invade into adjacent structures, such as the dural or superior sagittal sinuses. Invasion into brain parenchyma is less common and more likely to occur with higher-grade meningioma. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

32.

The correct answer is D. In this retrospective review, 140 patients with resected meningioma were treated with a median of 54 Gy radiotherapy. This study included a very mixed population of benign and malignant tumors: Twenty-­three patients had malignant meningioma. Five-year progression-free survival was 89 % in those that underwent gross total resection and 48 % in those that underwent subtotal resection. With adjuvant radiotherapy, subtotally resected meningioma 5-year progression-free survival approached that of gross totally resected tumors. Five-year progression-free survival of malignant meningioma after adjuvant radiation was 40–50 %. One of the most important points of the study is that outcomes are worse with total dose <52 Gy. Goldsmith BJ et al. Postoperative irradiation for subtotally resected meningiomas. A retrospective analysis of 140 patients treated from 1967 to 1990. J Neurosurg. 1994;80(2):195–201.

33.

The correct answer is B. Meningiomas tend to be hypointense or isointense on pre-contrast T1-weighted images and uniformly enhance with gadolinium contrast. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

34.

The correct answer is B. The majority (2/3) of low-grade oligogliomas tend to be hypointense on T1 pre-contrast sequences, non-enhancing after gadolinium administration on T1 sequences, but hyperintense on T2-weighted sequences. Brown PD, Shaw EG, van den Bendt MJ. Low-grade gliomas. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 443–59.

35.

The correct answer is D. The fact that meningiomas are typically well circumscribed and well demarcated on MRI lends them to treatment with stereotactic radiosurgery (SRS). SRS can be used to improve local control after subtotal resection of meningiomas. One rationale for the use of SRS in treatment of meningiomas is that the alpha/beta ratio of meningiomas is thought to be akin to that of late-reacting tissue and thus may be more sensitive to the larger dose per fraction given with SRS. Despite the steep dose gradients possible with SRS, treatment of a lesion with in a location with significant mass effect may be associated with a higher risk of posttreatment edema. Vernimmen FJ et al. Stereotactic proton beam therapy of skull base meningiomas. Int J Radiat Oncol Biol Phys. 2001;49:99. Vermeulen S et al. A comparison of single fraction radiosurgery tumor control and toxicity in the treatment of basal and nonbasal meningiomas. Stereotact Funct Neurosurg. 1999;72(Suppl 1):60.

36.

The correct answer is B. To reduce the risk of optic neuropathy, a dose limit of 8–10 Gy to the visual apparatus is suggested. Shrieve DC et al. Dose fractionation in stereotactic radiotherapy for parasellar meningiomas: radiobiological considerations of efficacy and optic nerve tolerance. J Neurosurg. 2004;S3:390.

37.

The correct answer is D. Rosenthal fibers are pathognomic for pilocytic astrocytomas. Oligodendrogliomas have a fried egg appearance on microscopy. Germinomas are a round, blue cell tumor. Brown PD, Shaw EG, van den Bendt MJ. Low-grade gliomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 443–59. Chan MD, Rogers CL, Anderson BM, Khuntia D. Benign brain tumors: meningiomas and vestibular schwannomas. In: Gunderson LL, Tepper JE, editors, Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 473–91.

38.

The correct answer is B. Medulloblastoma is associated with Homer-­Wright rosettes, not pseudorosettes. These are associated with ependymoma. Merchant TE. Central nervous system tumors in children. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 1409–23.

39.

The correct answer is B. Ependymomas are relatively rare glial tumors. While children tend to present at a median age of 5, adults present in the third and fourth decades. The vast majority (90 %) of ependymomas are infratentorial and intracranial in children. In adults, ependymomas present more commonly in the spine or supratentorially. Stieber VW, Siker ML. Spinal cord tumors. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 511–28.

40.

The correct answer is B. Anaplastic ependymomas have a much worse prognosis compared to well-differentiated myxopapillary, solid squamous, and cellular ependymomas. The myxopapillary subtype tends to be found in the cauda equina region. Solid squamous papillary ependymomas have a more favorable prognosis and are found in adults. Stieber VW, Siker ML. Spinal cord tumors. In Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 511–28.

41.

The correct answer is A. Gross total resection is more commonly achieved in supratentorial (50 %) rather than infratentorial tumors (30 %). Extent of resection is the most important predictor of outcome. As recurrences tend to be local, postoperative radiotherapy is typically tumor bed + margin to 59.4 Gy. Stieber VW, Siker ML. Spinal cord tumors. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 511–28.

42.

The correct answer is B. One-third of chordomas present in the clivus/craniocervical junction (base of skull). One-third present in the thoracic/lumbar spine, and the remaining third present in the sacrum. Stieber VW, Siker ML. Spinal cord tumors. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: Elsevier Saunders; 2012. p. 511–28.

43.

The correct answer is C. The inherent characteristic of protons that is most useful for the treatment of some chordomas is the Bragg Peak and subsequent steep dose gradient. Protons have been used to treat chordomas with excellent 5-year local control rates. Protons have an RBE of 1.1, while photons have an RBE of 1. Stieber VW, Siker ML. Spinal cord tumors. In: Gunderson LL, Tepper JE, editors. Clinical radiation oncology. 3rd ed. Philadelphia: