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78 Cards in this Set

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131. Ewing’s sarcoma predominantly occurs in:
(A) African American males and arises within the diaphysis of long bones.
(B) Caucasian males and arises within the diaphysis of long bones.
(C) African American males and arises within the metaphysis of long bones.
(D) Caucasian males and arises within the metaphysis of long bones.


Key: B
Rationale: Other characteristics for Ewing's sarcoma include a greater percentage of cases presenting in the axial skeleton, the t11;22 translocation. Osteosarcomas occur in greater frequency in African American males, at the growth plate of long bones.


193. The most common site of metastatic disease for retroperitoneal soft tissue sarcoma is in the:
(A) brain.
(B) liver.
(C) lung.
(D) mediastinum.

Key: B
Rationale: Soft tissue sarcomas of the extremities tend to metastasize to the lungs, whereas retroperitoneal sarcomas will metastasize to the liver.
References: Hansen, Eric K. et. al. Handbook of Evidence-Based Radiation Oncology. 2nd edition Springer Scientific. 2010. Page 616.


212. Which soft tissue sarcoma has the lowest incidence of lymph node metastases?
(A) Epithelial sarcoma
(B) Clear cell sarcoma
(C) Synovial cell sarcoma
(D) Malignant fibrous histiocytoma

Key: D
Rationale: Malignant fibrous histiocytoma ~10%; synovial sarcoma ~14%; clear cell sarcoma 28% and epithelial sarcoma 20%.
References: Hansen, Eric K. et al. Handbook of Evidence-Based Radiation Oncology. 2nd edition. Springer Scientific, 2010. p 616.


26. What is the most common genetic aberration in rhabdoid tumors of the kidney?
(A) EGFR amplification
(B) 1p/19q co-deletion
(C) Deletion in Chromosome 10p
(D) Deletion in Chromosome 22q

Key: D
Rationale: Both AT/RT and the analogous tumor outside the brain, malignant rhabdoid tumor, share a polyphenotypic immunoprofile and frequent 22q deletions with inactivation of the IN11/hSNF5 gene.


108. What common cytogenetic abnormality is found in patients with Ewing's sarcoma?
A. t(11;22)
B. LOH 16q
C. Codeletion 1p and 19q
D. N-myc amplification

Correct answer is A. RATIONALE: A fusion protein resulting from t(11;22) (q24;q11.2-12) fuses the Ewing's sarcoma (EWS) gene of chromosome 22 to the FLI1 gene of chromosome 11, which functions as a master regulator. Other translocations that are also found are t(21;22) and t(7;22). LOH 16q is found in Wilms tumor, codeletion 1p and 19q is found in oligodendroglioma, and N-myc amplification is associated with neuroblastoma.


4. Which of the following AJCC staging criteria for soft tissue sarcomas is correct?
A. The three-grade system is reclassified into a four-grade system.
B. Dermatofibrosarcoma protuberans is excluded from the list of histologic subtypes.
C. Desmoid tumors are included in the list of histologic subtypes.
D. Stage N1 disease is reclassified as stage III.

Correct answer is D. RATIONALE: The 7th edition of the AJCC Cancer Staging Manual has instituted the following changes for soft tissue sarcomas: The grading system has been reformatted from a four-grade system into a three-grade system. Angiosarcoma, extraskeletal Ewing’s sarcoma, and dermatofibrosarcoma protuberans have been added to the histological subtypes. Desmoid tumors, Kaposi’s sarcoma, and infantile fibrosarcoma are no longer included in the histological subtypes for this site. Gastrointestinal stromal tumors are now classified separately. Stage N1 disease has been reclassified from stage IV to stage III. REFERENCE: AJCC Cancer Staging Manual. 7th ed. 2009. p 345.


102. Which of the following statements is CORRECT concerning the management of retroperitoneal sarcomas?
(A) Post-operative irradiation is preferred to pre-operative irradiation.
(B) A pre-radiation differential renal scan is beneficial to confirm total renal function.
(C) Tumor extension into the peritoneal cavity will increase risk for local recurrence.
(D) Post-operative radiation field design will include residual tumor plus a planning volume.

Key: B
Rationale: Radiation for retroperitoneal sarcomas will commonly include at least one kidney that will be treated beyond renal tolerance. A differential renal scan will confirm activity of both kidneys and assure the treated kidney is not a dominant organ. Tumor extension into the peritoneal cavity does not increase the risk for local recurrence. It is extension into the soft tissues of the retroperitonium that creates the risk for local recurrence. Pre-operative irradiation is preferred because there is less risk of late effects when compared with post-operative irradiation. Post-operative field design should include the preoperative tumor volume with appropriate compartment delineation as well.
References: Chao, K.S. Clifford, et al. Radiation Oncology Management Decisions. 3rd edition. Lippincott, Williams and Wilkins. 2011. Pages 703-718.


3. Treatment for stage I extremity soft tissue sarcoma that is completely excised with negative margins is:
(A) observation.
(B) post-operative radiation.
(C) adjuvant imatinib and post-operative irradiation.
(D) adjuvant anthrocycline based chemotherapy and post-operative radiation.

Key: A
Rationale: Recommended therapy is observation.
References: Hansen, Eric K., et. al. Handbook of Evidence-Based Radiation Oncology. 2nd edition. Springer Scientific. 2010. Page 619.


325. Which of the following factors is considered a risk factor for the development of
osteosarcoma?
A. Prior trauma to the bone
B. Exposure to alkylating agents
C. Hyperparathyroidism
D. Osteopetrosis

Correct answer is B. RATIONALE: Osteosarcoma is the most common secondary malignancy in patients who received radiation therapy for a pediatric solid tumor. In addition, exposure to alkylating agents has also been implicated as a risk factor in a dose-dependent fashion. Paget's disease, a condition associated with accelerated bone turnover, is a risk factor, particularly in long-standing cases. Prior trauma to the bone has not been commonly associated with the development of osteosarcoma.


317. Surgery is preferred in the treatment of patients with Ewing’s sarcoma that involves the:
A. rib.
B. femur.
C. sacrum.
D. humerus.

Correct answer is A. RATIONALE: There have been no trials directly comparing surgery to radiation for local therapy, thereby causing the local treatment of choice to remain somewhat controversial. Generally, though, surgery is preferred for expendable bones, such as the rib or small bones of the hands and feet. Initially, concerns over dissemination of tumor throughout the bone marrow lead to larger radiation fields; but, with the use of multi-agent chemotherapy, this has fallen out of favor for "tailored fields" involving pre- and post-chemotherapy volumes.


310. Which of the following treatments is most appropriate for patients with chondrosarcoma?
A. Adjuvant chemotherapy for low-grade tumors
B. Wide local excision for intermediate- to high-grade tumors
C. Radiation therapy for both low- and intermediate-grade tumors
D. Radiation doses of 50 to 60 Gy for localized tumors

Correct answer is B. RATIONALE: Grade and histology are key in the decision-making
process for definitive management of chondrosarcomas. While intralesional curettage with
adjuvant therapy can be performed in low-grade tumors, wide local excision is recommended for
intermediate- to high-grade tumors. Low- and intermediate-grade tumors are thought to be
radioresistant due to a slower proliferation rate, but radiation can be used for high-grade tumors,
which are more radiosensitive. It is recommended that the radiation dose exceed 60 Gy, but
this can be hard to achieve due to dose constraints of adjacent organs. Chemotherapy is not
recommended after resection of low-grade chondrosarcomas.


304. Which of the following findings is associated with the best prognosis for patients with
Ewing's sarcoma?
A. Elevated alkaline phosphatase
B. Elevated creatinine phosphokinase (CPK)
C. Reduced polymorphonuclear leukocyte counts (PMN)
D. Presence of certain chromosomal translocations

Correct answer is D. RATIONALE: Systemic symptoms such as fever and elevated CPK are
considered poor prognostic factors. Patients with metastatic tumors in the axial skeleton tend to
have a poorer prognosis compared to those with metastatic disease in the extremities. At least
18 chromosomal translocations have been identified in the Ewing's sarcoma family (EWSF).
For example, the presence of the EWSF exon 7 fusion to FLI1 exon 6 occurs in approximately
60% of patients and is a favorable prognosticator.


298. Which of the following tumor sites in patients with Ewing’s sarcoma is associated with
the worst prognosis?
A. Rib
B. Shoulder girdle
C. Femur
D. Pelvis

Correct answer is D. RATIONALE: The duration of time from initial onset of symptoms to
diagnosis for Ewing's sarcoma is one of the longest of all pediatric malignancies. Although
overt metastases are present in approximately 25% of patients at diagnosis, subclinical
metastases are suspected in 80-90% of patients. Patients with tumors in the extremity
generally have a better prognosis than those in the axial skeleton, such as the pelvis.


176. A patient with a 6 cm rhabdomyosarcoma of the liver with no clinically involved regional lymph nodes and no evidence of metastases has what stage disease?
(A) 1
(B) 2
(C) 3
(D) 4

Key: A
Rationale: Based on the TNM pre-treatment staging classification.


291. Which of the following statements about cutaneous angiosarcoma is true?
A. The presenting lesion appears as a nodular mass.
B. Histological grade is considered in the staging system for angiosarcomas.
C. This tumor most commonly develops in women who have lymphedema after therapy for breast cancer.
D. The standard treatment consists of complete resection followed by irradiation.

Correct answer is D. RATIONALE: Cutaneous angiosarcoma is a rare malignancy of vascular
origin that usually arises in the scalp and face of elderly white males. Sixty percent of all
angiosarcomas arise in the skin and superficial soft tissues, and 50% of cutaneous
angiosarcomas are found in the head and neck area. The primary tumor typically presents as a
"spreading bruise" that varies from blue to red in color. A nodular component develops as the
tumor progresses. Typically, the primary tumor is more extensive than is apparent on physical
examination. Many patients present with multifocal disease. Optimal treatment consists of wide
local excision followed by radiation therapy to the primary site and to the regional lymph nodes.
There is no staging system for angiosarcomas. REFERENCE: Mendenhall W, Mendenhall CM,
Werning JW, et al. Cutaneous Angiosarcoma. American Journal of Clinical Oncology.
2006;29:524-528.


283. Which of the following statements about the AJCC staging system for soft tissue
sarcomas is true?
A. MRI findings are not used for T staging.
B. Kaposi’s sarcoma is staged according to AJCC criteria.
C. Regional lymph node involvement is classified as stage IV disease.
D. The site of primary disease is included.

Correct answer is C. RATIONALE: All clinical examination and radiological findings are used to
define the soft tissue sarcoma stage. The site of disease is an important prognostic factor that
is not incorporated into the current AJCC staging system. Kaposi’s sarcoma is not staged
according to the rules of soft tissue sarcomas. Regional lymph node involvement is assigned
stage IV status, regardless of grade, tumor size, and absence of diffuse metastatic disease.
REFERENCE: Kotilingam D, et al. Staging soft tissue sarcoma: evolution and change. CA: A
Cancer Journal for Clinicians. 2006;56:282-291.


277. Which of the following statements about radiation-induced soft tissue sarcomas is true?
A. Patients who have an Rb (retinoblastoma) gene mutation are at increased risk for
development of the condition.
B. Soft tissue sarcomas of the breast are most commonly associated with ipsilateral
irradiation from breast-conserving therapy.
C. Radiation-induced soft tissue sarcomas can develop outside the area in which highdose
therapy was administered.
D. Angiosarcoma is the most common histological subtype.

Correct answer is A. RATIONALE: The Rb (retinoblastoma) gene mutation plays a significant
role in the development of radiation-induced soft tissue sarcomas. For patients with genetic
retinoblastoma, the actuarial risk of developing a sarcoma in the radiation field within the next
18 years has been 6.6% (Trott). The most common radiation-induced malignancy after breast
irradiation as part of conservation management is breast cancer in the contralateral breast. A
cautious estimate of breast cancer risk suggests the lifetime risk for one breast exposed to 1 Gy
is approximately 5% if irradiated at an age younger than 35 years, <3% at the age of 35-45
years, and much less or zero at an older age. The most common histology of soft tissue
sarcoma associated with radiation is malignant fibrous histiocytoma. Radiation-induced soft
tissue sarcoma tends to occur within the region of high-dose therapy, with very rare appearance
in tissue receiving a dose of <10 Gy. REFERENCES: Halperin EC, Perez CA, Brady LW. The
discipline of radiation oncology. Halperin EC, Perez CA, Brady LW, eds. Principles and Practice
of Radiation Oncology. 5th edition. Chapter 1. Philadelphia: Lippincott Williams & Wilkins
Publishers. 2008;54-55. Trott K-S, Kamprad, F. Estimation of cancer risks from radiotherapy of
benign diseases. Strahlenther Oncol. 2006;8:431-436.


262. Which of the following types of therapy is most likely to improve overall survival in a
patient with osteosarcoma?
A. Surgery alone
B. Adjuvant chemotherapy
C. Neoadjuvant chemotherapy
D. Neoadjuvant irradiation

Correct answer is C. RATIONALE: The percent of necrosis after neoadjuvant chemotherapy in
nonmetastatic cases is associated with improved overall survival. Patients having tumor with
>90% necrosis have an overall survival near 70% compared with 50% in those with <90%
necrosis. Recommended treatment is neoadjuvant chemotherapy ----> surgery ------> adjuvant
chemotherapy for 4-6 months. Radiation therapy is indicated whenever surgical margins are
positive. Surgical resection of lung metastases improves overall survival. REFERENCES:
Larrier NA. Osteosarcoma. Halperin EC, Perez CA, Brady LW, eds. Principles and Practice of
Radiation Oncology. 5th edition. Chapter 80. Philadelphia: Lippincott Williams & Wilkins
Publishers. 2008;1801-1805. Hansen, EK and Roach M, eds. Handbook of Evidence-based
Radiation Oncology. Springer. 2007; 416-421.


259. Which of the following genetic aberrations is most commonly found in patients with
Ewing’s sarcoma?
A. t(2;13)
B. t(11;22)
C. 1p deletion
D. 11p13 deletion

Correct answer is B. RATIONALE: The genetic aberration t(11;22)(q24;q12) is found in 85% of
patients with the Ewing’s sarcoma family of tumors (ESFT) and is considered pathognomonic
for the disease. Genetic abnormalities noted on 11p13 are associated with Wilms tumor; t(2;13)
or t(1;13) is noted in alveolar rhabdomyosarcoma; 1p deletion or loss of heterozygosity (LOH) at
1p is noted in neuroblastoma.


219. Osteosarcoma most commonly develops in the:
A. mandible.
B. thoracic spine.
C. iliac wing of the pelvis.
D. diaphysis of the long bone.

Correct answer is D. RATIONALE: Ninety percent of all osteosarcomas present in the
diaphysis of the long bone (femur or humerus). The mandible is the most common site in the
head and neck region. Osteosarcoma occurs in the pelvis in less than 10% of cases, but the
pelvis is the most common non-extremity site. Osteosarcoma is seen in the spine in less than
2% of cases. REFERENCES: Larrier NA. Osteosarcoma. Halperin EC, Perez CA, Brady LW,
eds. Perez and Brady’s: Principles and Practice of Radiation Oncology. 5th edition. Chapter 80.
Philadelphia: Lippincott Williams & Wilkins Publishers. 2008;1801.


12. What is the expected 10-year overall survival rate for a 45-year-old man who had a
surgical resection with negative surgical margins for a grade 1 chondrosarcoma?
A. 90%
B. 80%
C. 70%
D. 50%

Correct answer is B. RATIONALE: Grade 1 chondrosarcomas are characterized as having a
very low metastatic potential. The average age of presentation by a patient is in the mid-40s,
and the expected 10-year overall survival rate for patients is 83%. REFERENCE: Evans HL,
Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of bone: a clinicopathologic
analysis with emphasis on histologic grading. Cancer. 1977;40:818.



449. Which one of the following is the MOST common sarcoma in children younger
than 10 years?
A. Ewing sarcoma
B. Osteosarcoma
C. Rhabdomyosarcoma
D. Synovial sarcoma

Answer: C
Answer 449 is C. Osteosarcoma, Ewing's sarcoma and synovial sarcoma are usually
seen in the older, adolescent age group.

450. Which one of the following pediatric sarcomas frequently arises in bone as well as
soft tissue?
A. Ewing sarcoma
B. Osteosarcoma
C. Rhabdomyosarcoma
D. Synovial sarcoma

Answer: A
Answer 450 is A. Although most Ewing's sarcomas occur in the bone, some occur in the
soft tissues (extraosseous Ewing's sarcoma)


454. For patients with nonmetastatic Ewing sarcoma, radiotherapy is MOST likely to be
used for local control for which one of the following sites?
A. Fibula
B. Lumbar spine
C. Calvarium
D. Clavicle

Answer 454 is B. Surgery is often preferred for local control of Ewing’s sarcoma, but
the morbidity of surgical resection in the spine is often excessive, with radiotherapy
playing a major role there.


481. Which one of the following subtypes of liposarcoma is most likely to fail in
nonpulmonary distant sites?
A. Well differentiated
B. Myxoid
C. Pleomorphic
D. Dedifferentiated

Answer 481 is B. The most common site of distant metastasis for almost all sarcomas is
pulmonary, with myxoid liposarcoma being an exception.


482. Which type of sarcoma is MOST commonly seen in the fingers, hands or forearm of
young adults?
A. Angiosarcoma
B. Epithelioid sarcoma
C. Liposarcoma
D. Neurofibrosarcoma

Answer: B
Answer 482 is B. Epithelioid sarcoma occurs predominantly in the distal extremities of
young adults.


483. A 29-year-old man has a 4-cm high-grade liposarcoma of the retroperitoneum. He
has no nodal involvement or distant metastasis. What is the AJCC stage?
A. I
B. II
C. III
D. IV

Answer 483 is B. The patient has a T1bN0M0 G3-4 lesion, which is Stage II.


484. Advantages of preoperative over postoperative radiotherapy for soft-tissue sarcomas
include all of the following, EXCEPT:
A. Smaller radiotherapy volume
B. Lower radiotherapy dose
C. Decreased intraoperative seeding of tumor cells
D. Lower risk of wound complications

Answer 484 is D. One of the disadvantages of preoperative radiotherapy is delayed
wound healing.

146. Which of the following statements about desmoid tumors is FALSE?
A. They rarely metastasize.
B. Definitive radiation therapy rarely can achieve local control.
C. Surgical excision is the most common treatment of choice.
D. They are locally infiltrative.

Answer 146 is B. Retrospective studies show good local control rates (approx. 75% or higher)
for definitive RT.


149. Askin tumors arise from what location?
A. Chest wall
B. Cervical spine
C. Long bones
D. Pelvis

Answer 149 is A. An Askin tumor is a Ewing’s sarcoma arising from the chest wall.

154. According to NCCN guidelines, radiation therapy is NOT recommended for which of the
following resectable soft tissue sarcomas of the extremity?
A. High-grade, large, deep lesions
B. High-grade, large, superficial lesions
C. Low-grade, large, deep lesions
D. Low-grade, small, superficial lesions resected with > 1 cm margins

Answer 154 is D. NCCN guidelines are available at www.nccn.org.

155. What tumor characteristic distinguishes stage I from stage II soft tissue sarcomas,
according to the AJCC staging system?
A. Low-grade versus high-grade tumor
B. Negative versus positive results for lymph node involvement
C. Tumor size <5 cm versus >5 cm
D. Superficial versus deep tumor location

Answer 155 is A. Size determines T1 versus T2 status. Location determines a (superficial)
versus b (deep) status. Nodal positivity is Stage IV.

156. What is the most common radiation-induced sarcoma?
A. Liposarcoma
B. Angiosarcoma
C. Chondrosarcoma
D. Osteosarcoma

Answer 156 is D. Over half are osteosarcomas. See J Surg Oncol 2000; 75:42-50.

382. Which of the following statements about radiation therapy for Ewing sarcoma is FALSE?
A. MRI of the primary site should be used to determine the local extent of the tumor.
B. The whole bone should be irradiated to 39.6 Gy, followed by a boost to 16.2 Gy.
C. Before initiation of chemotherapy, the bone and soft tissue tumor need to be included in the original field that receives 45 Gy.
D. All children receive multiagent chemotherapy during radiation therapy.

Answer 382 is B. A randomized clinical trial has demonstrated that the entire bone need not be
included in the irradiation field in a child with Ewing sarcoma.


4. Which of the following chemotherapy agents is most effective against soft tissue sarcoma?
A. Thiotepa
B. Cyclophosphamide
C. Dactinomycin
D. Doxorubicin

Correct answer is D. RATIONALE: Doxorubicin has been a mainstay of chemotherapy for advanced soft tissue sarcomas, with a 20-30% response rate. More recent studies also have demonstrated the efficacy of ifosfamide.

28. Which of the following soft tissue sarcomas is most likely to be found in a patient with neurofibromatosis, type 1?
A. Liposarcoma
B. Fibrosarcoma
C. Leiomyosarcoma
D. Malignant peripheral nerve sheath tumor

Correct answer is D. RATIONALE: Approximately 5% of patients with neurofibromatosis will develop malignant peripheral nerve sheath tumor. Reference: DeVita, et al. Sarcomas of the soft tissue and bone. Cancer Principles and Practice of Oncology. 5th edition.

43. Which of the following primary bone tumors is most common?
A. Ewing sarcoma
B. Malignant giant cell tumor
C. Chondrosarcoma
D. Osteosarcoma

Correct answer is D. RATIONALE: Osteosarcoma is the most common malignant primary bone tumor (excluding multiple myeloma).


79. Prior to the advent of chemotherapy, the overall survival rate for patients with osteosarcoma treated with surgery alone was:
A. 05%.
B. 20%.
C. 50%.
D. 70%.

Correct answer is B. RATIONALE: Surgical ablation including amputation alone resulted in long-term survival in approximately 20% of patients.


135. Which of the following statements about postoperative brachytherapy for fibrosarcoma of an extremity is true?
A. The entire muscle compartment including the tumor bed should be irradiated.
B. Catheters are loaded with radioactive sources approximately 5 days after surgery.
C. Brachytherapy gives better local control than external-beam radiation therapy.
D. Brachytherapy is effective for both low-grade and high-grade sarcomas.

Correct answer is B. RATIONALE: Catheters are loaded 5 days after surgery to prevent wound-healing complications.

166. Which of the following statements about osteosarcoma is true?
A. It rarely occurs in African Americans.
B. The amount of tumor necrosis at resection is an important prognostic factor.
C. The addition of ifosfamide and etoposide to standard VACA-based chemotherapy improves survival or patients with nonmetastatic disease.
D. Radiation therapy is the preferred treatment for local control.

Correct answer is B. RATIONALE: The amount of tumor necrosis at resection is a prognostic indicator and surgery is the local treatment of choice. Options A and C occur in Ewing sarcoma.

196. Soft tissue sarcoma most commonly arises from what site?
A. Head and neck
B. Retroperitoneum
C. Lower extremity
D. Upper extremity

Correct answer is C. RATIONALE: By far, the most common location is the lower extremity, which is more prevalent than the upper extremity. Head and neck rhabdomyosarcoma accounts for ≤10% of cases in most series.

239. Which of the following primary sites is most commonly involved in patients who are diagnosed with Ewing sarcoma?
A. Pelvis
B. Humerus
C. Fibula
D. Tibia

Correct answer is A. RATIONALE: Based on the CCG 7881/POG 8850 trial, the primary site of involvement was the pelvis in 24% of patients with Ewing sarcoma. Involvement of the humerus occurred in 7% of patients, the tibia in 10% of patients, and the fibula in a lower percentage of patients than those with involvement of the humerus.

243. Which of the following statements about desmoid tumors is true?
A. They commonly metastasize to the lungs.
B. They commonly metastasize to the bones.
C. Surgical excision is the most common treatment of choice.
D. Definitive radiation therapy can achieve local control in 25% of cases.

Correct answer is C. RATIONALE: Retrospective studies show good local control rates (approx. 75% or higher) for definitive radiation therapy. These tumors rarely metastasize. Surgery is the initial treatment of choice.

259. Which of the following radiation doses is standard definitive radiation therapy for an unresectable Ewing sarcoma?
A. 45.0 Gy
B. 55.8 Gy
C. 64.8 Gy
D. 70.2 Gy

Correct answer is B. RATIONALE: The standard radiation dose is 55.8 Gy. Doses >60 Gy have been associated with high rates of secondary malignancy.

262. Which of the following statements about mesenchymal chondrosarcoma is true?
A. It occurs most commonly in the flat bones.
B. It occurs most commonly in elderly patients.
C. It has a better prognosis than most other forms of chondrosarcoma.
D. It has a monophasic histologic pattern.

Correct answer is A. RATIONALE: Mesenchymal chondrosarcomas are more aggressive and have a worse prognosis than the more common subtypes. They most commonly occur in the spine or non-pelvic flat bones.

277. Which of the following genetic abnormalities is most commonly associated with Ewing sarcoma?
A. t(8;14)
B. t(11;22)
C. 1p/19q gene deletion
D. Loss of heterozygosity (LOH) of the retinoblastoma gene

Correct answer is B. RATIONALE: t(11;22) is the classic translocation in Ewing sarcoma. The translocation t(8;14) is most commonly seen in Burkitt lymphoma; LOH-RB in retinoblastoma; and 1p/19q gene deletion in oligodendrogliomas.

297. What is the most common site of metastatic disease in patients with Ewing sarcoma?
A. Lung
B. Bone
C. Bone marrow
D. Lymph nodes

Correct answer is A. RATIONALE: The most common site of distant spread in patients with Ewing sarcoma is the lungs, followed closely by the bone.

360. What is the most common site of metastasis for patients with soft tissue sarcoma?
A. Regional lymph nodes
B. Bone
C. Lung
D. Liver

Correct answer is C. RATIONALE: Most metastases occur in the lung. Regional lymph node involvement occurs in <15% of cases.

84. Which of the following features is NOT associated with Ewing sarcoma?
A. It has t(11;22) translocation.
B. It has a distant metastatic rate of 5%.
C. The peak age of occurrence is in late adolescence.
D. Chemotherapy agents used typically include VAC-IE.

Correct answer is B. RATIONALE: Ewing sarcoma has a 20% to 25% distant metastatic rate.

98. A 55-year-old patient has a 10-cm painless mass on the posterior thigh. A radiograph of the thigh does not reveal abnormalities of the femur. Which of the following management steps is most appropriate to perform next?
A. Amputation, followed by CT scan of the chest
B. Excision biopsy of the mass, followed by a CT scan of the chest
C. MRI scan of the thigh, followed by core-needle or incision biopsy
D. MRI scan of the thigh, followed by simple excision of the mass

Correct answer is C. RATIONALE: Appropriate workup of a soft tissue mass is essential. MRI is generally the preferred imaging for all extremity masses and may yield a definitive diagnosis for entities such as lipomas and hemangiomas. All suspicious masses on MRI should be biopsied with either a core- or fine-needle biopsy or incision biopsy prior to resection, unless they are very small and a margin-negative excision could be done. Excision biopsies or simple excisions of masses later determined to be sarcomas are generally inadequate for definitive treatment since they may contaminate the tissues and negate the ability to consider the use of lower-dose, preoperative radiation therapy. The rate of residual disease following unplanned excision is high (45%) and may result in reduced ultimate local control. Reference: Frassica F, et al. Magnetic Resonance Imaging Clinics of North America. Nov. 2000;8(4):915-27. Zagars, et al. Cancer. 2003;97:2544-53.

133. Preoperative radiation therapy for patients with stage III soft tissue sarcoma of the leg decreases the risk for:
A. wound complications after surgery compared to that of patients treated with postoperative radiation therapy.
B. 5-year overall survival compared to that of patients treated with amputation alone.
C. distant metastases compared to that of patients treated with surgery alone or surgery and postoperative radiation therapy.
D. late effects of radiation therapy compared to patients treated with postoperative radiation therapy.

Correct answer is D. RATIONALE: Preoperative and postoperative radiation therapy for soft tissue sarcoma have been shown to have essentially equivalent outcomes with respect to local control and survival. The major benefit of preoperative therapy has been to allow a reduction of dose and decrease the risk of late effects of radiation treatment. Reference: Davis, et al. Radiotherapy and Oncology. 2005;75:48–53.

160. Which of the following primary bone tumors is most common?
A. Chondrosarcoma
B. Primary skeletal fibrosarcoma
C. Malignant giant cell tumor
D. Osteosarcoma

Correct answer is D. RATIONALE: Osteosarcoma is the most common primary bone tumor (excluding plasmacytoma/multiple myeloma).

192. Which of the following conditions is associated with an increased incidence of
aggressive fibromatosis?
A. Familial adenomatous polyposis
B. Neurofibromatosis type 1
C. Li-Fraumeni syndrome
D. Stewart-Treves syndrome

Correct answer is A. RATIONALE: Aggressive fibromatosis occurs more often in patients with familial adenomatous polyposis (FAP) with either one or a few tumors that mostly involve the abdominal wall or florid presentations of multiple tumors occurring early in life and usually involving the axial skeleton or extremities. The other conditions have been associated with increased risks of certain soft tissue sarcomas, but not desmoid tumors. Reference: Mendenhall. American Journal of Clinical Oncology. 2005;28:211–215.

205. Which of the following categories of soft tissue sarcoma should be treated with
brachytherapy alone after definitive resection?
A. Low-grade tumor, positive margins
B. High-grade tumor, positive margins
C. Low-grade tumor, negative margins
D. High-grade tumor, negative margins

Correct answer is D. RATIONALE: The risk of local recurrence following adjuvant
brachytherapy alone has been found to be higher for low-grade sarcomas and sarcomas of any grade with positive resection margins. The American Brachytherapy Society guidelines recommend brachytherapy alone only for intermediate- to high-grade lesions with negative or close margins. References: Pisters, et al. Journal of Clinical Oncology. 1996;14(3):859–868. Alekhteyar, et al. International Journal of Radiation Oncology, Biology, Physics. 1996;36(2):321–324. Nag, et al. International Journal of Radiation Oncology, Biology, Physics. 2001;Vol. 49, No. 4, pp. 1033–1043.

239. Which of the following tumors is called an Askin tumor when it arises from the rib?
A. Ewing sarcoma
B. Chondrosarcoma
C. Plasmacytoma
D. Osteosarcoma

Correct answer is A. RATIONALE: A Ewing sarcoma arising from the rib is known as an Askin tumor.

311. Which of the following dose-fractionation schemes is most appropriate for preoperative radiation therapy of soft tissue sarcoma?
A. 25.0 Gy in 5 fractions
B. 50.0 Gy in 25 fractions
C. 66.0 Gy in 33 fractions
D. 70.2 Gy in 39 fractions

Correct answer is B. RATIONALE: Based on the randomized trial, the dose-fractionation scheme used for preoperative radiation therapy for soft tissue sarcoma was 50.0 Gy in 25 fractions. Reference: O'Sullivan, et al. Lancet. 2002;359:2235–41.

326. Which of the following radiation dose ranges would be most appropriate for a patient who has undergone resection of a desmoid tumor in an extremity with microscopically positive margins?
A. 30 Gy to 36 Gy
B. 50 Gy to 56 Gy
C. 60 Gy to 66 Gy
D. 70 Gy to 74 Gy

Correct answer is B. RATIONALE: There is currently no evidence that doses above 50 Gy improve local control in the adjuvant therapy of desmoid tumors. Doses above 56 Gy have been associated with increased toxicity. Reference: Ballo, et al. International Journal of Radiation Oncology, Biology, Physics. 1998; Vol. 42, No. 5, pp. 1007-1014.

334. A patient who has had a complete resection of a recurrent desmoid tumor is referred for postoperative irradiation. What is the probability of local control after postoperative irradiation?
A. 10% to 20%
B. 30% to 40%
C. 50% to 60%
D. 70% to 80%

Correct answer is D. RATIONALE: Adjuvant radiation therapy is generally considered after resection of desmoid tumors with positive margins. Local control is estimated to be in the range of 75% to 80%. Radiation therapy can also be used alone or if gross residual disease is left following surgery. Reference: Ballo, et al. Journal of Clinical Oncology. 1999;17:158-67. Goy, et al. International Journal of Radiation Oncology, Biology, Physics. 1997;39:659-65.

346. A 55-year-old patient has a stage III soft tissue sarcoma of the posterior thigh. The
patient's risk for developing metastatic disease within the next 5 years is approximately
what percent?
A. 10%
B. 25%
C. 50%
D. 80%

Correct answer is C. RATIONALE: High grade and tumor size >5 cm are the most significant predictors of the risk of metastatic recurrence in patients with soft tissue sarcoma. Zagars, et al, reported a 5-year metastatic control rate of 53% in patients with both high-grade and large-size sarcomas. Reference: Cancer. 2003;97:2530–43.

5. Which of the following bones is most commonly involved in patients with osteosarcoma?
A. Humerus
B. Femur
C. Spine
D. Tibia

Correct answer is B. RATIONALE: The following bones are most commonly involved in osteosarcoma: Femur = 40%. Humerus = 15%. Tibia = 15%. Spine = <5%. REFERENCE: Halperin, et al. Pediatric Radiation Oncology.

9. Which of the following statements about staging and prognostic factors for primary malignant bone tumors is true?
A. MRI is best for evaluating metastatic disease in the thorax.
B. MRI is best for staging of intraosseous or extraosseous tumors.
C. A tumor >8 cm in greatest dimension is classified as stage T3.
D. A worse prognosis is associated with lung metastases than with bone or hepatic metastases.

Correct answer is B. RATIONALE: Option A: CT is the exam of choice for evaluating the thorax for metastatic disease. CT is preferred over MRI for flat bones (such as the pelvis, scapula, or posterior elements of the vertebrae) where characterization of a lesion by radiography may be incomplete or difficult because of an inadequately visualized lesion. The role of CT in these circumstances is to characterize the lesion and determine whether it is potentially malignant or not. CT images may suffice for local staging. Option B: MRI provides the most accurate depiction of intraosseous or extraosseous tumors. To improve imaging in locations such as the pelvis or vertebrae, special sequences could be done. Bone scan is the exam of choice to evaluate for multiple skeletal lesions. Option C: Stage T1 = tumor 8 cm or less); stage T2 = tumor greater than 8 cm; stage T3 =discontinuous tumors in the primary bone site. Option D: Bone and hepatic metastases have a worse prognosis than lung metastases. REFERENCE: Greene FL, Page DL, Fleming ID, Fritz AG, eds. AJCC Cancer Staging Manual, 6th edition. 2002; pp. 187-190.

93. According to the NCI Canada randomized trial, which of the following statements about the use of preoperative versus postoperative radiation therapy for patients with sarcoma of the extremities is true?
A. Field sizes were significantly larger.
B. It resulted in a higher incidence of postoperative wound complications.
C. It increased the incidence of late fibrosis.
D. It improved local control.

Correct answer is B. RATIONALE: The NCI Canada study was terminated after interim analysis showed a higher rate of wound complications in the preoperative radiation therapy (RT) arm. The preoperative arm also used smaller fields sizes. This is expected since the full operative bed/scars do not have to be included in the preoperative setting. The updated report of this trial demonstrated a trend towards less late fibrosis with preoperative RT compared to postoperative RT. No local control difference was seen between the two arms of the study. REFERENCES: O'Sullivan, et al. Lancet. 2002. Davis, et al. Radiotherapy & Oncology. 2005.

110. Which of the following therapeutic options would be best after resection with negative surgical margins for a desmoid tumor?
A. Radiation therapy to 50 to 55 Gy
B. Radiation therapy to 60 to 66 Gy
C. Systemic chemotherapy
D. Observation

Correct answer is D. RATIONALE: Desmoid tumors have a low recurrence rate following complete surgical resection.

161. Which of the following statements about chondrosarcoma is true?
A. Thirty percent of chondrosarcomas are high-grade lesions.
B. Primary lesions can develop from a preexisting chondroid lesion.
C. Primary chondrosarcomas are typically peripheral, arising from the surface of the bone.
D. A favorable prognosis is strongly correlated with histological grade and adequate surgical excision.

Correct answer is D. RATIONALE: Option A: 5% to 10% of chondrosarcomas are high-grade lesions. These lesions have a high potential to metastasize. Option B: Primary lesions are not associated with preexisting lesions. Secondary lesions develop from preexisting chondroid lesions, such as enchondroma or osteochondroma. Option C: Chondrosarcomas are classified as central when they arise from the medullary cavity and peripheral when they arise from the surface of bone. Primary chondrosarcomas are nearly always central; secondary chondrosarcomas can be central or peripheral. Option D: Prognosis is generally favorable and is strongly correlated histologic grade and adequacy of surgery. REFERENCE: Chow W.A. Update on chondrosarcomas. Current Opinion in Oncology. 2007;19:371-376.

164. Classic Kaposi sarcoma (occurring in an elderly male of Mediterranean ancestry) most commonly is observed first on the skin of the:
A. lower legs.
B. palms.
C. trunk.
D. face.

Correct answer is A. RATIONALE: The initial distribution of classic Kaposi sarcoma (KS) virtually always is on the lower legs (followed occasionally by the palms and rarely the mucosa of the oral cavity); whereas there is far less predictability for other types of KS.

207. Based on the Massachusetts General Hospital (DeLaney) trial, what is the 5-year local control rate after radiation therapy is administered to patients with soft tissue sarcoma and positive margins after wide excision?
A. 76%
B. 54%
C. 33%
D. 12%

Correct answer is A. RATIONALE: This large retrospective study showed an overall 5-year local control rate of 76% in patients with soft tissue sarcoma and positive margins treated with excision and irradiation. REFERENCE: DeLaney, et al. International Journal of Radiation Oncology, Biology, and Physics (IJROBP). 2007;67:1460.

255. Which of the following dose ranges of EBRT is most appropriate for an adult patient who has undergone resection with negative surgical margins for soft tissue sarcoma of the extremity?
A. 68 to 70 Gy
B. 60 to 66 Gy
C. 55 to 59 Gy
D. 45 to 50 Gy

Correct answer is B. RATIONALE: The most appropriate postoperative radiation dose is in the range of 60 to 66 Gy.

265. Which of the following doses of definitive radiation therapy is most appropriate for a patient with unresectable Ewing's sarcoma of the pelvis?
A. 70.0 Gy
B. 64.8 Gy
C. 55.8 Gy
D. 50.4 Gy

Correct answer is C. RATIONALE: The recommended radiation dose for gross disease in a patient with Ewing's sarcoma of the pelvis is 55.8 Gy.

272. The recommended planning volume around gross tumor for treatment of retroperitoneal sarcomas is a minimum of:
(A) 1.0 cm.
(B) 1.5 cm.
(C) 2.0 cm.
(D) 3.0 cm.

Key: D
Rationale: The recommended margin around gross tumor in the retroperitoneum is 3-5 cm.
References: Chao, K.S. Clifford, et al. Radiation Oncology Management Decisions. 3rd edition. Wippincott, Williams and Wilkins. 2011, page 708.

289. Which of the following management options would be most appropriate for low-grade chondrosarcoma?
A. Curettage for primary treatment of affected extremity sites
B. Curettage and cryotherapy for primary treatment of affected axial and pelvic sites
C. Postoperative neutron radiation therapy after complete surgical resection
D. Postoperative photon radiation therapy of >65 Gy

Correct answer is D. RATIONALE: Options A and B (see references 1 and 4 below): Tumors treated with curettage and cryotherapy have lower or comparable recurrence rates (6%) to en-block resection (25%) at extremity sites. En-block resection recurrences (17%) are lower for axial-pelvic sites when compared to curettage and cryotherapy (43%). Option C (see reference 2 below): Neutrons have been used for treatment of unresectable sarcomas. Option D (see reference 3 below): Optimal treatment of chondrosarcoma requires doses of >65 Gy.
REFERENCES:
(1) Chow, W.A. Update on chondrosarcomas. Current Opinion in Oncology. 2007;19:371-376.
(2) Gunderson & Tepper eds. Clinical Radiation Oncology. 2nd edition, 2007;379.
(3) Gunderson & Tepper eds. Clinical Radiation Oncology. 2nd edition, 2007;549- 552.
(4) Gelderblom H, Hogendoorn PCW, Dijkstra SD, et al. The clinical approach towards chondrosarcoma. The Oncologist. 2008;13:320-329.

332. Which of the following prognostic factors is most important in determining the risk for distant metastases and the overall survival rate for adults with soft tissue sarcoma?
A. Tumor size
B. Tumor grade
C. Histologic subtype
D. Performance status

Correct answer is B. RATIONALE: Tumor grade has the most important influence on outcome.

349. Which of the following histologic findings is most characteristic of Ewing's sarcoma tumor cells?
A. Vimentin staining is usually positive.
B. Cytokeratin staining is usually positive.
C. Cells are large with small hypochromatic nuclei.
D. Cells are in Homer-Wright rosette pattern.


Correct answer is A. RATIONALE: The tumor cells are small with hyperchromatic nuclei, typically in a pseudorosette pattern. The cells undergo frequent mitosis. Vimentin staining is uniformly positive, and cytokeratin staining is usually negative.

35. The MOST importantprognostic factor for soft tissue sarcoma is:


(A) age.


(B) grade.


(C) lymph node status.


(D) histologic subtype.

Key: B Rationale:Histologic grade andtumor size have been consistently found to be independent predictors ofprognosis.

49. Desmoid tumors arising in patientswith familial adenomatous polyposis predominate in which of the followinganatomic sites?


(A) Thorax


(B) Abdomen


(C) Extremities


(D) Head and neck

Key: B Rationale: In patients with FAP,intra-abdominal desmoids predominate and tend to be associated with surgicalsites and anastomoses following colectomy. In patients with sporadic desmoids,the most common sites are the extremities, the shoulder girdle and the buttockareas.

81. Which of the following isthe MOST appropriate treatment for intermediate and high grade chondrosarcomas?


(A) Surgical excision


(B) Definitive radiationtherapy


(C) Neoadjuvant chemotherapyfollowed by surgery


(D) Neoadjuvant chemoradiationfollowed by surgery

Key: A Rationale:Surgery is thetreatment of choice for intermediate and high grade tumors.

114. Which of the followingdoses is MOST appropriate for definitive local RT for an L1 Ewing sarcoma?


(A) 41.4 Gy in 23 fractions


(B) 45 Gy in 25 fractions


(C) 55.8 Gy in 31 fractions


(D) 61.2 Gy in 34 fractions

Key: B Rationale: 41.4 Gy is an insufficient dose. Dose is constrainedby risk of spinal cord myelitis. The current Children’s Oncology Group protocolAEWS 1031 uses 45 Gy to the pre-chemotherapy extent of disease plus margin. Aboost of 5.4 to the pre-operative bone extent/post-chemotherapy soft tissueextent of disease can be considered if feasible with spinal cord tolerance. References:Halperin, PediatricRadiation Oncology.

173. According toMirels’ criteria, which feature is the MOST critical in determining the needfor surgical intervention of a metastasis to a long bone?


(A) Functional pain
(B) Location in thedistal femur


(C) Tumor appearsprimarily blastic


(D) 25% of the corticalbone is destroyed

Key: A Rationale: Mirels criteria hasbeen used by orthopedic oncologist for several decades which predict forfracture following patients treated with radiotherapy alone. There is pointscoring system that was developed where 4 components are evaluated, each scoredfrom 1 to 3. The components considered for the highest score are location inthe femoral neck, patients with functional pain, tumors that are primarilylytic as opposed to blastic and the size of of the cortical involvement withthe highest measured as > 2/3 of the cortex destroyed.

191. Which histologicsubtype of sarcoma has the lowest risk of lymph node metastasis?


(A) Liposarcoma


(B) Angiosarcoma


(C) Clear cell sarcoma


(D) Epithelioid sarcoma

Key: A Rationale: Increased risk in clearcell sarcoma (28%), angiosarcoma (23%), epithelioid sarcoma (20%),rhabdomyosarcoma (15%), synovial sarcoma (14%). (Mnemonic: SCARE for synovial,clear cell, angio, rhabdo, epithel.)