26 Sarcomas

Brian A. Van Tine and Angela C. Hirbe

QUESTIONS

Each of the numbered items below is followed by lettered answers. Select the ONE lettered answer that is BEST in each case unless instructed otherwise.

Question 26.1 Which of the following inherited syndromes represent a predisposing factor for the development of soft tissue sarcoma?

A. Retinoblastoma

B. Li–Fraumeni syndrome

C. Neurofibromatosis type I

D. All of the above

Question 26.2 Which of the following are TRUE about radiation-induced sarcomas?

A. Cancer history usually includes breast cancer, lymphoma, and cervical cancer.

B. They usually occur 10 to 30 years after radiation exposure.

C. Osteogenic sarcoma, undifferentiated pleomorphic sarcoma (UPS), angiosarcoma, and lymphangiosarcoma are the usual histologic subtypes.

D. All of the Above.

Question 26.3 Which clonal cytogenetic abnormality is associated with the CORRECT sarcoma subtype?

A. Ewing sarcoma and t(11;22) (q24;q12)

B. Synovial sarcoma and t(12;16) (q13;p11)

C. Myxoid liposarcoma and t(X;18) (p11;q11)

D. Alveolar rhabdomyosarcoma and t(17;22) (q22;q13)

Question 26.4. A 20-year-old man presents with right knee pain. X-ray reveals a “sun-burst” appearance in the distal femur. Biopsy reveals high-grade osteosarcoma. No distant metastases are identified. Which of the following is the most appropriate treatment?

A. Limb-sparing resection

B. Limb-sparing resection and adjuvant chemotherapy

C. Definitive radiation

D. Neoadjuvant chemotherapy, limb-sparing resection, and adjuvant chemotherapy

Question 26.5 In addition to site, which of the following variables is used to estimate the risk of sarcoma-specific death for a given patient?

A. Tumor grade and histology

B. Tumor size and depth

C. Age

D. All of the above

Question 26.6 Which of the following is TRUE regarding the staging of soft tissue sarcomas?

A. Staging involves a four grade system.

B. Stage IV includes N1 disease.

C. High-grade tumors can be classified as stage I depending on the size of the tumor.

D. Histologic grade, size, depth, and presence or absence of nodal and distant metastases are variables used to determine tumor stage.

Question 26.7 A 52-year-old man underwent resection of a 3-cm mass from the lateral left thigh. Pathology revealed a high-grade leiomyosarcoma, and the lateral surgical margin was positive. The most appropriate next step in the treatment of this patient’s cancer would be:

A. Radiation.

B. Adjuvant chemotherapy.

C. Reresection.

D. Observation.

Question 26.8 A 68-year-old woman presented with a purplish nodular lesion in the occipital scalp. Resection revealed an angiosarcoma measuring 3 cm. Surgical margins were negative. What is the most appropriate next step in the treatment of this patient’s cancer?

A. Radiologic imaging to look for nodal metastases and referral for adjuvant radiation

B. Monitoring

C. Adjuvant chemotherapy with an anthracycline

D. Adjuvant chemotherapy with paclitaxel

Question 26.9 A 65-year-old woman presented with abdominal pain and iron-deficiency anemia. Workup revealed a gastric mass and multiple large intra-abdominal masses and liver hypodensities. Biopsy of the gastric mass revealed a spindle cell neoplasm thought to be a leiomyosarcoma. After three cycles of doxorubicin and ifosfamide, imaging showed disease progression. The appropriate next step in the management of this patient’s cancer would be:

A. Docetaxel and gemcitabine.

B. Dacarbazine.

C. Request the pathologist to perform a CD117 (c-Kit) stain.

D. Palliative radiation.

Question 26.10 A 55-year-old man presented with a 10-cm mass in the medial left thigh. Biopsy revealed a high-grade liposarcoma. Imaging revealed no evidence of distant metastases. The most appropriate treatment of this patient’s cancer would be:

A. Definitive radiation.

B. Definitive radiation and concurrent doxorubicin.

C. Limb-sparing resection followed by adjuvant radiation.

D. Preoperative chemotherapy followed by resection.

Question 26.11 A 52-year-old man with metastatic unresectable gastrointestinal stromal tumor (GIST) was treated with imatinib (400 mg/d). Imaging showed initial disease response; however, the disease progressed after 28 months on therapy. The most appropriate treatment would be:

A. Erlotinib.

B. Sunitinib.

C. Doxorubicin.

D. Imatinib 800 mg/d.

Question 26.12 Which of the following factors are independent predictors of poorer disease-specific survival in patients with nonmetastatic soft tissue sarcoma?

A. Large tumor size (>10 cm)

B. High-grade histology

C. Older age (>60 years)

D. All of the above

Question 26.13 A 48-year-old woman underwent complete resection of a 9-cm high-grade leiomyosarcoma arising in the lower extremity. Postoperative adjuvant radiation was administered. Two years later, a chest computed tomography (CT) scan revealed a new single 3-cm, round, noncalcified pulmonary nodule. What is the most appropriate next treatment?

A. Complete resection of the lung nodule

B. Radiation

C. Ifosfamide and doxorubicin

D. Docetaxel and gemcitabine

Question 26.14 Which of the following is TRUE about patients with metastatic or locally recurrent soft tissue sarcoma?

A. Median survival is 12 months, although 20% to 25% of patients are alive 2 years after diagnosis.

B. Complete resection of oligometastases to the lung never results in long-term survival.

C. Radiation is the preferred treatment of a locally recurrent sarcoma.

D. Combination chemotherapy improves overall survival compared with single-agent chemotherapy.

Question 26.15 Which of the following is TRUE about chemotherapy treatment of metastatic soft tissue sarcoma?

A. Escalating doses of doxorubicin or ifosfamide do not improve tumor response rates over standard doses of these agents.

B. Leiomyosarcoma is uniquely sensitive to ifosfamide, whereas synovial sarcoma is not.

C. Paclitaxel shows broad-spectrum activity.

D. Dacarbazine has modest activity.

Question 26.16 A 30-year-old man presents with a permeative bone tumor in the distal femur. Open biopsy reveals a MFH. Radiologic imaging does not find distant metastases. What is the most appropriate treatment?

A. Limb-sparing resection with wide margins

B. Definitive radiation

C. Chemotherapy

D. Preoperative chemotherapy, limb-sparing resection, and adjuvant chemotherapy

Question 26.17 Which of the following is TRUE regarding osteosarcoma?

A. Approximately 20% of patients with localized high-grade disease treated with resection and chemotherapy remain disease free 5 years later.

B. Approximately 60% to 80% of patients with localized high-grade disease treated with resection and chemotherapy remain disease free 5 years later.

C. Parosteal (low-grade cortical) osteosarcoma is best treated with resection and chemotherapy.

D. Periosteal osteosarcoma is best treated with resection.

Question 26.18 A 45-year-old man presents with a left-sided pelvic pain. CT reveals a 5 cm mass with appearance of chondroid matrix, arising from the left side of the pelvic girdle. Bone biopsy reveals chondrosarcoma. Which of the following is TRUE regarding chondrosarcomas?

A. Most are low-grade tumors.

B. Most are treated with resection and radiation therapy.

C. Children have a better prognosis than adults.

D. Adjuvant chemotherapy has no role in the management of nonmetastatic disease.

Question 26.19 A 22-year-old woman presents with a giant cell tumor (GCT) of the distal femur. Appropriate treatment would be:

A. Curettage and debridement.

B. Amputation.

C. Radiation.

D. Preoperative chemotherapy, resection, and adjuvant chemotherapy.

Question 26.20 A 16-year-old female patient presents with a painful rapidly growing scapular mass. Core needle biopsy reveals a Ewing sarcoma. Staging evaluation shows no evidence of metastatic disease. The most appropriate therapy is:

A. Resection.

B. Preoperative chemotherapy (with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide, IE), resection, and adjuvant chemotherapy.

C. Resection and radiation.

D. Preoperative chemotherapy (with vincristine, doxorubicin, and cyclophosphamide), resection, and adjuvant chemotherapy.

Question 26.21 A 25-year-old woman presents with a painless right thigh mass, which has been slowly growing over a span of 8 years. Core needle biopsy reveals alveolar soft part sarcoma. What cytogenetic abnormality would you expect to see in this tumor?

A. t(11;22) (q24;q12)

B. t(12;16) (q13;p11)

C. der (17) t(X;17) (p11;q25)

D. t(X;18) (p11;q11)

Question 26.22 Which of the following targeted agents is used to treat alveolar soft part tissue sarcoma?

A. Imatinib

B. Sunitinib

C. Dasatinib

D. Nilotinib

Question 26.23 Which of the following sites of soft tissue sarcomas carries the best prognosis?

A. Head and neck

B. Extremity

C. Visceral

D. Retroperitoneal

Question 26.24 A 60-year-old woman with advanced soft tissue sarcoma is on third-line therapy with pazopanib and develops jaundice. Which of the following should be done next?

A. Abdominal imaging

B. Laboratory testing

C. Dose reduction

D. Immediately discontinue pazopanib

Question 26.25 Which of the following criteria are predictive of the behavior of GIST tumors and are useful for stratification of risk of recurrence and metastasis? (Select two correct responses)

A. Mitotic rate

B. Nuclear atypia

C. Tumor site

D. Mucosal invasion

Question 26.26 What is the mechanism of action of mesna?

A. Binds and detoxifies ifosfamide metabolites in the kidney and bladder

B. Coats the bladder

C. Inhibition of monoamine oxidase (reducing further chloroacetaldehyde formation)

D. Rescuing impaired hepatic metabolic pathways by acting as an electron acceptor

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