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97 Cards in this Set
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41. What is the current standard dose for a gross tumor in Ewing's sarcoma not within the spinal column? |
Key: C Rationale: This is the recommended dose for gross Ewings sarcoma. One exception is the spine, which is classically 45 Gy. Though, modern treatment planning can allow for higher doses with sparing of the spinal cord. |
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124. Which of the following conditions is NOT associated with Wilms tumor? (A) WAGR syndrome (B) Denys-Drash syndrome (C) Beckwith-Wiedemann syndrome (D) von Hippel-Lindau syndrome |
Key: D Rationale: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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174. What is an appropriate radiation dose for a 15-year-old boy with stage IIB Hodgkin lymphoma of the neck and mediastinum without bulky disease is to be treated with involved-field radiation therapy after a complete response to four cycles of chemotherapy? (A) 15 Gy (B) 21 Gy (C) 36 Gy (D) 45 Gy |
Key: B Rationale: Based on COG AHOD 0031. |
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183. Appropriate radiotherapy management of supra-tentorial primitive neuroectodermal tumor (PNET) in a 6-year-old patient after complete resection would include: (A) 18 Gy to the craniospinal axis followed by a 36 Gy boost to the tumor bed plus margin. (B) 23.4 Gy to the craniospinal axis followed by a 30.6 Gy boost to the tumor bed plus margin. (C) 36 Gy to the craniospinal axis followed by an 18 Gy boost to the tumor bed plus margin. (D) 54 Gy to the tumor bed plus margin. |
Key: C Rationale: All PNETs of the central nervous system require craniospinal irradiaiton (CSI) due to their propensity for drop metastases and include: medulloblastoma, supratentorial PNET, atypical teratoid/rhabdoid tumor, and pineoblastoma. The recommended dose is 36 Gy. The one exception is children >3 years old with standard risk medulloblastoma, in which the dose is lowered to 23.4 Gy and concurrent vincrisitne is administered. Dose reduction for CSI in patients under 3 years-old is currently under investigation. |
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31. What is the MOST appropriate dose in Gy for PCI in a 10-year-old boy with high risk acute lymphoblastic leukemia (ALL) and no evidence of central nervous system involvement? (A) 5.2 (B) 18 (C) 23.4 (D) 30.6 |
Key: B Rationale: For prophylactic treatment, 18 Gy is sufficient. Recent protocols use even lower doses of 12 Gy. |
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14. Which of the following is the MOST appropriate treatment after near total resection (>90%) of a large left temporal lobe ependymoma in a 30-year-old male if there is no evidence of drop metastasis on brain and spinal MRI? (A) Observation (B) Involved field radiotherapy alone (C) Combined chemotherapy and involved field radiotherapy (D) Craniospinal irradiation followed by involved field radiotherapy |
Key: B References: Merchant TE. Current management of childhood ependymoma. Oncology (Williston Park). 2002 May; 16(5):629-42, 644; discussion 645-6, 648. Review. Paulino AC. Radiotherapeutic management of intracranial ependymoma. Pediatr Hematol Oncol. 2002 Jul-Aug; 19(5):295-308. Paulino AC et al. Intracranial ependymomas: an analysis of prognostic factors and patterns of failure. Am J Clin Oncol. 2002 Apr; 25(2):117-22. |
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150. For a 12-year-old boy with a paratesticular embryonal rhabdomyosarcoma, work-up should include all of the following EXCEPT: (A) a brain MRI. (B) a bone scan. (C) a bilateral bone marrow aspiration/biopsy. (D) an ipsilateral retroperitoneal lymph node dissection. |
Key: A Rationale: The standard work up for a child over 10 years of age with a paratesticular embryonal rhabdomyosarcoma is delineated in the Children's Oncology Group Protocols, including ARST0531, and include ipsilateral retroperitoneal lymph node dissection, bilateral bone marrow aspiration/biopsy, and bone scan, but not brain MRI. |
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452. A 3-month-old child is diagnosed with Reese-Elsworth stage 3 retinoblastoma of the right eye. Family history is significant for 2 uncles with retinoblastoma. Which one of the following is CORRECT? A. The child has a greater than 50% chance of developing retinoblastoma in the left eye B. The child is likely male C. Enucleation should result in a normal life expectancy D. Cure rate is approximately 50% |
Answer 452 is A. Hereditary retinoblastoma has a high incidence of bilaterality. Children like the one in the test scenario have a high incidence of second malignancies, which can limit their life expectancy. Cure from the retinoblastoma should be about 90%. |
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453. A 5-year-old girl is diagnosed with a Wilms tumor of the left kidney without evidence of local spread. Staging evaluation shows a .5-cm metastasis to a single site. Which one of the following single metastatic sites would be associated with the BEST prognosis? A. Bone marrow B. Brain C. Femur D. Lung |
Answer 453 is D. Small lung metastases in Wilms’ tumor are usually associated with favorable histology and carry a favorable prognosis for cure. Metastases to other organs are more often associated with unfavorable histology, which carries a more guarded prognosis. |
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8. For a 12-year-old boy with Hodgkin lymphoma and stage IIA with bulk disease, the work-up and initial studies should include all of the following EXCEPT: (A) bone scan. (B) pulmonary function tests. (C) bilateral bone marrow biopsies. (D) CT of the neck, chest, abdomen, and pelvis. |
Key: A Rationale: Based on the work-up for COG AHOD 0031. |
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91. Which of the following are indications for whole-abdominal radiation for a favorable histology Wilms tumor? (A) Malignant ascites (B) Involvement of the renal hilum (C) Involvement of regional lymph nodes (D) Microscopic positive surgical margins |
Key: A Rationale: The "SPAR" pneumonic for indications for whole-abdominal radiation include: Spillage during surgery, Peritoneal seeding, Ascites and preoperative Rupture. |
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127. Which of the following medulloblastoma subtypes confers the most favorable outcome in non-infant patients with this disease? (A) WNT group, CTNNB1 mutation (B) SHH group, PTCH1/SMO/SUFU mutation, GLI2 amplification, or MYCN amplification (C) Group 3, MYC amplification, photoreceptor/GABAergic gene expression (D) Group 4, gene expression of neuronal/glutamatergic, CDK6 amplification, MYCN amplification |
Key: A Rationale: Transcriptional profiling show the existence of four main subgroups (Wnt, Shh, Group 3, and Group 4). • Very good prognosis: WNT group, CTNNB1 mutation • Infants good prognosis, others intermediate: SHH group, PTCH1/SMO/SUFU mutation, GLI2 amplification, or MYCN amplification • Poor prognosis: Group 3, MYC amplification, photoreceptor/ GABAergic gene expression • Intermediate prognois: Group 4, gene expression of neuronal/ glutamatergic, CDK6 amplification, MYCN amplification. References: Acta Neuropathol. 2012 April; 123(4):473-484. |
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379. An asymptomatic 5-year-old child with neurofibromatosis type 1 (NF1) has a lowgrade glioma of the optic chiasm. Recommended management should consist of which of the following? A. Biopsy, followed by chemotherapy and low-dose irradiation to 40 Gy B. Intensity-modulated radiation therapy (IMRT) to 50.4 Gy C. Gross total resection, followed by six cycles of chemotherapy with vincristine and carboplatin D. Close monitoring with imaging, visual, and neurologic testing |
Answer 379 is D. A young, asymptomatic NF1 child with an optic chiasm glioma can be managed by serial imaging and neurologic testing without surgery. If there is clinical evidence of tumor progression, chemotherapy with or without irradiation may be employed. |
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149. Which of the following statements about trilateral retinoblastoma is FALSE? (A) It is associated with an intracranial neuroblastic tumor. (B) It has a favorable prognosis with approximately 80% overall survival. (C) It has a higher incidence in patients with germline Rb mutations. (D) It requires heightened surveillance with MRI until the age of 5 years. |
Key: B Rationale: Paulino AC. Trilateral retinoblastoma: is the location of the intracranial tumor important? Cancer 1999; 86(1):135-41. Kivelä T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. Journal of Clinical Oncology 1999; 17(6):1829-37. |
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386. Which of the following findings does NOT require irradiation of the flank or abdomen after nephrectomy? A. Stage II diffuse anaplastic tumor B. Stage II clear cell sarcoma of the kidney C. Stage II Wilms tumor with favorable histology D. Wilms tumor with diffuse peritoneal tumor spillage and favorable histology |
Answer 386 is C. Children with Stage II favorable histology Wilms tumor do not require flank or abdominal irradiation. All the other situations require flank (A,B) or whole abdominal irradiation (D). |
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392. What is the most important prognostic factor for patients with Wilms tumor? A. Age at diagnosis B. Tumor size C. Lymph node involvement D. Histology |
Answer 392 is D. Through the NWTS studies, the finding of diffuse anaplasia is the single most predictive factor of outcome. Although lymph node involvement remains a predictor of failure it is not as significant as histology. |
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398. A 7-year-old patient presents with a 6-cm alveolar rhabdomyosarcoma of an extremity and undergoes biopsy. The metastatic work-up is otherwise negative. How should this patient’s disease be classified? A. Stage 2, clinical group II B. Stage 2, clinical group III C. Stage 3, clinical group II D. Stage 3, clinical group III |
Answer 398 is D. Extremity site, > 5 cm size (T2b Nx M0; clinical group III). |
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13. What is the most common secondary malignant neoplasm inside the radiation field for patients with retinoblastoma? A. Squamous cell carcinoma B. Non-Hodgkin lymphoma C. Fibrosarcoma D. Osteosarcoma |
Correct answer is D. RATIONALE: It is important to know that common secondary malignant neoplasms inside the radiation field for patients with retinoblastoma are osteosarcoma, fibrosarcoma, or spindle cell sarcoma, with osteosarcoma being the most common. |
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25. According to the IRS-I and IRS-II data analyses, which of the following primary sites of rhabdomyosarcoma is associated with the highest risk of lymph node metastasis? A. Orbit B. Prostate C. Lower extremity D. Parameningeal |
THIS ITEM WAS NOT SCORED. RATIONALE: Analysis of IRS-I and IRS-II data revealed that among all of the sites listed, the prostate had a 42% risk of lymph node metastasis compared to other sites, such as the orbit (0%), the non-orbit head and neck sites (8%), and the lower extremity (9%). Lymph node metastasis has prognostic significance. |
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39. A 9-month-old infant has a mass in the left flank, periorbital ecchymosis, and bone metastasis to the right femur. Biopsy results are consistent with neuroblastoma. What cancer stage would be most appropriate for this patient's condition? A. Stage 2 B. Stage 3 C. Stage 4 D. Stage 4S |
Correct answer is C. RATIONALE: If there were no presence of bone metastases, this infant’s disease would be classified as cancer stage 4S. However, bone involvement (not bone marrow) upstages the infant to cancer stage 4, and treatment is entirely different. |
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52. What percent of patients with neuroblastoma have metastases at diagnosis? A. 10% B. 25% C. 60% D. 80% |
Correct answer is C. RATIONALE: Overall, 60% of patients with neuroblastoma have metastases at diagnosis. The knowledge of this percentage is important because it justifies the need for routine metastatic workup of patients with the disease. |
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64. Which of the following types of abdominal tumor most commonly occurs in a 2-year-old child? A. Neuroblastoma B. Rhabdomyosarcoma C. Non-Hodgkin lymphoma D. Wilms tumor |
THIS ITEM WAS PARTIALLY SCORED (BOTH OPTIONS A & D SCORED CORRECT). RATIONALE: Wilms tumor is the most common abdominal tumor that occurs during childhood, followed closely by neuroblastoma. The median age at diagnosis of neuroblastoma is about 2 years of age, and the median age at diagnosis of Wilms tumor is 3.5 years of age. |
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76. Which of the following histologic subtypes of rhabdomyosarcoma has the worst prognosis? A. Alveolar B. Embryonal C. Spindle cell D. Botryoid |
Correct answer is A. RATIONALE: Data from the literature showed that histologic subtype bears prognostic significance for rhabdomyosarcoma. Of these, alveolar histology has the worst survival. |
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90. What percent of neuroblastomas exhibit N-myc amplification? A. 05% B. 25% C. 50% D. 75% |
Correct answer is B. RATIONALE: Overall, 25% of neuroblastomas exhibit N-myc amplification. |
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104. Which of the following body sites is most commonly involved in patients with rhabdomyosarcoma? A. Thorax B. Extremities C. Head and neck D. Genitourinary tract |
Correct answer is C. RATIONALE: This item is based on epidemiological data. Approximately 40% of rhabdomyosarcomas occur in the head and neck region. |
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116. What is the most common group classification of rhabdomyosarcoma at initial diagnosis? A. Group I B. Group II C. Group III D. Group IV |
Correct answer is C. RATIONALE: This item is based on epidemiological data. The most common presentation is Group III disease. Reference: Donaldson, SS, and Anderson, J. Factors that influence treatment decisions in childhood rhabdomyosarcoma. Intergroup Rhabdomyosarcoma Study Group of the Children's Cancer Group, the Pediatric Oncology Group, and the Intergroup Rhabdomyosarcoma Study Group Statistical Center. Radiology. 1997;203:17-22. |
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317. A 3-year-old girl has Wilms tumor with a favorable histology and a positive surgical margin. CT scan of the chest does not reveal any pulmonary nodules. What cancer stage is most appropriate for the patient's condition? A. Stage I B. Stage II C. Stage III D. Stage IV |
Correct answer is C. RATIONALE: Cancer stage III is associated with this patient’s condition because of the positive surgical margin. |
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338. Which of the following radiation dose ranges administered to the cranium would be most appropriate for providing CNS prophylaxis to patients with acute lymphoblastic leukemia? A. 05 Gy to 10 Gy B. 12 Gy to 18 Gy C. 24 Gy to 30 Gy D. 36 Gy to 40 Gy |
Correct answer is B. RATIONALE: When CNS prophylaxis is provided in the management of acute lymphoblastic leukemia (ALL), a radiation dose of 12 Gy to 18 Gy is typically recommended. Intrathecal methotrexate is commonly used in adults. |
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22. Common presentations of neuroblastoma include all of the following EXCEPT: A. hematuria. B. a neck mass. C. a palpable abdominal mass. D. respiratory compromise. |
Correct answer is A. RATIONALE: Options B, C, and D are common presenting signs and symptoms of neuroblastoma. Reference: Matthay KK, Haas-Kogan DA, Constine LS. Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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34. The Cade technique was associated with which of the following tumor types? A. Osteosarcoma B. Neuroblastoma C. Rhabdomyosarcoma D. Skull-base Ewing sarcoma |
Correct answer is A. RATIONALE: The Cade technique, named for Sir Stanford Cade, was used prior to the advent of effective chemotherapy for osteosarcoma. It involved initial definitive radiation therapy to tumors of the extremity, followed by a waiting period of approximately 6 months. If no lung metastases developed in that time, radical surgery would be performed. This time interval allowed many patients who were destined to develop metastatic disease to avoid unnecessary amputation. It also is important in establishing the responsiveness of this tumor to radiation therapy. |
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41. Which of the following histologies is NOT associated with rhabdomyosarcoma? A. Desmoplastic B. Embryonal C. Alveolar D. Botryoid |
Correct answer is A. Reference: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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177. Which of the following statements about CNS-related atypical teratoid/rhabdoid tumors (AT/RT) is true? (A) It has the same prognosis as medulloblastoma. (B) The median age of diagnosis is less than 1 year. (C) Standard treatment involves postoperative involved-field radiation therapy alone. (D) Leptomeningeal spread rarely occurs in patients with AT/RT. |
Key: B |
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96. Which of the following statements about neuroblastoma is FALSE? A. Ten to fifteen percent of children have a family history of the disease. B. About 60% of children will have metastatic disease (either lymphatic or hematogenous) at the time of clinical presentation. C. Immunohistochemical stains will demonstrate neuron-specific enolase (NSE), synaptophysin, chromogranin A (CGA), and neuronal filaments. D. MYCN amplification is seldom identified in stages 1, 2, and 4S. |
Correct answer is A. RATIONALE: Although neuroblastoma usually occurs sporadically, 1% to 2% of patients have a family history of the disease. Reference: Matthay KK, Haas-Kogan DA, Constine LS, Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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101. Which of the following conditions is NOT associated with Wilms tumor? A. WAGR syndrome B. Denys-Drash syndrome C. Beckwith-Wiedemann syndrome D. von Hippel-Lindau syndrome |
Correct answer is D. Reference: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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105. Which of the following factors correctly matches the hereditary and sporadic characteristics associated with patients who have retinoblastoma? Hereditary Sporadic A. Age at occurrence Older Younger B. Risk of secondary malignancies Lower Higher C. Bilateral disease Less common More common D. Incidence Lower Higher |
Correct answer is D. Reference: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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139. The standard diagnostic evaluation of neuroblastoma includes all of the following EXCEPT: A. bone scan. B. MIBG scan. C. lumbar puncture. D. urinary catecholamines. |
Correct answer is C. Reference: Matthay KK, Haas-Kogan DA, Constine LS. Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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184. Which of the following statements about the epidemiology of neuroblastoma is true? A. The median age of diagnosis is 5 years. B. Sixty-five percent of tumors secrete VMA or HVA in the urine. C. Thirty-five percent of patients are diagnosed at less than 1 year of age. D. Amplification of MYCN occurs in 80% of patients with an advanced stage of disease. |
Correct answer is C. RATIONALE: The median age of diagnosis of neuroblastoma is 22 months. More than 90% of neuroblastomas secrete vanillylmandelic acid (VMA) or homovanillic acid (HVA) in the urine. Amplification of MYCN occurs in 30% to 40% of patients with advanced stage neuroblastoma. References: Young J.L., Jr., Ries L.G., Silverberg E., et al. Cancer incidence, survival, and mortality for children younger than age 15 years. Cancer. 1986;58(2 Suppl):pp. 598-602. Matthay KK, Haas-Kogan DA, Constine LS. Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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206. Which of the following statements about trilateral retinoblastoma is FALSE? A. It is associated with an intracranial neuroblastic tumor. B. It has a favorable prognosis with approximately 80% overall survival. C. It has a higher incidence in patients with germline Rb mutations. D. It requires heightened surveillance with MRI until the age of 5 years. |
Correct answer is B. References: Paulino AC. Trilateral retinoblastoma: is the location of the intracranial tumor important? Cancer. 1999;86(1):135-41. Kivelä T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. Journal of Clinical Oncology. 1999;17(6):1829-37. |
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212. Which of the following statements about Wilms tumor is true? A. Calcification is found in 40% of tumors. B. Patients most commonly present with hematuria. C. Anaplastic Wilms tumor is extremely rare in infants. D. The tumor cells have a small, round, blue appearance. |
Correct answer is C. Reference: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott Williams & Wilkins. |
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236. Which of the following statements about rhabdomyosarcoma is true? A. Most patients present with group II disease. B. Its peak incidence occurs at 7 to 9 years of age. C. The head and neck region is the most common site. D. More than 50% of patients have congenital anomalies. |
Correct answer is C. Reference: Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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250. Which of the following statements about children who were prenatally exposed to ionizing radiation is true? A. Approximately 5% will develop leukemia. B. They are at a significantly increased risk of developing breast and lung cancers. C. There will be an estimated 200 to 250 excess cancer deaths in the first 10 years of life/10,000 persons/Gy absorbed dose. D. Most of the excess cancer deaths are attributable to malignancies of the central nervous system. |
Correct answer is C. RATIONALE: For children exposed to ionizing radiation in utero, it has been estimated that of the 200 to 250 excess cancer deaths in the first 10 years of life per 10,000 persons per Gy absorbed dose, one half will consist of leukemias, and one fourth will consist of malignancies of the nervous system. Associations between in-utero exposure and breast and lung cancer have not been described. |
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50. A 5-year-old boy who has pure germinoma with normal blood and CSF β−hCG and α−fetoprotein levels has a complete response to subtotal resection and four cycles of systemic chemotherapy. Which of the following treatment recommendations would be most appropriate? A. Craniospinal radiation therapy with a boost dose to the primary site B. Local radiation therapy C. Whole-brain radiation therapy D. Stereotactic radiosurgery |
Correct answer is B. RATIONALE: Local radiation therapy is recommended for a 5-year-old patient who has had a good response to chemotherapy. The appropriate radiation treatment in patients with CNS germ cell tumors has evolved from craniospinal radiation therapy to local fields in the era of chemotherapy. Withholding radiation therapy is associated with an unacceptable risk of recurrence. |
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259. What is the most common site of bony involvement in Langerhans cell histiocytosis? A. Long bones B. Pelvis C. Skull D. Ribs |
Correct answer is C. Reference: Halperin, et al. Pediatric Radiation Oncology. 4th edition; page 492 table – summary of 3 large studies. |
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263. According to the Children's Cancer Group (CCG) studies, which of the following conditions is NOT associated with children treated for acute lymphocytic leukemia (ALL) at 10 years? A. Brain tumors B. Parotid gland tumors C. Acute myelogenous leukemia D. Hodgkin's lymphoma |
Correct answer is D. RATIONALE: Hodgkin's lymphoma was not found to be increased in the over 8,000 patients from the Children's Cancer Group study mentioned. Non-Hodgkin's lymphomas, however, were increased in addition to the other tumors listed. |
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317. Which of the following diagnoses is most likely indicated for a 15-year-old patient who has a pineal mass with an elevated serum -fetoprotein level and a normal -hCG level? A. Atypical teratoid rhabdoid tumor B. Primary pineal parenchymal tumor C. Endodermal sinus tumor (yolk sac tumor) D. Pure germinoma |
Correct answer is C. RATIONALE: An elevated -fetoprotein level is most suggestive of a non-germinomatous germ cell tumor with yolk sac tumor (otherwise known as endodermal sinus tumor) elements. Atypical teratoid rhabdoid tumors and pineal parenchymal tumors do not secrete known serum markers. Pure germinoma may secrete -hCG, but it does not produce -fetoprotein. |
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1. Common presentations of neuroblastoma include all of the following EXCEPT: (A) hematuria. (B) a neck mass. (C) a palpable abdominal mass. (D) respiratory compromise. |
Key: A Rationale: Choices B, C, and D are common presenting signs and symptoms of neuroblastoma. References: Matthay KK, Haas-Kogan DA, Constine LS. Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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332. Which of the following statements about childhood malignant renal tumors is true? A. Atypical rhabdoid tumors are associated with a better prognosis. B. Anaplastic Wilms tumor has the highest risk of brain metastases. C. Local spillage during surgery requires whole-abdominal irradiation for patients with Wilms tumor. D. Beckwith-Wiedemann syndrome is associated with the development of Wilms tumor. |
Correct answer is D. RATIONALE: Rhabdoid renal tumors are considered to be an unfavorable histology and have a worse prognosis than Wilms tumors. Rhabdoid tumors have the highest incidence of brain metastases. Local spillage requires flank irradiation; only diffuse spillage requires whole-abdominal irradiation. Beckwith-Wiedemann syndrome is associated with a high incidence of Wilms tumors. |
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97. Which of the following treatments should be performed for patients with bilateral Wilms tumor? A. Bilateral renal biopsy, followed by preoperative chemotherapy B. Bilateral renal biopsy, followed by immediate radiation therapy and chemotherapy C. Bilateral nephrectomy and consideration for kidney transplant D. Nephrectomy of the worse side, followed by chemotherapy and radiation therapy |
Correct answer is A. RATIONALE: The goal in patients with bilateral Wilms tumor is to obtain local control while sparing renal parenchyma. Therefore, upfront cytoreduction with chemotherapy is usually offered before a second-look surgery. The 10-year overall survival for patients with synchronous tumors is 70%, according to NWTS-2 and -3 and SIOP 1, 2, and 5 studies. Bilateral Wilms tumor is regarded as stage V disease. |
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193. Which of the following characteristics is associated with a diagnosis of retinoblastoma? A. Eighty percent of children are diagnosed in the first year of life. B. Approximately 80% of children with bilateral disease have a family history of the disease. C. Children who present with bilateral disease are usually 2 years older than those who present with unilateral involvement. D. It is rare in children older than 6 years of age. |
Correct answer is D. RATIONALE: Eighty percent of children with retinoblastoma are diagnosed before 3 years of age. There is a family history of the disease in approximately 20% of children diagnosed with bilateral retinoblastoma. Patients with bilateral disease usually present at a younger age (14–16 months) than patients with unilateral disease (29–30 months). The diagnosis of retinoblastoma in children 6 years or older is extremely rare. REFERENCE: Chintagumpala, et al. The Oncologist. October 2007;12(10):1237-1246. |
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214. Which of the following disease states is included in the intergroup rhabdomyosarcoma study (IRS) clinical group III classification? A. Localized tumor with resected positive lymph nodes with microscopic residual disease B. Localized or locally extensive disease with gross residual disease after a biopsy C. Localized disease with infiltration from the organ of origin to an adjacent structure with complete resection D. Distant metastatic disease present at diagnosis |
Correct answer is B. RATIONALE: The IRS clinical group staging system refers to the extent of disease, degree of resection, and the presence or absence of involved lymph nodes and/or metastasis; whereas the TNM staging classification relies on the primary anatomic site first, then on size of the primary disease, invasiveness of the primary disease, and presence or absence of involved lymph nodes and/or metastasis. |
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228. Which of the following chromosomal translocations may identify patients with alveolar rhabdomyosarcoma associated with a very poor prognosis? A. t(X;18) with SYT-SSX1 fusion transcript B. t(2;13) with PAX3-FKHR fusion transcript C. t(11;22) with EWS-FL1 fusion transcript D. t(9;22) with bcr-abl fusion transcript |
Correct answer is B. RATIONALE: The t(2;13) with the PAX3-FKHR fusion transcript identified a group of patients in the IRS-IV study population who had a 4-year overall survival rate of <10%. The PAX7-FKHR transcript identified a group of patients who had alveolar rhabdomyosarcoma (RMS) and a better prognosis (i.e., a 4-year overall survival rate of >70%). These translocations and fusion transcripts are not identified in embryonal RMS. Option A is seen in synovial sarcoma. Option C is seen in Ewing's sarcoma, and option D is seen in chronic myelogenous leukemia (CML). |
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266. Which of the following stages would be most appropriate for a patient who has Wilms tumor with diffuse tumor spillage into the abdominal cavity? A. I B. II C. III D. IV |
Correct answer is C. RATIONALE: This is the definition of stage III disease: Residual intraabdominal tumor (nonhematogenous) exists after the completion of surgery. Lymph node findings are positive, or peritoneal implants are found. The resected specimen has histologically positive margins, or the tumor has spilled into the abdominal cavity. |
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272. Which of the following radiation doses and fractionation schemes would be most appropriate for a 3-year-old child who has orbital embryonal rhabdomyosarcoma with gross disease? A. 36 Gy delivered in 1.8-Gy fractionation daily B. 45 Gy delivered in 1.8-Gy fractionation daily C. 50.4 Gy delivered in 1.8-Gy fractionation daily D. 59.4 Gy delivered in 1.1-Gy fractionation twice daily |
Correct answer is B. RATIONALE: The current recommendation is 45 Gy given in 25 fractions of 1.8 Gy with a 5-year overall survival (OS) rate of 96%, and a 5-year failure-free survival (FFS) rate of 89% based on IRS III data. |
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282. Which of the following syndromes is associated with neuroblastoma? A. Sick sinus syndrome B. Restless legs syndrome C. Opsomyoclonus-ataxia syndrome D. SIADH secretion |
Correct answer is C. RATIONALE: Opsomyoclonus-ataxia syndrome is seen in 1.3% of patients who have neuroblastoma. |
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301. Which of the following diagnostic imaging time points for tumor assessment should be used for EBRT target volume definition in a 3-year-old child with stage 4, high-risk neuroblastoma? A. Preinduction chemotherapy B. Postinduction chemotherapy but preoperative C. Postoperative D. Post-PBSCT |
Correct answer is B. RATIONALE: The postinduction, preoperative tumor volume allows coverage of the sites of risk and sparing of normal tissues if anatomy was distorted by mass effect of the tumor. |
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330. Which of the following conditions most commonly is found in patients with Langerhans cell histiocytosis? A. Diabetes insipidus B. Hyperthyroidism C. SIADH secretion D. Precocious puberty |
Correct answer is A. RATIONALE: Diabetes insipidus is seen in patients with Langerhans cell histiocytosis (LCH) because of histiocytic infiltration of the hypothalamus and pituitary gland. |
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342. Which of the following radiation doses to metastatic sites is recommended after PBSCT in a 3-year-old child who has high-risk neuroblastoma with multiple bony metastases on 131I-metaiodobenzylguanidine (MIBG) scan at diagnosis? A. 21.6 Gy to all initial sites B. 45 Gy to all initial sites C. 21.6 Gy to persistent sites on MIBG scan after induction chemotherapy D. 45 Gy to persistent sites on MIBG scan after induction chemotherapy |
Correct answer is C. RATIONALE: Current treatment regimens recommend 20-25 Gy to metastatic sites, which are resistant to the initial chemotherapy. |
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359. Which of the following subtypes of rhabdomyosarcoma has the worst prognosis? A. Botryoid B. Alveolar C. Embryonal D. Spindle cell |
Correct answer is B. RATIONALE: Alveolar rhabdomyosarcoma subtype, being younger than 1 year of age or older than 10 years of age, primary site, and higher stage or group are all independent prognostic factors for worse 5-year disease-free survival and overall survival outcomes. |
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8. A 2-year-old girl has had a complete resection of the right kidney for Wilms tumor involving the kidney, perihilar lymph nodes, and renal sinus vessels. No tumor spillage occurred during surgery, and chest radiograph is normal. Which of the following treatments would be most appropriate? A. Vincristine and dactinomycin alone B. Cyclophosphamide and etoposide alone C. 10.8 Gy of flank irradiation with chemotherapy D. 21.0 Gy of flank irradiation without chemotherapy |
Correct answer is A. RATIONALE: According to the current national Wilms tumor study group staging system, this child has stage II disease and would be treated with vincristine and dactinomycin alone. Stage III disease requires 10.8 Gy of local irradiation depending on the extent of involvement and tumor spill. |
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23. Which of the following stages is most appropriate for a patient with bilateral Wilms tumor? A. Stage IIIb B. Stage IV C. Stage IVb D. Stage V |
Correct answer is D. RATIONALE: Patients with bilateral Wilms tumors have stage V disease. Each kidney is considered separately as far as radiation therapy management; however, the therapeutic approach emphasizes a renal-function-sparing approach. |
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37. Which of the following characteristics is associated with patients who have Langerhans cell histiocytosis (LCH)? A. Ten percent of patients have cutaneous involvement. B. The mortality rate is 25% in patients with unifocal bone LCH. C. Immunohistochemical stains show a positive reaction for S-100 protein and CD1a. D. Multiorgan disease is more common in children older than 5 years of age. |
Correct answer is C. RATIONALE: Fifty percent to eighty percent of patients manifest cutaneous involvement. Studies of neonates and children under the age of 4 years have shown that 51% to 71% of children with Langerhans cell histiocytosis (LCH) present with multiorgan disease—much higher than that in older children and adults. Mortality for patients with unifocal bone LCH is 0.9%. REFERENCE: Satter, et al. Pediatric Dermatology. 2008;25:291-295. |
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42. Which of the following treatments is most appropriately performed first for a 4-year-old patient who has an International Classification Group D (Reese-Ellsworth Group 5) unilateral retinoblastoma? A. Enucleation B. Exenteration C. Radiation therapy D. Chemotherapy |
Correct answer is A. RATIONALE: International Classification Group D and Reese-Ellsworth Group 5 include very large tumors with vitreous seeding. Typically, there is a low probability of vision salvage; therefore, enucleation is recommended. |
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52. Which of the following patients with Langerhans cell histiocytosis (LCH) has the highest mortality rate? A. 1-year-old boy with multisystem involvement B. 6-year-old boy with multifocal bone disease C. 10-year-old girl with multisystem involvement D. 35-year-old man with isolated pulmonary LCH |
Correct answer is A. RATIONALE: Mortality rates for patients with Langerhans cell histiocytosis (LCH) are approximately as follows: An adult with isolated pulmonary LCH = 27%. A 1-year-old boy with multisystem involvement = 60% to 70%. A 10-year-old girl with multisystem involvement = 10% to 20%. A 6-year-old boy with multifocal bone disease = 5%. REFERENCE: Satter, et al. Pediatric Dermatology. 2008;25:291-295. |
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67. Which of the following staging classifications would apply for a 4-year-old girl with a 7-cm, unresectable, vaginal rhabdomyosarcoma with a 1-cm inguinal lymph node and no distant metastasis? A. Stage 1, group III B. Stage 2, group II C. Stage 3, group II D. Stage 3, group III |
Correct answer is A. RATIONALE: The rhabdomyosarcoma (RMS) staging classification incorporates the site, size, lymph node status, and metastatic status. RMS clinical grouping classification considers the degree of resection. All primary vaginal cancers with no metastasis are considered stage I; unresectable disease is considered to be group III. |
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95. What percent of neuroblastomas occur in the low thoracic or abdominal paraspinal ganglia? A. 5% B. 30% C. 45% D. 60% |
Correct answer is B. RATIONALE: According to a population-based study, 30% of neuroblastomas occur in the low thoracic or abdominal paraspinal ganglia. It is important for a radiation oncologist to know the common locations of neuroblastoma. REFERENCE: Bernstein, et al. A population-based study of neuroblastoma incidence, survival, and mortality in North America. Journal of Clinical Oncology. 1992;10(2):323-329. |
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101. Which of the following features is associated with Hodgkin lymphoma in children? A. Five percent of children present with stage IV disease. B. Forty to fifty percent of children have B symptoms. C. Eighty percent of children present with cervical lymphadenopathy. D. Lymphocyte-predominant lymphoma is the most common histologic subtype. |
Correct answer is C. RATIONALE: Fifteen to twenty percent of children present with stage IV Hodgkin lymphoma. Twenty-five to thirty percent of children have B symptoms. Nodular sclerosing histology is the most common subtype in all age groups. |
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120. Which of the following treatments would be most appropriate for a 6-month-old child with stage 1, low-risk neuroblastoma after complete tumor resection with no organ- or lifethreatening symptoms? A. Chemoradiation therapy B. Radiation therapy only C. Chemotherapy only D. Close follow-up |
Correct answer is D. RATIONALE: Patients with low-risk neuroblastoma have a cure rate of higher than 90%. After complete tumor resection for stage 1 disease, close observation or follow-up is the most appropriate management. REFERENCES: NCI PDQ Neuroblastoma. (http://www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional). COG 9641 protocol. Matthay KK, Haas-Kogan D, Constine l. Neuroblastoma. Pediatric Radiation Oncology. 4th edition. Chapter 6. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 179- 222. Perez, et al. Biologic variables in the outcome of stages I and II neuroblastoma treated with surgery as primary therapy: a children's cancer group study. Journal of Clinical Oncology. Jan 2000;18(1):18-26. |
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138. Which of the following treatments (listed in order of administration) would be most appropriate after incomplete resection of a high-risk neuroblastoma? A. Local radiation therapy, surgery, followed by chemotherapy B. Chemotherapy, local radiation therapy, followed by cis-retinoic acid C. Chemotherapy, surgery, myeloablative therapy, followed by local radiation therapy D. Chemotherapy, surgery, myeloablative therapy, local radiation therapy, followed by cis-retinoic acid |
Correct answer is D. RATIONALE: Myeloablative therapy has been shown to be superior to maintenance chemotherapy in the treatment of high-risk neuroblastoma, according to two randomized trials. In one of those trials, the addition of cis-retinoic acid has been shown to improve treatment outcome. REFERENCES: Matthay KK, Haas-Kogan D, Constine l. Neuroblastoma. Pediatric Radiation Oncology. 4 edition. Chapter 6. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 179-222. Berthold F, Boos J, Burdach S, et al. Myeloablative megatherapy with autologous stem-cell rescue versus oral maintenance chemotherapy as consolidation treatment in patients with high-risk neuroblastoma: a randomized controlled trial. Lancet Oncology. 2005;6(9):649-58. Matthay KK, Villablanca JG, Seeger RC, et al. Treatment of high-risk neuroblastoma with intensive chemotherapy, radiotherapy, autologous bone marrow transplantation, and 13-cis-retinoic acid. Children's Cancer Group. New England Journal of Medicine (NEJM). 1999;341(16):1165-73. NCI PDQ Neuroblastoma. (http://www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional) |
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150. Which of the following factors increases the risk for CNS dissemination in a patient with retinoblastoma? A. Extension into the orbital fat B. Invasion of >50% of the choroid C. Presence of multiple intraocular tumors D. Involvement of the optic nerve beyond the lamina cribrosa |
Correct answer is D. RATIONALE: Involvement of the optic nerve beyond the lamina cribrosa is a known risk factor for CSF spread because of the access of retinoblastoma cells to the subarachnoid space (the optic nerve is surrounded by meninges). REFERENCE: Halperin EC, Kirkpatrick JP. Retinoblastoma. Pediatric Radiation Oncology. 4th edition. Chapter 5. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 135-176. |
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180. Which of the following radiation treatments is most appropriate for acute lymphoblastic leukemia involving the testes? A. 18 Gy in 10 fractions B. 24 Gy in 12 fractions C. 30 Gy in 15 fractions D. 36 Gy in 18 fractions |
Correct answer is B. RATIONALE: Because the testicles are regarded as a sanctuary site, testicular radiation therapy to a dose of 20-24 Gy in 2-Gy fractions is given as a consolidative therapy after chemotherapy. REFERENCES: Hustu HO, et al. Extramedullary leukemia. Clinical Haematology (Clin Haematol). 1978;7(2):313-337. Bowman WP, et al. Isolated testicular relapse in acute lymphocytic leukemia of childhood: categories and influence on survival. Journal of Clinical Oncology. Aug 1984;2(8):924-9. Kun L. Leukemias in children. Pediatric Radiation Oncology. 4th edition. Chapter 2. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 15-39. |
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195. Myeloablative therapy for childhood leukemia: A. is used as consolidative therapy after initial remission in children with acute myeloid leukemia. B. is indicated for children who have acute lymphoblastic leukemia without the Philadelphia chromosome. C. does not include total body irradiation as part of the bone marrow transplant conditioning regimen. D. requires an allogeneic bone marrow transplant. |
Correct answer is A. RATIONALE: Myeloablative therapy is indicated in children who have acute lymphoblastic leukemia (ALL) with the Philadelphia chromosome (BCR-ABL or MLL-AF4). Cyclophosphamide/TBI as a conditioning regimen is better than busulfan cyclophosphamide for leukemia control. Both allogeneic and autologous bone marrow transplantations are effective in treating leukemias. REFERENCE: Kun L. Leukemias in children. Pediatric Radiation Oncology. 4th edition. Chapter 2. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 15-39. |
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120. What is the recommended dose in Gy for an 8-year-old boy with testicular relapse of acute lymphoblastic lymphoma (ALL)? (A) 12 (B) 24 (C) 36 (D) 45 |
Key: B Rationale: 24 Gy is the preferred dose. |
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202. A 3½-year-old patient who has average-risk medulloblastoma should receive which of the following craniospinal radiation therapy doses as part of a total radiation dose of 54 Gy before chemotherapy? A. 13.2 Gy B. 23.4 Gy C. 30.6 Gy D. 36.0 Gy |
Correct answer is B. RATIONALE: The current North American standard of craniospinal irradiation (CSI) in patients older than 3 years of age is 23.4 Gy with a boost to 54 to 55.8 Gy to the posterior fossa, followed by chemotherapy. Studies are underway to evaluate the avoidance of radiation therapy (RT) vs. the use of a lower dose of 18 Gy CSI. Some studies use weekly vincristine during RT, others do not. All studies use post-RT chemotherapy. |
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210. Which of the following findings is associated with a poor prognosis in children who present with acute lymphoblastic leukemia? A. White blood cell count of <50,000/mm3 B. B-cell immunophenotype C. Chromosomal hyperdiploidy D. Presence of t(9;22) |
Correct answer is D. RATIONALE: Options A-C are favorable features for acute lymphoblastic leukemia (ALL). The presence of translocation t(9;22) is a high-risk feature associated with a worse treatment outcome. REFERENCE: Kun L. Leukemias in children. Pediatric Radiation Oncology. 4th edition. Chapter 2. Philadelphia: Lippincott Williams & Wilkins Publishers. pp 15-39. |
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220. A 6-year-old patient undergoes complete resection of a fourth ventricular nonmetastatic grade II ependymoma. What is the most appropriate next management step? A. EBRT to the primary site B. Multiagent systemic chemotherapy C. Craniospinal radiation therapy with a boost D. Observation with serial MRI scans |
Correct answer is A. RATIONALE: Complete surgical resection has been found to be the most important treatment for patients with ependymoma. Following maximal surgery, local radiation therapy is recommended. Routine chemotherapy and craniospinal radiation therapy are no longer recommended because of the lack of efficacy. |
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226. What is a significant concern with the use of proton radiation therapy for pediatric patients? A. Inability to control the exit dose deposition in small separations B. Longer times required to deliver treatments and daily anesthesia C. Increased whole-body dose due to neutron production in the treatment room D. Increased whole-body dose due to lateral proton scattering |
Correct answer is C. RATIONALE: Proton machines can be a significant source of neutrons, which can result in an elevated whole-body dose for pediatric patients. While there is no consensus on this issue, it is something that is routinely considered when discussing pediatric proton beam treatments. |
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231. Which of the following hereditary syndromes is most likely to be found in a 1-year-old patient who has an enlarged abnormally enhancing optic chiasm? A. Neurofibromatosis B. Tuberous sclerosis C. von Hippel-Lindau D. Li-Fraumeni |
Correct answer is A. RATIONALE: An enlarged, abnormally enhancing optic chiasm in a 1-year-old patient is suggestive of an optic nerve glioma, which is associated with neurofibromatosis, type 1. Tuberous sclerosis is associated with subependymal giant cell tumors; von Hippel Lindau disease is associated with hemangioblastomas; Li-Fraumeni is associated with brain tumors and other tumors. |
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245. A 15-year-old girl with a large, unresectable, nonmetastatic iliac wing Ewing’s sarcoma has an excellent response to 12 weeks of chemotherapy. Which of the following chemoradiation therapy approaches should be recommended? A. 30 Gy to the prechemotherapy bone and soft tissue volume B. 45 Gy to the prechemotherapy bone and soft tissue volume C. 45 Gy to the prechemotherapy bone volume and postchemotherapy non-infiltrative soft tissue volume D. 55.8 Gy to the prechemotherapy bone volume and postchemotherapy non-infiltrative soft tissue volume |
Correct answer is D. RATIONALE: Gross unresectable disease is treated with a radiation dose of 55.8 Gy. A full dose of radiation therapy is administered to the infiltrative disease before chemotherapy, and it is delivered to the non-infiltrative “pushing” tumor bulk after chemotherapy. |
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274. A 16-year-old patient presents with a painful, enlarging mass in the distal thigh. Plain film radiography reveals a permeative diaphyseal femoral lesion with periosteal new bone formation lifting the bone cortex. What is the most likely diagnosis? A. Aneurysmal bone cyst B. Synovial sarcoma C. Ewing’s sarcoma D. Osteosarcoma |
Correct answer is D. RATIONALE: Osteosarcoma is the most likely diagnosis for a patient who is 16 years of age and presents with this disease location. The plain film with the cortical abnormality (Codman’s triangle) is suggestive of a malignant process. Ewing’s sarcoma tends to have metaphyseal rather than diaphyseal involvement. |
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9. An 8-year-old girl presents with petechiae, fatigue, leukocytosis, palsy of cranial nerve VI, and acute lymphoblastic leukemia with negative results on CSF cytology. The patient’s CNS classification status most likely is: A. CNS 0. B. CNS 1. C. CNS 2. D. CNS 3. |
Correct answer is D. RATIONALE: Any cranial nerve palsy at diagnosis of acute lymphoblastic leukemia is considered to be CNS leukemia, regardless of CSF cytology by definition. Therefore, CNS 3 is the proper classification. |
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21. Which of the following types of leukemia most commonly occurs in children? A. Acute lymphoblastic B. Acute myelogenous C. Chronic myelogenous D. Chronic lymphocytic |
Correct answer is A. RATIONALE: Acute lymphoblastic leukemia is the most common type of pediatric leukemia, accounting for 75% of cases. Acute lymphoblastic leukemia has a median age of occurrence of 10 to 12 years. The other disorders have a median age of occurrence in the 7th decade of life. REFERENCE: Gunderson LL, Tepper JE (eds). Clinical Radiation Oncology. 2000; p 1089. |
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32. What is the most appropriate EBRT regimen for a 15-year-old patient with an unresectable Ewing’s sarcoma of the pelvis after 12 weeks of systemic chemotherapy? A. 36 Gy to the initial tumor volume with a 9 Gy boost to the post-chemotherapy volume B. 45 Gy to the initial tumor volume with a 10.8 Gy boost to the post-chemotherapy volume C. 60 Gy to the initial tumor volume with a 6 Gy boost to the post-chemotherapy volume D. 60 Gy to the initial tumor volume with no boost |
Correct answer is B. RATIONALE: The current recommended dose is 55.8 Gy given in 31 fractions. The initial tumor volume encompasses the pre-chemotherapy volume with consideration of anatomic boundaries and boost of 10.8 Gy is given to the post-chemotherapy volume with consideration of normal tissue toxicities. |
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41. Which of the following genetic conditions is associated with an increased risk for the development of acute lymphoblastic leukemia? A. Down syndrome B. Warner syndrome C. Christmas syndrome D. von Hippel-Lindau syndrome |
Correct answer is A. RATIONALE: Down syndrome is associated with a 15% increased risk of developing acute lymphoblastic leukemia (ALL). REFERENCE: DeVita VT, Hellman S, Rosenberg SA (eds). Cancer: Principles & Practice of Oncology. 6th ed. Lippincott: 2001; p 2407. |
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48. A patient with a 15 cm Wilms tumor in the right kidney that extends into the renal vein, negative lymph nodes, and no tumor spill at the time of surgery has which of the following cancer stages? A. I B. II C. III D. IV |
Correct answer is B. RATIONALE: Stage I: A tumor confined to the kidney with an intact capsule and no prior biopsy. Stage II: Any tumor that is completely resected with negative margins but with extension beyond the kidney by regional extension of the tumor or of the blood vessels within the nephrectomy specimen outside the renal parenchyma. Lymph nodes are negative and there is not intra-operative spill. Stage III: Residual non-hematogenous tumor following surgery confined to the abdomen with any one of the following: Abdominal or pelvic lymph node involvement. Tumor penetration through the peritoneal surface. Peritoneal tumor implants. Incomplete resection because of tumor infiltration into vital structures. Tumor spillage before or during surgery. Tumor that is treated with pre-operative chemotherapy (with or without biopsy of any sort). Tumor removed in more than one piece. If the tumor thrombus in the renal vein is removed separately from the nephrectomy specimen. Extension of the primary tumor within vena cava into thoracic vena cava and heart (this is not stage IV). Stage IV: Distant metastasis outside of the abdomen or pelvis. Stage V: Synchronous bilateral tumors. |
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80. A 2-year-old patient’s tumor ruptures during resection of a large, right-sided Wilms tumor. Chemotherapy is scheduled to start during the second postoperative week. Which of the following radiation treatments would be most appropriate? A. 10.8 Gy to the preoperative tumor volume at the end of chemotherapy B. 10.8 Gy to the preoperative tumor volume starting before chemotherapy C. 10.5 Gy to the whole abdomen with a 10.8 Gy boost to the primary site starting before day 9 D. 10.5 Gy to the whole abdomen starting before postoperative day 9 |
Correct answer is D. RATIONALE: With a diffuse abdominal spill, 10.5 Gy is given to the whole abdomen on or before postoperative day 9. If there is no spill and there is a need for radiation therapy, 10.8 Gy is given to the primary site by day 9. |
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266. The standard diagnostic evaluation of neuroblastoma includes all of the following EXCEPT: (A) bone scan. (B) MIBG scan. (C) lumbar puncture. (D) urinary catecholamines. |
Key: C References: Matthay KK, Haas-Kogan DA, Constine LS. Neuroblastoma. Halperin E, Constine L, Tarbell N, Kun L. (eds). Pediatric Radiation Oncology. 4th edition. 2004; Lippincott, Williams & Wilkins. |
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201. Which of the following radiation dose regimens is most appropriate for group III orbital embryonal rhabdomyosarcoma? A. 45 Gy in 25 daily fractions B. 50.4 Gy in 28 daily fractions C. 54 Gy in 30 daily fractions D. 59.4 Gy in 54 twice-daily fractions |
Correct answer is A. RATIONALE: The recommended radiation dose used for group III orbital rhabdomyosarcoma in IRS V is 45 Gy in 25 fractions with good local control and survival. A dose of 50.4 Gy is given to non-orbital gross disease, 59.4 Gy given with a hyperfractionated regimen was not found to be better than 50.4 Gy given in a daily regimen in a prospective randomized IRS study. REFERENCE: Friedman, Tarbell, Constine. Rhabdomyosarcoma. Halperin, Constine, Tarbell, Kun (eds). Pediatric Radiation Oncology. 4th ed. pp 319-346. |
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215. Which of the following postoperative radiation therapy doses should be administered to a 7-year-old girl with a completely resected, 9 cm embryonal rhabdomyosarcoma of the vagina with negative histologic margins? A. 00 Gy B. 36 Gy C. 41.4 Gy D. 50.4 Gy |
Correct answer is A. RATIONALE: No radiation is required for a stage 1, group I embryonal rhabdomyosarcoma of the vagina. |
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228. What is the staging and group classification for a 3 cm alveolar rhabdomyosarcoma of the thigh with positive microscopic margins and inguinal lymph node involvement? A. Stage 3, group III B. Stage 3, group II C. Stage 2, group III D. Stage 2, group II |
Correct answer is B. RATIONALE: A 3 cm lesion in the extremity with positive lymph nodes would be stage 3. Positive microscopic margins are classified as group II. |
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220. A patient with a 6 cm rhabdomyosarcoma of the bladder 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: C Rationale: Based on the TNM pre-treatment staging classification. |
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241. Which of the following structures is a parameningeal site associated with rhabdomyosarcoma? A. Orbit B. Parotid gland C. Nasopharynx D. External auditory canal |
Correct answer is C. RATIONALE: Parameningeal RMS sites include the infratemporal fossa, middle ear, mastoid region, nasal cavity, nasopharynx, paranasal sinus, and pterygopalatine fossa (IMMNNPPP). The parotid and external auditory canal would be considered head and neck, non-parameningeal sites. The orbit is not a parameningeal site and has its own treatment algorithm. REFERENCE: Friedman, Tarbell, Constine. Rhabdomyosarcoma. Halperin, Constine, Tarbell, Kun (eds). Pediatric Radiation Oncology. 4th ed. pp 319-346. |
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254. What was the cumulative 10-year incidence of secondary neoplasms in patients, according to the Children's Cancer Group (CCG) acute lymphoblastic leukemia trials? A. <1% B. 1% to 3% C. 4% to 9% D. >10% |
Correct answer is B. RATIONALE: The cohort of over 8,000 patients from the Children's Cancer Group (CCG) studies showed an incidence of 1.3%. A large German study showed the incidence to be <3%. |
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257. Which of the following factors is associated with a favorable prognosis for patients with CNS germ cell tumors? A. Age of less than 3 years B. Elevated serum AFP level C. Pure germinoma histology D. Origin in the pineal gland |
Correct answer is C. RATIONALE: Unlike mixed germ cell tumors, pure CNS germinomas have an excellent prognosis after chemoradiation regimens or extended-field radiation therapy alone. Being less than 3 years of age is associated with worse event-free survival because of the reluctance to use radiation therapy. An elevated serum AFP level is diagnostic of a nongerminomatous component. The site of the disease (pineal vs. hypothalamic) is not of prognostic significance. |
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267. Which of the following types of abdominal tumor most commonly occurs during adolescence? A. Neuroblastoma B. Rhabdomyosarcoma C. Non-Hodgkin lymphoma D. Wilms tumor |
Correct answer is C. RATIONALE: According to SEER 2002 data, non-Hodgkin lymphoma is very uncommon in children under 3 years of age. The incidence increases in adolescence. The incidence of neuroblastoma, rhabdomyosarcoma, and Wilms tumor is highest in patients under 10 years of age and is very uncommon in the adolescent age group. |
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272. Which of the following doses of prophylactic cranial irradiation would be most appropriate for a 4-year-old boy with high-risk acute lymphoblastic leukemia? A. 18 Gy B. 23 Gy C. 36 Gy D. 45 Gy |
Correct answer is A. RATIONALE: For prophylactic treatment, 18 Gy is sufficient. Recent protocols use even lower doses of 12 Gy. |
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305. Which of the following brain tumors occurs most commonly in children? A. Astrocytoma B. Ependymoma C. Medulloblastoma D. Germinoma |
Correct answer is A. RATIONALE: Astrocytomas make up approximately 30% of pediatric brain tumors in patients younger than 15 years of age and 20% of patients between 15 to 19 years of age per the Central Brain Tumor Registry of the United States (CBTRUS). Medulloblastoma is the most common posterior fossa tumor. Germinoma is more common in adolescent males. |
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312. Which of the following management options would be best for a 10-year-old girl who has undergone complete resection of a cerebellar pilocytic astrocytoma? A. Surveillance B. Temozolomide C. Carboplatin and vincristine D. 50 to 54 Gy of EBRT |
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