Neoplastic Diseases and Tumors

Nelson Self Assessments website 17th Edition

Question . 1. Which of the following cancers occurs primarily during childhood?

A. Breast cancer
B. Renal cell cancer
C. Wilms tumor
Explanation: Wilms tumor occurs most commonly among infants and young
children. (See Chapter 483 in Nelson Textbook of Pediatrics, 17th ed.)
D. Prostate cancer
E. Colon cancer

Question . 2. Which of the following cancer types is least common among children?

A. Leukemias
B. Brain and central nervous system tumors
C. Lymphomas
D. Bone tumors
E. Epithelial carcinomas
Explanation: In contrast with tumors among adults, epithelial carcinomas are
uncommon among children. (See Chapter 483 in Nelson Pediatrics, 17th ed.)

Question . 3. All of the following statements regarding adult and pediatric tumors are
true Except:

A. In comparison with adult cancers, childhood cancers are infrequent

B. Death due to childhood cancers results in a much longer loss of potential lifespan
than is the case with cancer in adults
C. Distribution of cancer types differs markedly between adults and children
D. Genetic abnormalities are associated with most pediatric cancers but not with
most adult cancers
Explanation: Specific genetic conditions are believed to account for <5% of all
pediatric malignancies. (See Chapter 483 in Nelson Pediatrics, 17th ed.)
E. Epithelial malignancies (carcinomas) are far more common in adults than in
children

Question . 4. All of the following statements regarding the epidemiology of childhood

cancer are true Except:

A. Childhood cancer accounts for approximately 15-20% of all cases of cancer

Explanation: Only about 1% of new cases of cancer in the United States occur in
children, yet malignancy remains the second leading among children 1-14 yr of
age. (See Chapter 483 in Nelson Textbook of Pediatrics, 17th ed.)
B. Malignant neoplasms are the second leading cause of death among children 1-14
yr of age
C. Leukemia and central nervous system tumors predominate in children
D. Chronic leukemia is more common in adults than children
E. The incidence of cancer during childhood shows a nadir between 7 and 12 yr of
age.
The Neoplastic Diseases and Tumors Nelson Self Assessments website 17th Edition 1
Question . 5. Which of the following cancers has the highest incidence in young children
(<7 yr of age)?

A. Ewing sarcoma
B. Hodgkin disease
C. Testicular cancer
D. Retinoblastoma
Explanation: The incidence of retinoblastoma peaks during the first 2 yr of life.
The other tumors occur with increasing incidence during childhood and
adolescence. (See Chapter 483 in Nelson Textbook of Pediatrics, 17th ed.)
E. Osteosarcoma

Question . 6. A 2-mo-old boy is found to have cryptorchidism. For which of the

following malignancies is he at increased risk?

A. Rhabdomyosarcoma
B. Pelvic osteosarcoma
C. Germ cell tumor
D. Wilms tumor
E. None of the above
Explanation: Cryptorchidism is a risk factor for testicular germ cell tumors. (See
Chapter 484 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 7. Which of the following cancers is most closely associated with Epstein-
Barr virus?

A. Osteosarcoma
B. Non-Hodgkin lymphoma
Explanation: Epstein-Barr virus is associated with African (endemic) Burkitt
lymphoma, nasopharyngeal carcinoma, post-transplantation non-Hodgkin
lymphoma, non-Hodgkin lymphoma in persons with congenital
immunodeficiencies (e.g., X-linked lymphoproliferative syndrome),
leiomyosarcomas in immunocompromised persons, and some cases of Hodgkin
disease. (See Chapter 484 in Nelson Textbook of Pediatrics, 17th ed.)
C. Ewing sarcoma
D. Wilms tumor
E. Hepatoblastoma

Question . 8. A 14-yr-old girl presents with bilateral cervical lymphadenopathy that has
progressed over the last 4 wk. It is slowly worsening despite antibiotic therapy with
cephalexin, which was prescribed 2 wk ago. On physical examination, the lymph nodes
are enlarged, matted, and nontender. There is mild splenomegaly. The most likely
diagnosis is:
A. Human immunodeficiency virus infection
B. Infectious mononucleosis (Epstein-Barr virus infection)
C. Tuberculosis
D. Methicillin-resistant Staphylococcus aureus infection
E. Acute lymphocytic leukemia
Explanation: Leukemias and lymphomas are the most common malignant
neoplasms among young children. (See Box 485-2 and Chapter 485 in)

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Question . 9. The definitive diagnostic procedure for the child described in Question 8 is:

A. Urinalysis
B. Lymph node biopsy and culture
Explanation: A lymph node biopsy and culture is the definitive means of
diagnosing lymphoma. (See Chapter 485 in Nelson Pediatrics, 17th ed.)
C. Plain x-ray film
D. Ultrasound study of the neck
E. Complete blood count and differential

Question . 10. Highly specific indicators of cancer in children include:

A. Diffuse enlargement of the pons (brainstem)

B. White reflex emanating from the retina
C. Both A and B
Explanation: Diffuse enlargement of the pons suggests central nervous system
tumor, and white retinal reflex suggests retinoblastoma. (See Box 485-2 and
Chapter 485 in Nelson Textbook of Pediatrics, 17th ed.)
D. Neither A nor B

Question . 11. Of the following signs of cancer, the most likely to occur in children is:

A. Abnormal discharge from a body orifice

B. Change in a mole or wart
C. Focal neurologic deficit
Explanation: Any focal neurologic deficit in the motor or sensory system,
especially a decrease in cranial nerve function, should prompt further
investigation for central nervous system malignancy. (See Box 485-2 and Chapter
485 in Nelson Textbook of Pediatrics, 17th ed.)
D. Cough, hoarseness, or difficulty swallowing
E. Change in urination or defecation pattern

Question . 12. Metabolic complications of anticancer therapy include:

A. Hyperuricemia
B. Hyperkalemia
C. Hyperphosphatemia
D. Hypocalcemia
E. All of the above
Explanation: Anticancer therapy can result in substantial breakdown of tumor
cells (tumor lysis syndrome) with release of large quantities of phosphates and
potassium into the circulation. Hypocalcemia can result in the setting of
inadequate renal function. (See Chapter 486 in Nelson Textbook of Pediatrics,
17th ed.)

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Question . 13. A 12-yr-old girl receives cranial, neck, and spinal irradiation for cancer.
All of the following are likely long-term sequelae of the radiotherapy Except:

A. Interstitial fibrosis
B. Scoliosis
C. Impaired cognition and intelligence
D. Pituitary dysfunction
Explanation: Many sequelae of radiation do not become apparent until the child is
fully grown. Irradiation can result in infertility, second cancers, scoliosis,
pulmonary dysfunction (interstitial fibrosis), leukoencephalopathy, impaired
cognition and intelligence, hypothyroidism, isolated growth hormone deficiency,
and panhypopituitarism. Cardiomyopathy is classically associated with the
anthracyclines (doxorubicin and daunomycin) but can also occur with irradiation.
(See Chapter 486 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 14. All of the following statements concerning childhood lymphocytic and
myelogenous leukemias are Except:

A. Leukemias as a group are the most common childhood cancer

B. Acute lymphocytic leukemia accounts for approximately 75% of cases
C. The incidence is higher in white children than in black children
D. The clinical features at presentation are similar
E. The responses to therapy and prognoses are similar
Explanation: The clinical features of the childhood leukemias are similar, because
all involve severe disruption of bone marrow function. There is marked variability
in response to therapy and in the prognosis. (See Chapter 487 in Nelson, 17th ed.)

Question . 15. All of the following statements concerning acute lymphocytic leukemia
(ALL) are true Except:

A. Most cases (about 85%) are derived from T-cell progenitors

Explanation: About 85% of cases of ALL are derived from progenitors of B cells,
about 15% are derived from T cells, and about 1% are derived from mature B
cells. (See Chapter 487 in Nelson Textbook of Pediatrics, 17th ed.)
B. Staging of ALL is based on bone marrow biopsy and cerebrospinal fluid
examination
C. Chromosomal abnormalities are identified in most cases of ALL
D. Exposure to medical radiation is associated with an increased incidence of ALL
E. Childhood ALL was the first form of cancer shown to be curable with
chemotherapy and irradiation

Question . 16. BCharacteristic presenting symptoms and signs of childhood leukemia include:

A. Pallor
B. Petechiae
C. Lymphadenopathy
D. Bone or joint pain
E. All of the above
Explanation: Most children with leukemia present with symptoms of less than 4
weeks' duration. Most children with ALL have pallor, 50% have petechiae or

The Neoplastic Diseases and Tumors Nelson Self Assessments website 17th Edition 4
 mucous membrane bleeding, 60% have lymphadenopathy, 25% have fever, and
about 25% have bone pain and arthralgias caused by leukemic infiltration of the
perichondral bone or joint or by leukemic expansion of the marrow cavity. (See
Chapter 487 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 17. Which of the following factors indicates an increased risk of relapse for
childhood ALL?

A. Age greater than 1 yr

B. Age younger than 10 yr
C. Presenting white blood cell count under 100,000/mm3
D. Any chromosomal abnormality
Explanation: Chromosomal abnormalities are found in most cases of ALL; some
indicate a favorable prognosis, some unfavorable, and some have no apparent
influence. (See Table 487-1 and Chapter 487.1 in Nelson Textbook of Pediatrics,
17th ed.)
E. Rapid response to therapy

Question . 18. A 4-yr-old boy has a bone marrow biopsy that shows 4% blasts. No blasts
are seen on peripheral blood smear. Findings on physical examination and other
laboratory studies are normal. The most likely diagnosis is:

A. Acute lymphoblastic leukemia

B. Acute myelogenous leukemia
C. Chronic myelogenous leukemia
D. Preleukemia syndrome
E. None of the above. this is a normal bone marrow biopsy
Explanation: ALL is diagnosed by a bone marrow evaluation that demonstrates
more than 25% of the bone marrow cells as a homogeneous population of
lymphoblasts. (See Chapter 487 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 19. A breakthrough treatment for chronic myelogenous leukemia in patients

with the BCR-ABL chromosomal translocation is:

A. Imatinib
Explanation: Imatinib was designed specifically to inhibit BCR-ABL tyrosine
kinase. (See Chapter 487.4 in Nelson Textbook of Pediatrics, 17th ed.)
B. Umbilical cord blood transplantation
C. Cis-retinoic acid
D. Timed intensive induction chemotherapy
E. Liposomal anthracycline

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Question . 20. All of the following statements about leukemia in children with Down
syndrome are true Except:

A. Leukemia occurs more frequently among children with Down syndrome

B. Acute lymphoblastic leukemia is the most common type of leukemia that occurs
in these children
C. Acute myeloid leukemia has a better outcome in children with Down syndrome
than in children without Down syndrome
D. Chemotherapy is easier to administer because of decreased toxicity compared
with the general population
E. Almost all neonates with Down syndrome and transient myeloproliferative
syndrome eventually develop leukemia
Explanation: About 20-30% of neonates with Down syndrome who develop a
transient leukemia or myeloproliferative syndrome will develop typical leukemia
within the first few years of life. (See Chapter 487.3 in Nelson Textbook of
Pediatrics, 17th ed.)

Question . 21. Leukemia in infants is commonly associated with:

A. A better prognosis than in older children

B. Philadelphia chromosome
C. FAB L3 (Burkitt) type
D. A translocation involving chromosome 11
Explanation: More than two thirds of cases of infant leukemia demonstrate
rearrangements of the MLL gene, classically a translocation involving the q23
band of chromosome 11, and it is this subset of patients that largely accounts for
the very high relapse rate. (See Chapter 487.6 in Nelson Pediatrics, 17th ed.)
E. Spontaneous resolution

Question . 22. The most important extramedullary site of relapse in childhood ALL is:

A. Adrenal glands
B. Kidney
C. Lung
D. Heart
E. Central nervous system
Explanation: The most important extramedullary sites of relapse of ALL are the
central nervous system and the testes. Intrathecal therapy is key to prevention of
later central nervous system relapse. Testicular relapse occurs in 1-2% of boys
with ALL, usually after completion of therapy. (See Chapter 487.1 in Nelson
Textbook of Pediatrics, 17th ed.)

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Question . 23. A neonate with Down syndrome experiences transient myeloproliferative
syndrome that spontaneously resolves. Which of the following statements best
characterizes the prognosis?

A. This almost never recurs

B. This recurs intermittently during early childhood
C. This indicates an increased risk of leukemia
Explanation: Neonates and infants with Down syndrome may experience transient
myeloproliferative syndrome, which mimics congenital leukemia. These children
have a 20-30% risk of subsequent acute leukemia within the first few years of life.
(See Chapter 487.3 in Nelson Textbook of Pediatrics, 17th ed.)
D. This invariably develops later into leukemia
E. This invariably develops later into malignant histiocytosis

Question . 24. An older child experiences weight loss and night sweats and has a white
blood cell count of 80,000/mm3 and a platelet count of 600,000/mm3. Chromosomal
analysis of bone marrow cells reveals a clonal disorder of stem cells with the specific
translocation, t(9;22)(q34;q11), which is also known as the Philadelphia chromosome.
The diagnosis is:

A. Acute myelocytic leukemia

B. Acute megakaryocytic leukemia
C. Chronic myelogenous leukemia
Explanation: The Philadelphia chromosome is associated with chronic
myelogenous leukemia. (See Chapter 487.4 in Nelson Pediatrics, 17th ed.)
D. Juvenile chronic myelogenous leukemia
E. Hodgkin disease

Question . 25. A 15-yr-old white girl reports that she has had a fever, weight loss, and
night sweats for 3 mo. On physical examination, she is found to have painless swelling of
the left cervical and supraclavicular lymph nodes. Her liver and spleen are not enlarged.
The nearest pediatric oncology center requires a 4-hr drive. Before transfer, the initial
evaluation of the patient should include:

A. Bone marrow aspiration

B. Abdominal CT study
C. Chest radiograph
Explanation: Chest radiography is important for two reasons: first, to document
mediastinal lymph node involvement, and second. to determine whether these
lymph nodes threaten the patency of the airway. (See Chapter 488 in Nelson
Textbook of Pediatrics, 17th ed.)
D. Head CT study
E. Erythrocyte sedimentation rate

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Question . 26. The chest radiograph of the patient described in Question 25 reveals
mediastinal lymphadenopathy. The best diagnostic test at the referral center is:

A. Abdominal CT study
B. Head CT study
C. Bone marrow biopsy
D. Lymph node biopsy
Explanation: At this time, a lymph node biopsy can confirm suspicion of Hodgkin
disease. Thereafter, CT scans and bone marrow biopsy are useful in staging the
extent of the lymph node and extranodal involvement. (See Chapter 488 Nelson)
E. Thoracic CT study

Question . 27. A 7-yr-old boy weighing 24 kg presents with painless right

supraclavicular lymphadenopathy. Chest radiograph reveals a mediastinal mass
measuring more than one-third of the thoracic diameter. Biopsy of the lymph node
reveals Hodgkin disease, nodular sclerosing type. Which of the following indicates a
poorer prognosis?

A. Hilar lymphadenopathy
Explanation: For Hodgkin disease, the presence of hilar lymphadenopathy with a
mediastinal mass larger than one third of the thoracic diameter indicates bulky
disease and a poorer prognosis. (See Chapter 488.1 in Nelson, 17th ed.)
B. Unilateral cervical adenopathy
C. Intense mediastinal uptake on gallium 67 scan
D. Pruritus
E. Weight loss of 2 kg

Question . 28. Small non-cleaved cell non-Hodgkin lymphoma (SNCCL) (Burkitt's type)
is characterized by all of the following Except:

A. t(8:14), t(8;22), or t(2;8) translocation

B. B lymphocyte origin
C. Presence of EBV genome
D. Mediastinal lymphadenopathy
Explanation: SNCCL presents as an abdominal tumor in 80% of U.S. cases. Jaw
involvement occurs in <20% of U.S. cases, compared with 70% of younger
patients in equatorial Africa. (See Chapter 488.2 in Nelson Pediatrics, 17th ed.)
E. Jaw involvement

Question . 29. In children with newly diagnosed non-Hodgkin lymphoma, which of the
following laboratory studies provides an indirect measure of tumor burden?

A. Serum uric acid

B. Erythrocyte sedimentation rate
C. Serum lactic dehydrogenase (LDH)
Explanation: Elevation of the level of serum lactate dehydrogenase (>500 U/L)
correlates with tumor mass and has proved useful for stratifying therapy intensity.
(See Chapter 488.2 in Nelson Textbook of Pediatrics, 17th ed.)
D. Serum ALT (alanine aminotransferase)
E. Absolute lymphocyte count

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Question . 30. A 5-yr-old boy presents with progressive headache that is associated with
nausea and vomiting. Physical examination reveals bilateral papilledema. The most likely
diagnosis is:

A. Pseudotumor cerebri
B. Central nervous system tumor
Explanation: The classic triad of headache, nausea and/or vomiting, and
papilledema is associated with midline and infratentorial tumors. (See Chapter
489 in Nelson Textbook of Pediatrics, 17th ed.)
C. Brain abscess
D. Cerebrovascular event
E. Central nervous system lupus

Question . 31. For the boy described in Question 30, the most appropriate management is:

A. Lumbar puncture, including measurement of CSF pressure

B. Blood culture and CSF culture and empirical antibiotic therapy
C. Cerebral angiography
D. Emergency MRI scan
Explanation: MRI is the preferred neuroimaging test for suspected central nervous
system tumors. (See Chapter 489 in Nelson Textbook of Pediatrics, 17th ed.)
E. ANA and high-dose methylprednisolone

Question . 32. A 4-yr-old child presents with a hard, fixed abdominal mass that causes
discomfort. Physical examination also shows hypertension. The most likely etiology is:

A. Hodgkin's disease
B. Ewing sarcoma
C. Nephroblastoma
D. Neuroblastoma
Explanation: Most neuroblastomas arise in the abdomen, either in the adrenal
gland or in retroperitoneal sympathetic ganglia. Catecholamine production may
cause hypertension, whereas other vasoactive substances may produce a secretory
diarrhea. (See Chapter 490 in Nelson Textbook of Pediatrics, 17th ed.)
E. Renal clear cell carcinoma

Question . 33. Which of the following is a favorable prognostic factor in neuroblastoma?

A. Age <1 yr
Explanation: Children with neuroblastoma with early-stage disease without
amplified MYCN or chromosome 1p deletion can usually be cured with surgery
alone. Children <1 yr of age have a 95% 3-yr survival rate, compared with 25-
50% for children 1-5 yr of age. (See Chapter 490 in Nelson Pediatrics, 17th ed.)
B. Age > 5yr
C. Stage 3 or 4
D. Amplified MYCN
E. Chromosome 1p deletion in 80-90% of cells

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Question . 34. A previously healthy 2-yr-old boy presents with irritability, low-grade
fever for 1 wk, weight loss, and limp. He has no history of vomiting or diarrhea or of
recent trauma. On physical examination, he is pale with a temperature of 38C. Blood
pressure is 150/95 mm Hg, respiratory rate 24/min, and pulse 130/min. Weight is
decreased 1 kg from his weight at a previous visit at 18 mo of age. Ears and throat are
normal, but there appear to be bruises below both eyes. Lungs are clear to auscultation
and the heart sounds are normal. His abdomen appears distended, but abdominal
examination is unrevealing because of his irritability that prevents adequate palpation.
When he tries to walk, he refuses to bear weight on his left leg, but the leg appears
normal. The most appropriate diagnostic test for this patient is:

A. Blood culture
B. Abdominal CT scan
Explanation: Patients with abdominal neuroblastoma present with a firm, nodular
mass that causes abdominal pain. Some tumors produce catecholamines that can
cause sweating and hypertension. (See Chapter 490 in Nelson Pediatrics, 17th ed.)
C. Coagulation screen including platelet count, PT, PTT, and fibrinogen
D. Synovial fluid analysis of left hip
E. Plain x-ray film of entire left leg

Question . 35. A previously healthy 20-mo-old girl is noted by her mother to be more
irritable and to be falling more frequently than usual when she walks. She seems to be
having trouble with balance but does not appear in pain. There was no decrease in
appetite, vomiting, diarrhea, or fever. Her mother observes her for a week, hoping the
clumsiness will resolve. It does not resolve but worsens, and the child begins to be more
uncooperative and to have random, darting eye movements. She is taken to her
pediatrician, who also notes these abnormalities and performs an MRI study of the brain.
Findings on the MRI study are normal. Which of the following is the most likely
explanation for these clinical findings?

A. Acute hepatic encephalopathy

B. Cysticercosis
C. Behavioral problems
D. Neuroblastoma
Explanation: Opsomyoclonus ("dancing eyes and dancing feet") is a
paraneoplastic syndrome of autoimmune origin that is associated with
neuroblastoma. (See Chapter 490 in Nelson Textbook of Pediatrics, 17th ed.)
E. Acute demyelinating encephalomalacia

Question . 36. A 3-yr-old child presents with an abdominal mass and microscopic
hematuria. The most likely tumor is:

A. Hodgkin disease
B. Ewing sarcoma
C. Wilms tumor
Explanation: Wilms tumor accounts for most renal neoplasms in children. (See
Chapter 491 in Nelson Textbook of Pediatrics, 17th ed.)
D. Neuroblastoma
E. Renal clear cell carcinoma

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Question . 37. A 9-yr-old previously healthy white girl manifests progressive painless
proptosis, periorbital edema, and decreased visual acuity of the left eye during a 2-mo
period. The most likely diagnosis is:

A. Pseudotumor cerebri
B. Trichinosis
C. Retinoblastoma
D. Rhabdomyosarcoma
Explanation: Orbital rhabdomyosarcoma is a common site for
rhabdomyosarcoma, which produces local signs as it grows and displaces normal
tissues. (See Chapter 492 in Nelson Textbook of Pediatrics, 17th ed.)
E. Orbital cellulitis

Question . 38. The most common soft tissue sarcoma in children is:

A. Synovial sarcoma
B. Malignant fibrous histiocytoma
C. Fibrosarcoma
D. Rhabdomyosarcoma
Explanation: Rhabdomyosarcoma is the most common soft tissue sarcoma in
childhood. (See Chapter 492 in Nelson Textbook of Pediatrics, 17th ed.)
E. Alveolar (soft part) sarcoma

Question . 39. A 4-yr-old boy presents with urinary obstruction. Ultrasound study reveals
a pelvic mass. There is no history of urinary tract infections or other illnesses. The most
likely diagnosis is:

A. Wilms tumor
B. Neuroblastoma
C. Germ cell tumor
D. Rhabdomyosarcoma
Explanation: Rhabdomyosarcoma is the most common soft tissue sarcoma of
childhood. Wilms tumor is an renal tumor, which would not be found in the
pelvis. (See Chapter 492 in Nelson Textbook of Pediatrics, 17th ed.)
E. Hydronephrosis

Question . 40. Osteosarcoma occurs with the highest incidence in which of the following
age groups?

A. Neonates
B. Children <5 yr of age
C. Children 5-10 yr of age
D. Adolescents
Explanation: The highest risk period for development of osteosarcoma is during
the adolescent growth spurt. (See Chapter 493 in Nelson Textbook of Pediatrics,
17th ed.)

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=====================

Figure 493-2 Radiograph of an osteosarcoma of the femur with typical "sunburst"

appearance of bone formation

Question . 41. A 15-yr-old boy presents with a palpable swelling of the humerus that is
associated with pain that awakens him at night. There is a sunburst pattern on the x-ray
film (Figure). The most likely diagnosis is:

A. Ewing sarcoma
B. Osteosarcoma
Explanation: Pain and swelling are the most common presenting symptoms of
osteosarcoma and Ewing sarcoma. Osteosarcoma has a characteristic sunburst
pattern on x-ray films. Ewing sarcoma is associated with periosteal elevation, or
"onion-skinning," on x-ray. (See Chapter 493 in Nelson Pediatrics, 17th ed.)
C. Histiocytosis X
D. Osteochondroma
E. Benign bone cyst

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Question . 42. A 15 yr-old female basketball player sustained a knee injury 4 wk
previously. An initial knee x-ray was read as normal. She has been receiving physical
therapy for the past 3 wk with no relief of her pain. Repeat examination shows diffuse
swelling around the knee, which is most prominent in the distal femur. Of the following,
which is the most appropriate next step in management of this patient?

A. Repeat knee x-ray

B. Treatment with a nonsteroidal anti-inflammatory drug
C. Arthroscopy
D. Continuation of physical therapy for another 2 weeks
E. MRI study
Explanation: A bone tumor should be suspected in a child or adolescent with
persistent bone pain and swelling, with or without systemic symptoms. (See
Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 43. A 12-yr-old boy complains of pain and swelling in his right upper arm
with onset 2 wk previously. He has also had night pain. He has had fever with
temperatures to 101C for the past 2 days. An x-ray film of the humerus shows periosteal
elevation along the shaft. The most appropriate next step is:

A. Blood culture
B. IV antibiotics
C. Erythrocyte sedimentation rate (ESR)
D. MRI study of humerus
Explanation: MRI is the neuroimaging test of choice for bone tumors. (See
Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)
E. Bone scan

Question . 44. A 12 yr-old boy has completed multiagent chemotherapy with

doxorubicin hydrochloride (Adriamycin), methotrexate, and cisplatin and limb salvage
surgery for nonmetastatic osteosarcoma. Possible late effects of chemotherapy include all
of the following Except:

A. Cardiotoxicity
B. Sterility
C. Hearing loss
D. Second malignancy
E. Cataracts
Explanation: Cardiotoxicity is associated with doxorubicin hydrochloride
(Adriamycin). (See Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)

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Question . 45. A 9-yr-old boy develops local pain and swelling of the left lateral aspect
of the skull. Physical examination reveals marked tenderness and warmth. Plain film
shows a well-defined radiolucent skull lesion. The skeletal survey is otherwise normal.
The most likely diagnosis is:

A. Eosinophilic granuloma
B. Letterer-Siwe disease
C. Hand-Schller-Christian disease
D. Osteomyelitis of the skull
E. Brain abscess
Explanation: Eosinophilic granuloma is a monostotic or polyostotic disease with
no extraskeletal involvement. This differentiates eosinophilic granuloma from
other forms of Langerhans cell histiocytosis (Letterer-Siwe or Hand-Schller-
Christian variants). (See Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)

Question . 46. Which of the following benign bone processes may be associated with
visceral involvement?

A. Osteoid osteoma
B. Aneurysmal bone cyst
C. Osteochondroma
D. Langerhans cell histiocytosis
Explanation: Children with Langerhans cell histiocytosis and bone lesions should
be evaluated for visceral involvement because treatment of Hand-Schller-
Christian disease and Letterer-Siwe disease is more complex and often requires
systemic therapy. (See Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)
E. Fibrous dysplasia

Question . 47. Which of the following bone neoplasms is associated with a characteristic
chromosomal translocation?

A. Ewing sarcoma
Explanation: A specific chromosomal translocation, t(11;22), or a variant thereof,
is present in most cases of the Ewing family of tumors. (See Chapter 493 in
Nelson Textbook of Pediatrics, 17th ed.)
B. Osteosarcoma
C. Fibrous dysplasia
D. Eosinophilic granuloma
E. Osteoid osteoma

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=======================================

.... sorry

Question . 48. A 5-yr-old boy presents with pain and swelling of the proximal right
lower leg over the past 2 weeks, with limitation of movement of the right knee. A
radiograph (Figure) of the femur is shown. The most likely diagnosis is:

A. Acute osteomyelitis
B. Chronic osteomyelitis
C. Osteoid osteoma
D. Ewing sarcoma
Explanation: Ewing sarcoma has a characteristic radiographic onion-skin
appearance, caused by periosteal reaction around the lytic bone lesion. (See
Chapter 493 in Nelson Textbook of Pediatrics, 17th ed.)
E. Osteosarcoma

Question . 49. A 14-yr-old boy presents with a bony, nonpainful mass in the distal femur
that has been slowly enlarging. There are no associated symptoms. The mass is most
likely to represent which of the following tumors?

A. Ewing sarcoma
B. Osteosarcoma
C. Langerhans cell histiocytosis
D. Osteochondroma
Explanation: Osteochondroma (exostosis) is one of the most common benign
bone tumors in children. Many of these tumors are asymptomatic or are detected
when the child or the parent notes a bony, nonpainful mass. Most arise in the
metaphyses of long bones. (See Chapter 493 in Nelson Pediatrics, 17th ed.)
E. Benign bone cyst

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Question . 50. The highest incidence of retinoblastoma has been noted in which of the
following age groups?
A. Infants
Explanation: Retinoblastoma occurs in 1 of 18,000 live births in the United States.
(See Chapter 494 in Nelson Textbook of Pediatrics, 17th ed.)
B. Children 2-5 yr of age
C. Children 5-10 yr of age
D. Adolescents
E. None of the above?the risk of retinoblastoma is similar in all age groups

Question . 51. On routine office ophthalmoscopic examination of a 14-mo-old girl, you

have a hard time seeing the fundus but observe no gross external physical abnormalities.
The extraocular muscles appear intact, and the remaining physical findings are normal. A
red reflex was reportedly present at birth. The most appropriate next step in management
would be to:

A.
Reassure the parents that nothing is wrong
B.
Obtain Toxoplasma and cytomegalovirus titers
C.
Obtain an MRI scan
D.
Refer the patient to an infectious disease specialist
E.
Refer the patient to an ophthalmologist for examination performed with the child
under general anesthesia
Explanation: Difficulty visualizing the fundus raises the suspicion of
retinoblastoma and warrants immediate attention and examination. An MRI scan,
or alternatively a CT scan, may also identify this ocular tumor. (See Chapter 494
in Nelson Textbook of Pediatrics, 17th ed.)
=========================================

Question . 52. An infant presents with the finding shown in the Figure. The cancer most
likely to be associated with this finding is:
A. Wilms tumor
B. Germ cell tumor
C. Retinoblastoma
Explanation: Leukocoria (white pupillary reflex) and strabismus are often the
presenting signs of retinoblastoma. (See Chapter 494 in Nelson Textbook of
Pediatrics, 17th ed.)
D. Rhabdomyosarcoma
E. Leiomyosarcoma

The Neoplastic Diseases and Tumors Nelson Self Assessments website 17th Edition 16
Question . 53. A 10-yr-old boy presents with a painless mass involving the right testicle.
Possible diagnoses include all of the following Except:

A. Leukemia
B. Rhabdomyosarcoma
C. Germ cell tumor
D. Testicular torsion
Explanation: Testicular (spermatic cord) torsion produces acute pain and swelling
of the scrotum. (See Chapter 495 in Nelson Textbook of Pediatrics, 17th ed.)
E. Hydrocele

Question . 54. Which of the following is the most common neoplasm involving the liver
in children?

A. Hepatocellular carcinoma
B. Hepatoblastoma
Explanation: Primary hepatic tumors are rare in children, accounting for 1% of
malignancies in childhood. Approximately two thirds of hepatic tumors in
children are hepatoblastomas. (See Chapter 496 in Nelson Pediatrics, 17th ed.)
C. Neuroblastoma
D. Wilms tumor
E. Rhabdomyosarcoma

Question . 55. An infant has a hemangioma that grows rapidly in size, leading to
thrombocytopenia and microangiopathic hemolytic anemia. Which of the following terms
describes this condition?

A. Kaposi-like form of infantile hemangioma

B. Albright syndrome
C. Kasabach-Merritt syndrome
Explanation: Kasabach-Merritt syndrome is trapping of platelet and red blood
cells within a hemangioma with activation of the clotting system within the
vasculature of the hemangioma. (See Chapter 497 in Nelson Pediatrics, 17th ed.)
D. Maffucci syndrome
E. Ollier disease

Question . 56. An infant has a hemangioma over the eyelid that grows rapidly in size,
obstructing vision. The most reasonable systemic therapy would be administration of:

A. Erythropoietin
B. Heparin
C. Platelet-activating factor
D. Corticosteroids
Explanation: For hemangiomas that are life-threatening or that threaten vital
functions such as eyesight, a trial of oral corticosteroids is warranted.
Approximately 30% respond dramatically and begin to regress within 1 wk, 40%
stabilize or show minimal response, and the remainder do not respond. Other
treatments for hemangiomas that do not respond to corticosteroids include
Interferon- , laser therapy, and surgery. (See Chapter 497 in Nelson, 17th ed.)
E. Vitamin A

The Neoplastic Diseases and Tumors Nelson Self Assessments website 17th Edition 17
Question . 57. A 1-yr-old girl has a history of fever, anemia, severe skin eruption,
generalized lymphadenopathy, and pulmonary infiltrates. A skull radiograph (Figure)
shows multiple lesions. The most likely diagnosis is:

A. Osteosarcoma
B. Osteochondritis
C. Langerhans cell histiocytosis
Explanation: Bone lesions with Langerhans cell histiocytosis may be single or
numerous and are seen most commonly in the skull. (See Chapter 499 in Nelson
Textbook of Pediatrics, 17th ed.)
D. Leukemia
E. Ewing sarcoma

Question . 58. The recommended treatment for the child described in Question 57 is:

A. Curettage of the bone lesions

B. IVIG
C. Interferon-
D. Combination chemotherapy
Explanation: Multisystem Langerhans cell histiocytosis should be treated with
systemic multiagent chemotherapy. (See Chapter 499 in Nelson Textbook of
Pediatrics, 17th ed.)
E. Corticosteroids

Question . 59. Regarding the radiographic finding in Question 57, if the same finding
were seen in a 4-yr-old girl with localized swelling on her skull, and there were no other
symptoms, which of the following would be the recommended treatment?

A. Curettage of the bone lesions

Explanation: Curettage of the bone lesion. Therefore, treatment should be
minimal and directed at arresting the progression of a lesion (e.g., a bone lesion)
that could result in permanent damage before it resolves spontaneously. Curettage
or low-dose radiation therapy may accomplish this goal. (See Chapter 499 in
Nelson Textbook of Pediatrics, 17th ed.)
B. IVIG
C. Interferon-
D. Combination chemotherapy
E. Corticosteroids

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