Musawi 24
Musawi 24
Musawi 24
Down syndrome
B. Klinefelter syndrome
C. Inguinal hernia
D. Testicular microlithiasis
E. Cryptorchidism
100. Which of the following gonadal tumors can cause virilization or precocious
puberty?
A. Yolk sac tumor
B. Dysgerminoma
C. Sertoli-Leydig cell tumor
D. Seminoma
E. Gonadoblastoma
107. A 15-year-old boy who recently has been referred as a case of thyroid mass
suggestive of tumor by sonography for evaluation and preoperative assessment.
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Upon further history gathering and physical examination, you noticed his father
was affected with thyroid cancer and underwent surgical resection and medical
treatment. On examination, he has marfanoid features and multiple oral
mucosal neuroma of the tongue with central midline thyroid swelling of 6*6 cm
that moves with swallowing and left sided prominent cervical
lymphadenopathy. The mother reported that he has long history of constipation
and being unable to cry tears since early childhood. Two years ago, he
underwent thorough investigation for multiple endocrine neoplasia.
Of the following, the MOST likely diagnosis is
A. differentiated thyroid carcinoma
B. papillary thyroid carcinoma
C. medullary thyroid carcinoma
D. follicular thyroid carcinoma
E. benign thyroid lesion
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Chapter 21
Cancer and Benign Tumors
Answers
HASANEIN H. GHALI
1.(C) Osteosarcoma and acute myeloid leukemia.
2.(B) Epstein-Barr virus is associated with lymphoproliferative disorders,
Hepatitis B virus and Hepatitis C virus are associated with hepatocellular
carcinoma, and Human papilloma virus with cervical cancer.
3.(C)
4.(D)
5.(D)
6.(A) School-age children might present with lymphoma or with brain tumors
7.(C)
8.(B) Lymphoma, especially during adolescence, often manifests as an anterior
mediastinal mass. Symptoms such as chronic cough, unexplained shortness of
breath, or “new-onset asthma” are typical with this presentation and are often
overlooked.
9.(D)
10.(D)
11.(C) t(9;22) is for CML, t(1;19) is for ALL, t(2;13) is for rhabdomyosarcoma, and
t(11;22) is for Ewing sarcoma.
12.(C)
13.(C) Pain is a serious cause of suffering among patients with cancer. It may be
the result of organ obstruction or compression or bone metastasis, or it may be
neuropathic.
14.(E) Methotrexate and cranial radiation.
15.(B) Cisplatin and Carboplatin
16.(D) Renal insufficiency is associated with the use of Ifosfamide, cisplatin and
carboplatin. Cardiomyopathy is linked to the use of daunomycin and
Adriamycin. Pulmonary fibrosis is linked to the use of bleomycin, busulfan and
lomustine. Peripheral neuropathy is linked to vincristine and vinblastine.
17.(D) Hyperkalemia due to tumor lysis syndrome (leukemia), hyponatremia
due to SIDH (leukemia and CNS tumors), disseminated intravascular coagulation
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due to sepsis and tumor factors (promyelocytic leukemia), superior vena cava
syndrome due to superior mediastinal mass (lymphoma).
18.(D)
19.(A)
20.(D) Most susceptible are tissues or organs with high rates of cell turnover:
bone marrow, oral and intestinal mucosa, epidermis, liver, and spermatogonia.
Least susceptible to chemotherapy and radiation therapy are cells that do not
replicate or that replicate slowly, such as neurons, muscle cells, connective
tissue, and bone.
21.(A) Dermatitis is the most common general adverse effect, because skin is
always in the treatment field.
22.(B)
23.(A) They should receive leukoreduced blood products to prevent transfusion-
associated reactions and infections.
24.(D) T-cell leukemia
25.(A) Blueberry muffin lesions or subcutaneous nodules
26.(B)
27.(C) They are discrete masses, known as chloromas or granulocytic sarcomas.
These masses can occur in the absence of apparent bone marrow involvement
and typically are associated with a t(8;21) translocation. Chloromas also may be
seen in the orbit and epidural space.
28.(C) PML-RARA, of favorable prognosis, is a gene rearrangement involving the
retinoic acid receptors.
29.(A) Acute promyelocytic leukemia, characterized by a gene rearrangement
involving the retinoic acid receptor [t(15;17); PML-RARA], is very responsive to
all-trans -retinoic acid (ATRA, tretinoin) combined with anthracyclines and
cytarabine. The success of this therapy makes marrow transplantation in first
remission unnecessary for patients with this disease.
30.(C) No treatment is indicated.
31.(C) Philadelphia chromosome t(9;22) (q34;q11), resulting in a BCR-ABL fusion
protein. This translocation, although characteristic of CML, is also found in a
small percentage of patients with ALL.
32.(D)
33.(B) It’s association with Noonan syndrome has been shown to have
spontaneous resolution.
34.(D) Children who are younger than 1 yr or older than 10 yr or who have an
initial leukocyte count of >50,000/μL are considered to be high risk. Additional
characteristics that adversely affect outcome include T-cell immunophenotype
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or a slow response to initial therapy. Chromosomal abnormalities, including
hypodiploidy, the Philadelphia chromosome t(9;22), and MLL gene
rearrangements, portend a poorer outcome. More favorable characteristics
include a rapid response to therapy, hyperdiploidy, trisomy of specific
chromosomes (4, 10, and 17), and rearrangements of the ETV6-RUNX1
35.(B) Daunomycin (Daunorubicin)
36.(A)
37.(B) Respiratory distress usually is related to anemia but can occur in patients
as the result of a large anterior mediastinal mass (e.g., in the thymus or nodes).
This problem is most frequently seen in adolescent boys with T-cell ALL. T-ALL
also usually has a higher leukocyte count.
38.(E) Systemic symptoms, classified as B symptoms, that are considered
important in staging are unexplained fever >38°C (100.4°F), weight loss >10%
total body weight over 6 mo, and drenching night sweats.
39.(A) Typically, patient presented with cervical or supra clavicular swelling.
40.(C) According to Lugano classification for Hodgkin lymphoma.
41.(D) According to Lugano classification for Hodgkin lymphoma.
42.(B) Patients whose disease relapses >12 mo after chemotherapy alone or
combined-modality therapy have the best prognosis, and their relapses usually
respond to additional standard therapy.
43.(E) Mediastinal tumor is stratified as stage III; a primary completely resected
gastrointestinal tumor is stratified as stage II; any paraspinal tumor is stratified
as stage III; two or more nodal sites on the same site of the diaphragm is
stratified as stage II.
44.(E) Lymphoblastic lymphoma typically manifests as a symptomatic
mediastinal mass and also has a predilection for spreading to the bone marrow,
CNS, and testes in males.
45.(D) Localized or advanced disease requires 12-24 mo of therapy, including
chemotherapy, intrathecal therapy, and cranial radiation in CNS-positive
lymphoma
46.(D) Radiation therapy is used only in special circumstances, such as CNS
involvement in lymphoblastic lymphoma or the presence of acute superior
mediastinal syndrome or paraplegias
47.(C) Newly diagnosed patients, especially those with BL or LBL, are at high risk
for TLS. These patients require vigorous hydration, frequent electrolyte
monitoring, and either an xanthine oxidase inhibitor (e.g., allopurinol, 10
mg/kg/day orally in 3 divided doses daily) or a recombinant urate oxidase (e.g.,
rasburicase, 0.2 mg/kg/day intravenously once daily for up to 5 days)
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48.(B)
49.(B) The overall mortality among this group approaches 30%. Patients with
CNS tumors have the highest morbidity—primarily neurologic—of all children
with malignancies.
50.(D) On chromosome 10q23.
51.(A) 17q11, the gene NF1.
52.(A) Pilocytic astrocytoma and medulloblastoma/primitive neuroectodermal
tumors are the most common tumors in children 0-14 years.
53.(A) During the 1st yr of life, supratentorial tumors predominate and most
often include choroid plexus complex tumors and teratomas.
54.(B)
55.(C) Supratentorial tumors are more frequently associated with focal motor
weakness, focal sensory changes, language disorders, focal seizures, and reflex
asymmetry. The classical triad of headache, vomiting and nausea is more in
favor of infratentorial tumors.
56.(B) MRI with and without gadolinium is the standard neuroimaging.
57.(C)
58.(A) The suprasellar and pineal regions are preferential sites for germ cell
tumors.
59.(C) The mean age is 6 years. The majority occurs in the posterior fossa. The
most important prognostic factor is the extent of tumor resection. The younger
the patient, the poorer the prognosis. Tumor in the posterior fossa is associated
with poorer outcome. Surgery is rarely curative.
60.(A)
61.(B)
62.(B)
63.(B)
64.(C)
65.(A) Massive involvement of the liver with metastatic disease, with or without
respiratory distress.
66.(C)
67.(B) Intractable secretory diarrhea caused by tumor secretion of vaso-
intestinal peptides. Tumors are generally biologically favorable.
68.(C) Infants <18 mo old also can present in unique fashion, termed stage MS
(previously 4S; see later), with widespread subcutaneous tumor nodules,
massive liver involvement, limited bone marrow disease, and a small primary
tumor without bone involvement or other metastases. The stage MS disease
can spontaneously regress.
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69.(A) Lung, regional lymph nodes and liver.
70.(C)
71.(C)
72.(E) Associated with BRCA2 and PALB2.
73.(B) WT1 missense mutation.
74.(C)
75.(B) Hypertension is present in about 25% of patients at presentation and has
been attributed to increased renin activity. Abdominal pain (40%), gross
painless hematuria (18%), and constitutional symptoms such as fever, anorexia,
and weight loss are other findings at diagnosis.
76.(A) Bilateral renal involvement. B, D, and E are stage IV while C is stage III.
77.(A) Mesoblastic nephroma is the most common solid renal tumor identified
in the neonatal period and the most frequent benign renal tumor in childhood.
78.(A) These tumors may occur at virtually any anatomic site but are usually
found in the head and neck (25%), orbit (9%), genitourinary tract (24%), and
extremities (19%); retroperitoneal and other sites account for the remainder of
primary sites. The incidence at each anatomic site is related to both patient age
and tumor type.
79.(A) Vaginal rhabdomyosarcoma may manifest as a grapelike mass of tumor
tissue bulging through the vaginal orifice, known as sarcoma botryoides, and
can cause urinary tract or large bowel symptoms. Vaginal bleeding or
obstruction of the urethra or rectum may occur. Similar findings can be noted
with uterine primaries.
80.(C) Both occurred in the second decade, both have a slight male predilection,
fever is common in Ewing and rare in osteogenic sarcoma, lung and bones are
common metastatic site for both.
81.(A) Rothmund-Thomson syndrome is a rare condition associated with short
stature, skin telangiectasia, small hands and feet, hypoplasticity or absence of
the thumbs, and a high risk of osteosarcoma.
82.(B) The lesion may be mixed lytic and blastic in appearance, but new bone
formation is usually visible. The classic radiographic appearance of
osteosarcoma is the sunburst pattern.
83.(D) One of the most important prognostic factors in osteosarcoma is the
histologic response to chemotherapy; a poor histologic response is ≥10% viable
tumor.
84.(D)
85.(A) The differential diagnosis for a paraspinal/perivertebral mass in a child is
wide and includes benign and malignant etiology. Malignant lesions such as
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chondrosarcoma, Ewing sarcoma, peripheral neuroectodermal tumor (PNET)
and neuroblastoma metastasis, amongst others. Benign lesions would not
usually show bone destruction.
86.(C) A, B and D are for osteosarcoma, while A and D are for chondrosarcoma.
87.(D) The other choices are radiosensitive.
88.(D) Osteochondroma, nonossifying fibroma, unicameral bone cyst, and
enchondroma can be diagnosed by radiographs only.
89.(B) Night pain that awakens a child suggests malignancy, but relief of such
pain with aspirin is common with osteoid osteomas
90.(E) Other choices are locally aggressive and need to be treated.
91.(A) It is the most common benign lesion in childhood, usually arise from the
long bones, with very rare malignant degeneration. It is diagnosed by
radiograph alone unless it is suspicious of malignant condition.
92.(C) And Ewing Sarcoma
93.(B) It has a high rate of malignant transformation
94.(D) Retinoblastoma classically presents with leukocoria, a white pupillary
reflex, which often is first noticed when a red reflex is not present at a routine
newborn or well-child examination or in a flash photograph of the child.
95.(A) The diagnosis is established by the characteristic ophthalmologic findings
of a chalky, white-gray retinal mass with a soft, friable consistency. Imaging
studies are not diagnostic, and biopsies are contraindicated.
96.(E) The differential diagnosis of retinoblastoma includes other causes of
leukocoria, including persistent hyperplastic primary vitreous, Coats disease,
vitreous hemorrhage, cataract, endophthalmitis from Toxocara canis, choroidal
coloboma, retinopathy of prematurity, and familial exudative vitreoretinopathy.
97.(B) All other distractors are associated with increased risk of testicular
cancer.
98.(C)
99.(B)
100.(C) Sertoli-Leydig cell tumors and granulosa cell tumors produce hormones
that can cause virilization, feminization, or precocious puberty, depending on
pubertal stage and the balance between Sertoli cells (estrogen production) and
Leydig cells (androgen production).
101.(D) Except for tumors of the central nervous system, radiation therapy is
limited to those tumors that are not amenable to complete excision and are
refractory to chemotherapy.
102.(C) The serum α-fetoprotein level is elevated with endodermal sinus tumors
and may be minimally elevated with teratomas.
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103.(D) The pure fetal histology subtype predicts a more favorable outcome,
and the small cell undifferentiated subtype is associated with normal AFP levels
and predicts a worse outcome.
104.(E) Hepatocellular carcinoma occurs mostly in adolescents and often is
associated with hepatitis B or C infection. It also occurs in the chronic form of
hereditary tyrosinemia, galactosemia, glycogen storage disease, α1 -antitrypsin
deficiency, progressive familial intrahepatic cholestasis, and biliary cirrhosis.
Alagille syndrome and aflatoxin B contamination of food are associated risk
factors.
105.(C) Hemangiomas, the most common benign tumors of infancy, occur in
approximately 5–10% of term infants.
106.(A) Ulceration may lead to secondary infection.
107.(C) Medullary thyroid carcinoma is an uncommon disease in childhood that
almost always occurs in the context of an autosomal dominant, hereditary
endocrine tumor syndrome that arises secondary to activating mutations in the
RET protooncogene: multiple endocrine neoplasia type 2a (MEN2A) or type 2b
(MEN2B).
108.(B) The skeleton is involved in 80%, Skin in 50%, Lymph nodes in 33%,
Hepatosplenomegaly in 20%, and lungs in 10%.
109.(C) The risk organs are liver, spleen, and the hematopoietic (bone marrow)
system.
110.(A) Late (fibrotic) complications, whether hepatic or pulmonary, are
irreversible and require organ transplantation to be definitively treated.
111.(C)
112.(A) Physical examination often reveals hepatosplenomegaly (70–100%),
lymphadenopathy (20–50%), respiratory distress (40–90%), jaundice, and
symptoms of CNS involvement (50%) that are not unlike those of aseptic
meningitis or acute demyelinating encephalomyelitis.
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Chapter 22
Nephrology
Questions
QAHTAN ALOBAIDY
1. When the blood passes through the glomerular capillaries, plasma is filtered
through the glomerular capillary walls.
Which of the following plasma molecules filter freely through the glomeruli?
A. Peptides
B. Albumin
C. Globulins
D. Interleukin-6
E. Myoglobin
2. The “bedside” Schwartz formula is the most widely used pediatric formula for
GFR estimation, and is based on the an empirical constant, patient height, and
which of the following?
A. Inulin
B. Cystatin c
C. Blood urea
D. Serum creatinine
E. Fructose polymer
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D. Porphyrin
E. Urates
8. A 7-year-old male presents with sudden onset of gross hematuria and edema.
Blood pressure is 130/90 mmHg. Investigations results; blood urea 160mg/dl,
low C3 level, and urinalysis shows RBC casts. He had history of treatment for
acute tonsillitis with penicillin for 10 days.
Which of the following is the MOST likely diagnosis?
A. IgA nephropathy
B. Alport syndrome
C. Essential mixed cryoglobulinemia
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D. Post infectious glomerulonephritis
E. Membranoproliferative glomerulonephritis
10. Which of the following can cause low serum complement (C3, CH 50)?
A. Polyaretritis nodosa
B. Goodpasture syndrom
C. Hypersensitivity vasiculitis
D. Henoch-Schönlein Purpura
E. Subacute bacterial endocarditis
11. A 4-year-old boy had three days history of hematuria and mild puffy face.
Blood pressure was 140/90 mmHg. He underwent surgery for hydrocephalus
one month ago. Urinalysis: RBC +++, protein +, granular cast +, hyaline cast +,
blood urea 80 mg/dl, serum creatinine 0.9 mg/dl and low complement level.
Which of the following is the MOST likely organism causing this complication?
A. Streptococcus viridans
B. Staphylococcus epidermidis
C. Staphylococcus aureus
D. Klebsiella pneumoniae
E. Pseudomonas aeruginosa
12. A 15-year-old female presented with fever, fatigue, and generalized edema.
On examination you find malar discoid rash, investigations shows Hb 7 g/dl,
WBC 10*103uL, platelet 50*103uL, positive antinuclear antibody test, serum
albumin 2.2mg/dl, total serum protein 4mg/dl, and urinalysis revealed +++
proteinuria.
Which of the following is the MOST likely class of this disease?
A. I
B. II
C. III
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D. IV
E. V
14. Which of the following are the MOST common post-renal transplant
bacterial infections?
A. Urinary tract infections
B. Osteomyelitis
C. Peritonitis
D. Meningitis
E. Sinusitis
15. Which of the following is an option for treatment of acute cellular rejection
post renal transplant?
A. Plasmapheresis
B. Intravenous immunoglobulin
C. Thymoglobulin
D. Rituximab
E. Proteasome inhibitors
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17. Which of the following microorganisms is blamed in causing hemolytic-
uremic syndrome?
A. Streptococcus pneumonia
B. Mycoplasma hominis
C. Urea plasma urealyticum
D. Staphylococcus epidermidis
E. Klebsiella pneumoniae
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21. A full term baby born to a 33-year-old diabetic mother presents with gross
hematuria and a palpable mass in the right flank. His blood pressure 100/60
mmHg, blood urea 80 mg/dl, serum creatinine 1.3 mg/dl with a maternal serum
creatinine prior to delivery of 0.9 mg/dl, and his complete blood count: HGB 12
g/dl, WBC 17*103 uL, platelet count 53*103uL with normal PTT, PT, and
fibrinogen.
Of the following, the MOST likely diagnosis is
A. Wilms tumor
B. multicystic dysplastic kideny
C. renal vein thrombosis
D. polycystic kidney disease
E. neuroblastoma
22. You are reviewing a 15-year-old boy who had recently been diagnosed with
autosomal dominant polycystic kidney disease (ADPKD) in the renal clinic. You
proceed to examine his cardiovascular system.
What other features you expect to find on examination?
A. Dilated cardiomyopathy
B. Mitral stenosis
C. Aortic stenosis
D. Renal bruit secondary to renal artery stenosis
E. Mitral valve prolapse
23. An 8 -year-old boy presents with severe anemia and polyuria. Examination
revealed growth failure, retinal degeneration with blood urea of 66 mg/dl and
serum creatinine of 3 mg/dl.
Of the following, the MOST likely diagnosis is
A. infantile polycystic disease
B. autosomal recessive polycystic kidney disease
C. nephronophthisis
D. Bardet-biedl syndrome
E. Von hippel-lindau syndrome
26. Which of the following is the BEST method to predict proteinuria in patient
with diabetes mellitus?
A. Microalbuminuria test
B. Dipstick testing
C. Boiling test
D. Spot urine for protein/creatinine ratio
E. 24 hr urine for protein and creatinine excretion
30. Which of the following types of nephrotic syndrome had the worst
prognosis?
A. Focal segmental glomerulosclerosis
B. Membranoproliferative glomerulonephritis
C. C3 glomerulopathy
D. Membranous nephropathy
E. Minimal change disease
35. Which of the following is the MOST appropriate initial treatment to reduce
proteinuria in minimal change disease?
A. Protein restriction in diet
B. Lipid lowering agent
C. Angiotensin converting enzyme inhibitor
D. Loop diuretics
E. Prednisolone
36. Which of the following drug is recommended as initial therapy for children
with steroid-resistant nephrotic syndrome?
A. Tacrolimus
B. Mycophenolate mofetil
C. Levamisole
D. Chlorambucil
E. Azathioprine
38. Which of the following urine findings can differentiate classic distal renal
tubular acidosis (type I) from proximal renal tubular acidosis (type II)?
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A. Phosphaturia
B. Aminoaciduria
C. Hypercalciuria
D. Glycosuria
E. Uricosuria
40. Which of the following is the BEST way to confirm the diagnosis of
cystinosis?
A. Detection of cystine crystals in the cornea
B. Measurement of leukocyte cysteine content
C. Normal anion gap (hyperchloremic) metabolic acidosis
D. Measurementof amino acids in urine
E. Cysteine urine concertation
41. Which of the following findings can differentiate renal tubular acidosis (type
IV) from other types?
A. Hyperchloremia
B. Hyperkalemia
C. Nephrocalcinosis
D. Normal anion gap metabolic acidosis
E. Normal glomerular filtration rate
42. Which of the following types of renal tubular acidosis (RTA) typically cause
urine pH >6?
A. Distal
B. Proximal
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C. Combined proximal and distal
D. Hyperkalemic type
E. Cystinosis
43. Which of the following types of renal tubular acidosis (RTA) is associated
with hypercalciuria?
A. Distal (type I) RTA
B. Proximal (type II) RTA
C. Combined proximal and distal (type III) RTA
D. Hyperkalemic (type IV) RTA
E. Nephropathic cystinosis
44. Which of the following causes false low bicarbonate level in interpretation
of arterial blood gas analysis?
A. Traumatic blood draws
B. Large volumes of blood sampling
C. Addison disease
D. Chronic diarrhea
E. Diabetic ketoacidosis
46. A first cousin parents brought their 13-month-old toddler with hyperthermia
and massive polyuria. Examination reveals failure to thrive, tachycardia,
tachypnea, and normal blood pressure. Investigations reveal blood urea
70mg/dL, serum creatinine 0.5mg/dL, ABG pH 7.55, HCO3 32 mEq/l, Na +2
127mg/dL, CL- 80mg/dl, K+2.8mg/dL, and lactate 2mmol/L, serum osmolality
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