PEDS Final EXAM Review EXAM
QUESTIONS AND CORRECT ANSWERS
WITH RATIONALE
, PEDS FINAL EXAM REVIEW yc yc yc
PEDIATRIC CANCER yc
Wilms Tumor : Unilateral (more common) or bilateral tumor of kidne
yc yc yc yc yc yc yc yc yc yc
y Most common renal neoplasm in children, good prognosis
yc yc yc yc yc yc yc yc yc
Median age @ dx: 2-3 yrs yc yc yc yc yc
Clinical Presentation yc
Palpable abd. mass in healthy child, vascularized encapsulated jelly-
yc yc yc yc yc yc yc yc
like (RUPTURE), regular borders Some have pain, microscopic or gross hematuria, malaise, fever,
yc yc yc yc yc yc yc yc yc yc yc yc yc
HTN
G/U abnl’s: ambig genitalia cryptorchidism yc yc yc
hypospadius Congenital hem yc yc
ihypertrophy
Aniridia
Assoc.syndrome: Beckwith- yc
Wiedeman Treatment: 80% good prognosis yc yc yc yc
Unilateral: Nephrectomy yc
Vincristine & Actinomycin-D for 6months- yc yc yc yc
15months Bilateral: Bilateral partial resection, if possible yc yc yc yc yc yc
Same therapy with addition of adriamycin yc yc yc yc yc
Remove kidney with largest tumor, abdominal radiation for remaining Che yc yc yc yc yc yc yc yc yc
motherapy complications: yc
Mucositis, hair loss, nausea vomiting, bone marrow depression
yc yc yc yc yc yc yc
NO INFECTIONS SHOULD BE TREATED LIGHTLY
yc yc yc yc yc
Infections are the leading cause of death. yc yc yc yc yc yc
Should avoid crowds or exposure to communicable diseases.
yc yc yc yc yc yc yc
Tumor lysis syndrome: metabolic complication caused by breakdown products of dying cells HYPERURIC
yc yc yc yc yc yc yc yc yc yc yc yc
EMIA, HYPERPHOSPHATEMIA, HYPERKALEMIA, HYPOCALCEMIA yc yc yc
Occurs within 24-48 hours of chemotherapy yc yc yc yc yc
Treatment: hydration to increase urine production, correct electrolyte imbalances
yc yc yc yc yc yc yc yc
Goal: prevent renal failure
yc yc yc yc
Management of Infusion Devices yc yc yc
Most children will have a external central line or implanted venous access device (IVAD or port-a-
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
cath) Guidelines for care are institution specific:
yc yc yc yc yc yc
Rapid, easily obtained venous access yc yc yc yc
Monitor lines for infection (must culture if febrile) M yc yc yc yc yc yc yc yc
aintain patency by heparin flushing q 4 weeks yc yc yc yc yc yc yc
Radiation long term effects: cognitive defects, slow bone growth, growth hormone deficiency, dental problems
yc yc yc yc yc yc yc yc yc yc yc yc yc
Brain Tumors: Most common solid tumor, 2nd only to leukemia among childhood cancers (17% of all childhood CA).
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
2200 new dx in US/yr. yc yc yc yc
Classification system is difficult due to lack of a universally accepted grading syste
yc yc yc yc yc yc yc yc yc yc yc yc
m. 5 year survival ranges 55-75%.
yc yc yc yc yc
Signs & Symptoms: yc yc
Headaches: Worse in am or nocturnal yc yc yc yc yc
Vomiting: Without nausea
yc yc yc
Increased ICP: yc
Personality changes yc
Drowsiness yc
Resistance to being held yc yc yc
Increased FOC: Delayed closure or elevation of anterior fontan yc yc yc yc yc yc yc yc
el Loss of developmental milestones
yc yc yc yc
Papilledema yc
Head tilt yc
Abnormal reflexes and speech yc yc yc yc
Cranial nerve abnormalities yc yc
Presentation varies with site, age, developmental level 6 yc yc yc yc yc yc yc
0% infratentorial (cerebellum or brain stem)
yc yc yc yc yc
Diploplia
Gait disturbance Gait ataxia, truncal ataxia Nystag yc yc yc yc yc yc
mus
Weakness
40% supratentorial (cerebral hemispheres, hypothalamus, thalamus, optic chiasm, pineal region) Sei
yc yc yc yc yc yc yc yc yc yc
zures
DownloadedycbyycWamboycJeniyc(
m)
, Headaches
Infants: Irritability, Listlessness, Vomiting
yc yc yc
DownloadedycbyycWamboycJeniyc(
m)
, Diagnosis: Head CT or MRI yc yc yc yc
Treatment: Surgery (complete resection is ideal)
yc yc yc yc yc
Radiation: effective, can not be used on child <4 due to effects on brain Chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc yc
therapy: resistant yc
LEUKEMIA (ALL (best), CML (teens), AML (worst prognosis), T-cell ALL)
yc yc yc yc yc yc yc yc yc
ALL - Acute Lymphoblastic Leukemia (lymphocyte)
yc yc yc yc yc
Most common childhood cancer (<15 yrs.) 90% cure rat
yc yc yc yc yc yc yc yc
e Accounts for 75% of all childhood CA
yc yc yc yc yc yc yc
5-
year relative survival with ALL diagnosed in 2000 is near 80%
yc yc yc yc yc yc yc yc yc yc yc
Males > females (1.2:1) yc yc yc
Peak age 2-3yrs yc yc
Annually: (2,400 cases per year) yc yc yc yc
Downs (trisomy 21) : 1 in 95 (10-20x greater)
yc better prognosis and cure rate, require lighter chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc yc
??? Etiology ??? yc yc
? Infection yc
? Electromagnetic field exposure yc yc yc
? Exposure during pregnancy yc yc yc
? Radiation yc
? Chemical or drug exposure yc yc yc yc
? Familial predisposition yc yc
? Chromosomal aberrations yc yc
T cell ALL -
yc yc yc
thymus, anterior mediastinal mass, deviated trachea Clinical Presen
yc yc yc yc yc yc yc yc
tation of Leukemia
yc yc
Leukocytosis/leukopenia
Neutropenia- absolute neutrophil count 3,000-5,000 yc yc yc yc
neutropenic if <1000 yc yc
Lymphoblasts on peripheral smear
yc yc yc yc
Anemia: usually present at dx, RBCs lysed by overabundance of WBCs
yc yc yc yc yc yc yc yc yc yc
Thrombocytopenia (75%) petechiae (does not blanch), purpura, mucosal (nasal/scleral) bleeding, (he yc yc yc yc yc yc yc yc yc yc
maturia & GI hemorrhage are rare) yc yc yc yc yc
Fever (60%): frequently associated with infection
yc yc yc y c yc
Pain in bones/joints (23%): due to bone marrow infiltration by leukemic cells (also called blasts) Ano
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
rexia (common) & abdominal pain
yc yc yc yc
Weight loss (infrequent) yc yc
Pallor and mucosal bleeding (48%)
yc yc yc yc
Lymphadenopathy(50%): Clavicular and epitrochlear nodes worrisome yc yc yc yc yc
Splenomegaly (63%) yc
Hepatosplenomegaly (68%) yc
Diagnostic labs NP/LMD’s office
yc yc yc yc
CBC: will give a high index of suspicion
yc yc yc yc yc yc yc
Low hgb, low platelets, high or low WBC count yc yc yc yc yc yc yc yc
Serum chemistry: uric acid elevation due to rapid turnover of malignant cells leads to uric acid nephropat
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
hy LDH - frequently elevated
yc yc yc yc
Lytes - especially if dehydrated
yc yc yc yc
Bone marrow aspirate/biopsy: should be done at tertiary care site, ONLY DIFINITIVE DIAGNOSTI
yc yc yc yc yc yc yc yc yc yc yc yc
C Lumbar puncture: Rule out CNS disease
yc yc yc yc yc yc
Tumor markers, DNA testing, special stains Fl
yc yc yc yc yc yc
ow cytometry yc
Cytogenetics
ALL Leukemia Tmtt: Multi-
yc yc yc
agent chemotherapy, 90% complete remission in 29 days (0.01% leukemia cells) Intrathecal chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc
through spinal tap (maintain trendelenburg to coat brain, remove CSF prior to)
yc yc yc yc yc yc yc yc yc yc yc
3-Phases (approx.. 2 yr. Duration): yc yc yc yc
Induction-
introduce chemo, hospitalized Consolidation-
yc yc yc yc
CNS chemo, weekly spinal taps
yc yc yc yc yc
Maintenance- beginning at 6- yc yc yc
8 months, hair returns, tolerable, monthly visits Will get radiation if relapses to CN
yc yc yc yc yc yc yc yc yc yc yc yc yc
S, WBCs cross blood brain barrier
yc yc yc yc yc
BMT for first relapse yc yc yc
DownloadedycbyycWamboycJeniyc(
m)
QUESTIONS AND CORRECT ANSWERS
WITH RATIONALE
, PEDS FINAL EXAM REVIEW yc yc yc
PEDIATRIC CANCER yc
Wilms Tumor : Unilateral (more common) or bilateral tumor of kidne
yc yc yc yc yc yc yc yc yc yc
y Most common renal neoplasm in children, good prognosis
yc yc yc yc yc yc yc yc yc
Median age @ dx: 2-3 yrs yc yc yc yc yc
Clinical Presentation yc
Palpable abd. mass in healthy child, vascularized encapsulated jelly-
yc yc yc yc yc yc yc yc
like (RUPTURE), regular borders Some have pain, microscopic or gross hematuria, malaise, fever,
yc yc yc yc yc yc yc yc yc yc yc yc yc
HTN
G/U abnl’s: ambig genitalia cryptorchidism yc yc yc
hypospadius Congenital hem yc yc
ihypertrophy
Aniridia
Assoc.syndrome: Beckwith- yc
Wiedeman Treatment: 80% good prognosis yc yc yc yc
Unilateral: Nephrectomy yc
Vincristine & Actinomycin-D for 6months- yc yc yc yc
15months Bilateral: Bilateral partial resection, if possible yc yc yc yc yc yc
Same therapy with addition of adriamycin yc yc yc yc yc
Remove kidney with largest tumor, abdominal radiation for remaining Che yc yc yc yc yc yc yc yc yc
motherapy complications: yc
Mucositis, hair loss, nausea vomiting, bone marrow depression
yc yc yc yc yc yc yc
NO INFECTIONS SHOULD BE TREATED LIGHTLY
yc yc yc yc yc
Infections are the leading cause of death. yc yc yc yc yc yc
Should avoid crowds or exposure to communicable diseases.
yc yc yc yc yc yc yc
Tumor lysis syndrome: metabolic complication caused by breakdown products of dying cells HYPERURIC
yc yc yc yc yc yc yc yc yc yc yc yc
EMIA, HYPERPHOSPHATEMIA, HYPERKALEMIA, HYPOCALCEMIA yc yc yc
Occurs within 24-48 hours of chemotherapy yc yc yc yc yc
Treatment: hydration to increase urine production, correct electrolyte imbalances
yc yc yc yc yc yc yc yc
Goal: prevent renal failure
yc yc yc yc
Management of Infusion Devices yc yc yc
Most children will have a external central line or implanted venous access device (IVAD or port-a-
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
cath) Guidelines for care are institution specific:
yc yc yc yc yc yc
Rapid, easily obtained venous access yc yc yc yc
Monitor lines for infection (must culture if febrile) M yc yc yc yc yc yc yc yc
aintain patency by heparin flushing q 4 weeks yc yc yc yc yc yc yc
Radiation long term effects: cognitive defects, slow bone growth, growth hormone deficiency, dental problems
yc yc yc yc yc yc yc yc yc yc yc yc yc
Brain Tumors: Most common solid tumor, 2nd only to leukemia among childhood cancers (17% of all childhood CA).
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
2200 new dx in US/yr. yc yc yc yc
Classification system is difficult due to lack of a universally accepted grading syste
yc yc yc yc yc yc yc yc yc yc yc yc
m. 5 year survival ranges 55-75%.
yc yc yc yc yc
Signs & Symptoms: yc yc
Headaches: Worse in am or nocturnal yc yc yc yc yc
Vomiting: Without nausea
yc yc yc
Increased ICP: yc
Personality changes yc
Drowsiness yc
Resistance to being held yc yc yc
Increased FOC: Delayed closure or elevation of anterior fontan yc yc yc yc yc yc yc yc
el Loss of developmental milestones
yc yc yc yc
Papilledema yc
Head tilt yc
Abnormal reflexes and speech yc yc yc yc
Cranial nerve abnormalities yc yc
Presentation varies with site, age, developmental level 6 yc yc yc yc yc yc yc
0% infratentorial (cerebellum or brain stem)
yc yc yc yc yc
Diploplia
Gait disturbance Gait ataxia, truncal ataxia Nystag yc yc yc yc yc yc
mus
Weakness
40% supratentorial (cerebral hemispheres, hypothalamus, thalamus, optic chiasm, pineal region) Sei
yc yc yc yc yc yc yc yc yc yc
zures
DownloadedycbyycWamboycJeniyc(
m)
, Headaches
Infants: Irritability, Listlessness, Vomiting
yc yc yc
DownloadedycbyycWamboycJeniyc(
m)
, Diagnosis: Head CT or MRI yc yc yc yc
Treatment: Surgery (complete resection is ideal)
yc yc yc yc yc
Radiation: effective, can not be used on child <4 due to effects on brain Chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc yc
therapy: resistant yc
LEUKEMIA (ALL (best), CML (teens), AML (worst prognosis), T-cell ALL)
yc yc yc yc yc yc yc yc yc
ALL - Acute Lymphoblastic Leukemia (lymphocyte)
yc yc yc yc yc
Most common childhood cancer (<15 yrs.) 90% cure rat
yc yc yc yc yc yc yc yc
e Accounts for 75% of all childhood CA
yc yc yc yc yc yc yc
5-
year relative survival with ALL diagnosed in 2000 is near 80%
yc yc yc yc yc yc yc yc yc yc yc
Males > females (1.2:1) yc yc yc
Peak age 2-3yrs yc yc
Annually: (2,400 cases per year) yc yc yc yc
Downs (trisomy 21) : 1 in 95 (10-20x greater)
yc better prognosis and cure rate, require lighter chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc yc
??? Etiology ??? yc yc
? Infection yc
? Electromagnetic field exposure yc yc yc
? Exposure during pregnancy yc yc yc
? Radiation yc
? Chemical or drug exposure yc yc yc yc
? Familial predisposition yc yc
? Chromosomal aberrations yc yc
T cell ALL -
yc yc yc
thymus, anterior mediastinal mass, deviated trachea Clinical Presen
yc yc yc yc yc yc yc yc
tation of Leukemia
yc yc
Leukocytosis/leukopenia
Neutropenia- absolute neutrophil count 3,000-5,000 yc yc yc yc
neutropenic if <1000 yc yc
Lymphoblasts on peripheral smear
yc yc yc yc
Anemia: usually present at dx, RBCs lysed by overabundance of WBCs
yc yc yc yc yc yc yc yc yc yc
Thrombocytopenia (75%) petechiae (does not blanch), purpura, mucosal (nasal/scleral) bleeding, (he yc yc yc yc yc yc yc yc yc yc
maturia & GI hemorrhage are rare) yc yc yc yc yc
Fever (60%): frequently associated with infection
yc yc yc y c yc
Pain in bones/joints (23%): due to bone marrow infiltration by leukemic cells (also called blasts) Ano
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
rexia (common) & abdominal pain
yc yc yc yc
Weight loss (infrequent) yc yc
Pallor and mucosal bleeding (48%)
yc yc yc yc
Lymphadenopathy(50%): Clavicular and epitrochlear nodes worrisome yc yc yc yc yc
Splenomegaly (63%) yc
Hepatosplenomegaly (68%) yc
Diagnostic labs NP/LMD’s office
yc yc yc yc
CBC: will give a high index of suspicion
yc yc yc yc yc yc yc
Low hgb, low platelets, high or low WBC count yc yc yc yc yc yc yc yc
Serum chemistry: uric acid elevation due to rapid turnover of malignant cells leads to uric acid nephropat
yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc yc
hy LDH - frequently elevated
yc yc yc yc
Lytes - especially if dehydrated
yc yc yc yc
Bone marrow aspirate/biopsy: should be done at tertiary care site, ONLY DIFINITIVE DIAGNOSTI
yc yc yc yc yc yc yc yc yc yc yc yc
C Lumbar puncture: Rule out CNS disease
yc yc yc yc yc yc
Tumor markers, DNA testing, special stains Fl
yc yc yc yc yc yc
ow cytometry yc
Cytogenetics
ALL Leukemia Tmtt: Multi-
yc yc yc
agent chemotherapy, 90% complete remission in 29 days (0.01% leukemia cells) Intrathecal chemo
yc yc yc yc yc yc yc yc yc yc yc yc yc
through spinal tap (maintain trendelenburg to coat brain, remove CSF prior to)
yc yc yc yc yc yc yc yc yc yc yc
3-Phases (approx.. 2 yr. Duration): yc yc yc yc
Induction-
introduce chemo, hospitalized Consolidation-
yc yc yc yc
CNS chemo, weekly spinal taps
yc yc yc yc yc
Maintenance- beginning at 6- yc yc yc
8 months, hair returns, tolerable, monthly visits Will get radiation if relapses to CN
yc yc yc yc yc yc yc yc yc yc yc yc yc
S, WBCs cross blood brain barrier
yc yc yc yc yc
BMT for first relapse yc yc yc
DownloadedycbyycWamboycJeniyc(
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