Pediatric Oncology

Question 1

_____ is the most common childhood malignancy followed by ____ and ____.

Answer 1

ALL followed by CNS tumors and lymphomas.

Question 2

ALL accounts for approx. ___ % of all childhood leukemias.

Answer 2

~97%

Question 3

ALL is a/w ____ disorders/conditions.

Answer 3

-Down syndrome (trisomy 21)

-Fanconi anemia

-Prior radiation
-SCID

-congenital bone marrow failure states (Diamond-Blackfan anemia etc.)

Question 4

ALL in children has a good prognosis with a 5-year survival rate of ___ %.

Answer 4

> 85%

Question 5

____ demographic groups have the highest incidence of ALL.

Answer 5

non-hispanic white male children between 2-5 years of age.

Question 6

AML is most commonly seen in ___ demographic groups.

Answer 6

black boys throughout childhood.

Question 7

Symptoms of ALL are ____ (? gradual, abrupt) in onset.

Answer 7

abrupt

Question 8

Clinical presentation of acute leukemia (ALL, AML) is marked by abrupt onset of ______ s/s (list most).

Answer 8

-Initial non-specific: anorexia, fatigue, weight loss.

-Bone pain with a limp/refusal to bear weight.
-Fever d/t Neutropenia,
-Pallor d/t anemia,
-Ecchymoses, petechiae d/t thrombocytopenia,

and/or
-hepatosplenomegaly
-Lymphadenopathy.

Question 9

Acute leukemias are characterized by greater than ____ % blasts in the peripheral blood smear or on bone marrow leading to a more rapid onset of symptoms.
In contrast, chronic leukemia has less than _____ % blasts with a relatively chronic onset of symptoms.

Answer 9

Acute leukemias > 20% blasts in the PBS/BM;

Chronic leukemia < 20% blasts in PBS/BM.

Question 10

The accelerated/blast phase is a transformation of ____ leukemia into an acute phase with a significantly higher degree of blasts.

Answer 10

The accelerated/blast phase is a transformation of CML into an acute phase with a significantly higher degree of blasts.

Question 11

True/False?

The WBC count is usually very high in leukemia.

Answer 11

False;

WBC count may be low, normal, or high in leukemia.

Question 12

Tumor lysis syndrome is marked by ____ electrolyte disturbances.

Answer 12

MN: PUKE Calcium

P: Hyperphosphatemia
U: Hyperuricemia
K: Hyperkalemia

C: HYPOcalcemia (as phosphate released from lysed tumor cells binds with calcium).

Question 13

What are the life-threatening sequelae of electrolyte imbalances seen in tumor lysis syndrome?

Answer 13

-acute renal failure
-arrhythmias
-death

Question 14

____ pharmaceutical agents can precipitate tumor lysis syndrome.

Answer 14

Corticosteroids

Question 15

______ can be used to reduce the risk of hyperuricemia- aka urate-induced nephropathy in patients undergoing chemotherapeutic treatment for leukemia.

Answer 15

Rasburicase
*rapidly lowers existing hyperuricemia.

Question 16

_____ can be used as a hyperuricemia preventative agent in leukemia patients starting chemotherapy.

Answer 16

Allopurinol

Question 17

____ is an embryonal tumor of neural crest origin, similar to a pheochromocytoma.

Answer 17

neuroblastoma

Question 18

Neuroblastoma most commonly originates in _____ anatomic location, followed by ___, ____, and ____ locations in descending order of occurrence.

Answer 18

Abdominal (adrenal medulla f/by
paraspinal sympathetic chain),

f/by thoracic, and cervical sympathetic chain (p/with Horner syndrome).

Question 19

True/False?

Over 50% of patients with neuroblastoma present at age under 2 years.

Answer 19

True.

Question 20

More than ___ % of patients with neuroblastoma have metastasis at diagnosis.

Answer 20

> 50%

Question 21

What are some s/s suggestive of metastasis in patients diagnosed with neuroblastoma?

Answer 21

-BM suppression: Anemia, fever, pancytopenia.
-Proptosis
-Hepatomegaly
-Subcutaneous nodules
-Opsoclonus-Myoclonus (Dancing eyes, dancing feet syndrome).

Question 22

Abdominal neuroblastoma may p/with ____ s/s.

Answer 22

-abdominal distension with a firm, irregular mass that crosses the midline.
-HTN
-spinal cord compression from para-spinal tumor.

-Opsoclonus-Myoclonus (Dancing eyes, dancing feet syndrome).

Question 23

Histologically, neuroblastoma presents as ____.

Answer 23

small, round blue cells with a characteristic rosette pattern that are Bombesin +ve.

Question 24

Diagnosis of a neuroblastoma entails _____ tests and imaging.

Answer 24

-Lab: 24-hour urinary catecholamines (VMA and homovanillic acid).
-CT scan/ MRI for tumor ascertainment
-MIBG Scan and BM aspirate for staging.

Question 25

____ is a renal tumor of embryonal origin from the renal _____, most commonly seen in children aged ____ years.

Answer 25

Wilms tumor;
originates from the metanephros (embryonal tissue);

children between 2-5 years of age.

Question 26

List some associations of Wilms tumor.

Answer 26

1. WAGR syndrome: Wilms tumor, Aniridia, GU abnormalities, retardation (mental).
2. Beckwith-Wiedemann syndrome: hemihypertrophy, visceromegaly, macroglossia.

Question 27

____ and ____ are some known risk factors for Wilms tumor.

Answer 27

Family history, and horse-shoe kidney.

Question 28

Wilms tumor typically presents as an asymptomatic, smooth abdominal mass that ____ (crosses, does not cross) the midline.

Answer 28

does not cross the midline

*unlike neuroblastoma in children < 2 yrs old which is a firm irregular mass that often crosses the midline.

Question 29

Other than a smooth abdominal mass what s/s may be a/with Wilms tumor?

Answer 29

-Abdominal pain
-fever
-weight loss
-HTN
-microscopic or gross hematuria.

Question 30

_____ is the best initial test in a suspected case of Wilms tumor.

Answer 30

Abdominal USG

Question 31

_______ test is needed for a definitive diagnosis of Wilms tumor.

Answer 31

Biopsy

Question 32

CT scan of the chest and abdomen is indicated to detect ____ in a patient with Wilms tumor.

Answer 32

distant metastasis

Question 33

A “sunburst” lytic bone lesion in the distal femur, with elevated alkaline phosphatase levels in a 14-year-old boy who p/with pain and swelling in the lower right thigh and no systemic symptoms is highly suggestive of _____.

Answer 33

Osteosarcoma
See the attached image for a “sunburst” type periosteal reaction.

Image Source: https://radiopaedia.org/cases/osteogenic-sarcoma-2

Question 34

Osteosarcoma is a malignant tumor of ____ origin, whereas Ewings sarcoma originates from _____.

Answer 34

Osteosarcoma: osteoblasts (mesenchyme) origin;

Ewings sarcoma originates from the neuroectoderm.

Question 35

____ malignant bone tumor is a/w chromosomal translocation 11:22.

Answer 35

Ewing sarcoma (neuroectoderm origin).

Question 36

Osteosarcoma is most commonly seen in ____ demographic groups.

Answer 36

Hispanic or white male adolescents with the peak incidence between the ages of 13-16 years.

Question 37

Ewing sarcoma is most commonly seen in ____ demographic groups.

Answer 37

White males between 10-20 years of age.

Question 38

Systemic symptoms such as fever, anorexia, and fatigue are rarely seen with ____ type of malignant bone tumor, whereas the same are commonly seen in patients with ____ bone tumor.

Answer 38

Osteosarcoma: systemic s/s are rare;

Ewing sarcoma: systemic s/s such as fever, anorexia, fatigue.

Question 39

Ewing sarcoma typically develops in ____ location on long bones.

Answer 39

diaphysis of long bones such as femur, pelvis, fibula, humerus.

Question 40

Metastasis to the lungs can be seen in ~ 20% of cases of ____ bone tumor (? osteosarcoma, Ewing sarcoma).

Answer 40

Osteosarcoma

Question 41

_____ triangle formed as a result of an aggressive periosteal reaction in patients with osteogenic sarcoma may be visible on X-ray.

Answer 41

Codman triangle (see attached image).

Image Source: https://radiopaedia.org/articles/codman-triangle-periosteal-reaction

Question 42

What imaging studies are indicated in a patient diagnosed with osteogenic sarcoma?

Answer 42

1. Plain X-ray of the affected bone
2. MRI of the entire length of the affected bone.
3. CT chest: for pulm. metastasis.
4. Radionuclide Tc bone scanning to assess the rest of the skeleton for bone lesions.

Question 43

Multilayered periosteal reaction aka a “lamellated” or “onion skin” periosteal reaction is typically seen in _____ bone tumor.

Answer 43

Ewing Sarcoma.

Question 44

Leukocytosis and elevated ESR may be seen in ____ bone tumor.

Answer 44

Ewing Sarcoma.