EXAM #3: CHILDHOOD CANCER II

Question 1

What is Acute Lymphoblastic Leukemia?

Answer 1

Hyperproliferation of lymphoid progenitor

Question 2

What gender is ALL more common in?

Answer 2

Males

Question 3

What are the signs and symptoms of Luekemia?

Answer 3

Constellation of symptoms associated with:

1) Anemia
2) Thrombocytopenia
3) Neutropenia

AND bone pain that causes a limp/decreased walking

Question 4

What luekemia is gingival hypertrophy associated with?

Answer 4

Monoblastic leukemia

Question 5

What are the CBC findings that are diagnostic for Leukemia?

Answer 5

1) Increased WBC
2) Lymphoblasts on PBS
3) 2+ cytopenias

Question 6

What should be included in the differential for Leukemia?

Answer 6

• EBV (Mono)
• Acute anemia
• Parvovirus B19
• ITP

Question 7

Kids with what genetic abnormality are at an increased risk for ALL?

Answer 7

Down’s Syndrome

Question 8

What are the nongenetic risk factors for ALL?

Answer 8

X-rays/ radiation

Question 9

What is the most common type of ALL?

Answer 9

B-ALL

Question 10

What is the most common involvement of B-ALL?

Answer 10

Extramedullary

Question 11

What group of children is T ALL more common in?

Answer 11

Male adolescents

Question 12

What is the presentation of T-ALL?

Answer 12

• Mediastinal mass
• High WBC mass
• LAD that resembles Mumps

Question 13

What is the greatest risk factor in B-ALL?

Answer 13

Response to induction therapy in the first 28 days

Question 14

What is the most common solid tumor of childhood?

Answer 14

Brain tumors

Question 15

What is the most common brain tumor of childhood?

Answer 15

Astrocytomas

Note that this is specifically a low grade JPA

Question 16

Where is the most common location for an astrocytoma?

Answer 16

Posterior fossa

Question 17

What is the standard of care for an astrocytoma?

Answer 17

Total resection i.e. neurosurgery

Question 18

What are the signs and symptoms of a brain tumor?

Answer 18

• Neuro/ increased ICP signs/sx

- Regression of milestones

Question 19

Why is chemotherapy relatively ineffective in treating astrocytomas?

Answer 19

• Low grade means less proliferation

- Slow growing tumors are hard to target with chemotherapy

Question 20

Radiation has a potential for use in all tumors but which one?

Answer 20

Choroid plexus tumors

Question 21

What are the long-term effects of radiation therapy in children for brain tumors?

Answer 21

1) Neuro-cognitive impairment
2) Hearing changes
3) Secondary cancer
4) Endocrine abnormalities
5) Skeletal growth abnormalities

Question 22

What disease are astrocytomas most commonly associated with? What is second?

Answer 22

1) NF-1

2) Li-Fraumeni Syndrome

Question 23

What is a Wilm’s tumor?

Answer 23

Renal tumor that is the 2nd most common type of abdominal malignancy in children

Question 24

What gene is mutated in Wilm’s tumors?

Answer 24

WT1 tumor suppressor mutation

Question 25

What race are Wilm's tumors more common in?

Answer 25

African Americans

Question 26

What are the symptoms associated with Wilm's Tumor?

Answer 26

- HTN - Hematuria - Fever

Question 27

Describe the etiology of Retinoblastoma.

Answer 27

Loss of both Rb alleles - 60% are spontaneous - 40% are hereditary

Question 28

Where are neuroblastomas located? Where are the common metastases of neruoblastomas located at diagnosis?

Answer 28

Any neural crest tissue for primary tumor i.e. - Adrenal tissue - Parasympathetic tissue (cerivical, thoracic, pelvic) Mets: bone, lymph nodes, skin, liver

Question 29

What are the signs and symptoms of a neuroblastoma?

Answer 29

- Proptosis - Bone lesions - Periorbital ecchymoses i.e. "Racoon eyes"

Question 30

How do neuroblastomas commonly present?

Answer 30

1) Large abdominal mass that crosses the midline 2) LE weakness 3) Cervical or high thoracic mass causing Horner's Syndrome

Question 31

What is the average age of onset for a neuroblastoma?

Answer 31

18 months

Question 32

What is the most common extracranial solid tumor in children?

Answer 32

Neuroblastoma

Question 33

What is the most common type of soft tissue sarcoma?

Answer 33

Rhabdomyosarcoma

Question 34

What are the signs and symptoms of a Rhabdomyosarcoma involving the head and neck?

Answer 34

Proptosis | Periorbital swelling

Question 35

What are the signs and symptoms of a parameningeal rhabdomyosarcoma?

Answer 35

1) CN palsy 2) Hearing loss 3) Chronic aural or sinus drainage

Question 36

What are the signs and symptoms of a male GU rhabdomyosarcoma?

Answer 36

- Hematuria | - Urinary obstruction

Question 37

What are the signs and symptoms of a paratesticular rhabdomyosarcoma?

Answer 37

Pain testicular mass

Question 38

What are the signs and symptoms of a vaginal/uteral rhabdomyosarcoma?

Answer 38

1) Abdominal mass 2) Vaginal mass 3) Vaginal bleeding or discharge

Question 39

What is a Rhabdomyosarcoma?

Answer 39

Undifferentiated mesenchymal cells that differentiate into muscle

Question 40

At what age do Ewing's Sarcomas peak?

Answer 40

11-15 years old

Question 41

How do Ewing's Sarcomas present?

Answer 41

Limb pain and swelling, frequently after a sports related injury ****More common in boys*****

Question 42

Where do osteosarcomas typically present?

Answer 42

Long bones metaphyses (wide part near growth plate): - Distal femur - Proximal tibia - Proximal humerus - Pelvis

Question 43

Where do Ewing Sarcoma's typically present?

Answer 43

Long bone diaphyses (narrow part) | Flat bones