282. Pediatric Cancer

Question 1

Epidemiology of Ped Cancer (how common is it, death rate)

Key differences b/w adult and ped cancer (with age distributions)

Etiology/RFs of childhood cancer

What is the most common childhood malignancy?

Answer 1

Epi: RARE (only 1% new cancer cases annually), but malignant neoplasms leading cause of disease-related death in children

Peds: tumors of developmental origin more common, most common are lymphoheme origin and CNS tumors
- rare for epithelial tumors of lung, breast, prostate, colon
Birth: neuroblastoma, Wilm’s tumor, Retinoblastoma
Early childhood: ALL, NonHL, Glioma
Late Childhood: sarcoma (OS, Ewing, STS), HL, Testicular cancer, Ovarian cancer

Etiology: genetics only account 5%, few enviro/prenatal/pregnancy RFs

Most common: ALL (assoc with Down Syndrome)

Question 2

What are the key genetic RFs for the following:

• ALL
• AML (2)
• Brain Tumor
• Osteosarcoma
• Rhabdomyosarcoma
• Hepatoblastoma
• Wilm’s Tumor (Nephroblastoma)

Answer 2

ALL: Down Syndrome
AML: Down Syndrome, NF1
Brain Tumor: NF2
Osteosarcoma: Li-Fraumeni (p53)
Rhabdo: Li-Fraumeni (p53)
Hepatoblastoma: Beckwith-Wiedemann Syndrome
Wilm’s Tumor: Beckwith-Wiedemann Syndrome, WAGR Syndrome, Denys-Drash Syndrome

Question 3

Pediatric Acute Leukemia

• epi
• types, which is more common
• tx/prognosis based on ages

Answer 3

Epi: most common malignant neoplasm in childhood
ALL (77%) vs. AML (11%)

Infants (<1y): poor prognosis, need SC tx
Adolescents (>10y): need more aggressive tx (but better survival)
Prognosis: overall survival 50-60%

Question 4

Pediatric Lymphoma

• epi
• types

Answer 4

Epi: 3rd most common malignancy in children

HL vs nHL

Question 5

Pediatric CNS Tumors

• epi
• which type has seen improvement in tx over time? which has not?

Answer 5

Epi: 2nd most common malignancy in children, but LEADING cause of cancer related mortality in children
Success: childhood medulloblastoma
Challenge: infant medulloblastoma, high grade glioma

Question 6

Neuroblastoma

• what is it
• age of pt
• epi
• course
• prognosis

Answer 6

• Embryonal cancer of peripheral symp nervous system
• age: younger children
• epi: most common extra-CNS solid tumor in childhood (usually near adrenal gland)
• course: variable - spontaneous resolution to very aggressive and unresponsive to multimodal tx
• prognosis: most have excellent outcomes except for HIGH RISK neuroblastoma: poor survival, need for surgery, chemo, autologous SC tx, mAb immunotx

Question 7

Wilm’s Tumor (nephroblastoma)

• epi
• CP
• prognosis
• assoc

Answer 7

Epi: 2nd most common extra-CNS solid tumor
CP: very large tumor at presentation
prognosis: excellent with use of surgery, chemo, RT (new goal = minimize late effects)
assos: Beckwith-Wiedeman and other predisposition syndromes

Question 8

Hepatoblastoma

• epi
• tx
• RF (4)

Answer 8

Epi: RARE developmental tumor of liver
Tx: chemotx, resection +/- liver tx
RF: PREMATURITY (esp with umbilical vein catheterization), Beckwith-Weidemann syndrome, hemihypertrophy, APC gene mutation

Question 9

Pediatric Sarcoma

• age of pt
• location
• prognosis
• most common soft tissue type and location
• other type that occurs during adolescent growth spurt (dx, tx)
• other type similar to growth spurt type (gene, tx, prognosis)

Answer 9

age: any age, usually adolescents or young adults
location: anywhere with bone and soft tissue
Prognosis: vary by histo, site, mets

Rhabdomyosarcoma: MOST common soft tissue sarcoma: anywhere (H/N, GU tract, extremities)

Osteosarcoma: highest risk during growth spurt (rapid bone growth), dx by biopsy (confirm dx), tx by surgery + chemotx (CURATIVE, but may be amputative, need prosthesis)

Ewings: undifferentiated sarcoma of bone with soft tissue component, t(11;22), tx is multimodal with surgery, chemo, RT (CURATIVE), poor outcomes with metastatic dz

Question 10

Retinoblastoma

• prognosis/epi
• complications
• etiology (2)
• gene

Answer 10

Prognosis: USA extremely high survival; worldwide can progress to metastatic dz and death in over 50% children

Comps: loss of vision and long-term tx adverse effects

Eti

1. Hereditary: younger age at dx, multifocal, bilateral
2. Sporadic: older age, unilateral, unifocal

Gene: loss of function of RB1 via mutation or deletion

Question 11

What is the single greatest factor for increased survival of children with cancer?

Answer 11

Treating children on CLINICAL PROTOCOLS (clinical trials, part of Children’s oncology group - multi-institutional research consortium)