CPHON Late Effects of Childhood Cancer Treatment 1 — Questions and Answers
Question 1: A childhood cancer survivor who received anthracycline chemotherapy is at greatest risk for which late effect?
- Cardiotoxicity and cardiomyopathy (Correct answer)
- Hepatic fibrosis
- Pulmonary hypertension
- Renal tubular acidosis
Correct answer: Cardiotoxicity and cardiomyopathy
Anthracyclines (doxorubicin, daunorubicin) cause cumulative dose-dependent cardiotoxicity, leading to cardiomyopathy and congestive heart failure as a significant late effect in childhood cancer survivors.
Anthracycline chemotherapy agents cause irreversible damage to cardiac myocytes through free radical generation and topoisomerase II inhibition. Cumulative doses above 300 mg/m² significantly increase the risk of cardiomyopathy, left ventricular dysfunction, and congestive heart failure. Late cardiac effects may not manifest for years to decades after treatment. Long-term surveillance with echocardiography is recommended for all survivors who received anthracyclines.
Question 2: Which childhood cancer treatment is most closely associated with the late effect of secondary leukemia?
- Alkylating agents such as cyclophosphamide (Correct answer)
- Vinca alkaloids such as vincristine
- Corticosteroids such as dexamethasone
- Antimetabolites such as cytarabine
Correct answer: Alkylating agents such as cyclophosphamide
Alkylating agents are associated with therapy-related myelodysplastic syndrome (t-MDS) and secondary acute myeloid leukemia (t-AML), typically occurring 5–10 years after initial treatment.
Alkylating agents, including cyclophosphamide, melphalan, and busulfan, cross-link DNA strands and can cause mutations in hematopoietic stem cells. These mutations may lead to therapy-related myelodysplastic syndrome or secondary AML, typically appearing 5–10 years post-treatment. Risk is cumulative-dose dependent. Survivors should have ongoing blood count monitoring as part of survivorship surveillance.
Question 3: A 16-year-old survivor of childhood ALL who received cranial radiation reports difficulty concentrating and declining academic performance. This is most consistent with which late effect?
- Neurocognitive impairment (Correct answer)
- Posterior fossa syndrome
- Cerebral vasculopathy
- Peripheral neuropathy
Correct answer: Neurocognitive impairment
Cranial radiation disrupts developing white matter and neural circuitry, leading to neurocognitive late effects including attention difficulties, processing speed deficits, and academic challenges—particularly in children treated at younger ages.
Cranial radiation therapy, once standard in pediatric ALL treatment, is now largely replaced by CNS-directed chemotherapy due to its significant neurocognitive late effects. Damage to white matter, especially in younger children whose brains are still developing, leads to deficits in attention, memory, processing speed, and executive function. Formal neuropsychological testing and school support are key components of survivorship care. Younger age at treatment, female sex, and higher radiation dose are risk factors.
Question 4: Which endocrine late effect is most commonly seen in children who received total body irradiation (TBI) as part of a stem cell transplant conditioning regimen?
- Growth hormone deficiency (Correct answer)
- Hyperparathyroidism
- Cushing syndrome
- Primary hyperaldosteronism
Correct answer: Growth hormone deficiency
TBI damages the hypothalamic-pituitary axis, most commonly affecting growth hormone secretion. Growth hormone deficiency is the most frequent endocrine late effect, leading to short stature if untreated.
Total body irradiation damages the hypothalamus and pituitary gland, disrupting the secretion of multiple hormones. Growth hormone deficiency is the most common endocrine sequela, resulting in growth failure and short stature. Other affected hormones may include thyroid-stimulating hormone, gonadotropins (leading to early or delayed puberty), and ACTH. Regular monitoring of growth velocity and hormone levels is essential in TBI survivors.
Question 5: A survivor of Wilms tumor who received flank radiation is at risk for which musculoskeletal late effect on the irradiated side?
- Scoliosis and spinal asymmetry (Correct answer)
- Avascular necrosis of the femoral head
- Osteoporosis and fragility fractures
- Muscle hypertrophy
Correct answer: Scoliosis and spinal asymmetry
Radiation to the flank in young children damages vertebral growth plates asymmetrically, leading to scoliosis and spinal curvature as bone on the irradiated side grows less than the contralateral side.
Radiation to the flank or abdomen in pediatric patients can damage the growth plates of vertebral bodies on the irradiated side. Because one side grows less than the other, progressive scoliosis develops. The severity depends on the child's age at treatment (younger = more growth remaining = more deformity) and the radiation dose. Orthopedic surveillance and physical therapy are important in survivorship care. Soft tissue hypoplasia and muscle wasting on the irradiated side also contribute to musculoskeletal asymmetry.
Question 6: Which late effect should a nurse specifically assess for in a female survivor of childhood cancer who received gonadotoxic treatment before puberty?
- Premature ovarian insufficiency and infertility (Correct answer)
- Polycystic ovarian syndrome
- Endometriosis
- Uterine fibroids
Correct answer: Premature ovarian insufficiency and infertility
Alkylating agents and pelvic/gonadal radiation destroy primordial follicles, leading to premature ovarian insufficiency (POI), absent or delayed puberty, and infertility.
Premature ovarian insufficiency (POI) is a significant late effect for female survivors who received high-dose alkylating agents or gonadal radiation. Follicle destruction reduces ovarian reserve and can cause absent puberty, amenorrhea, estrogen deficiency symptoms (hot flashes, vaginal dryness, osteoporosis risk), and infertility. Fertility preservation counseling before treatment is essential when possible. Long-term endocrine follow-up with hormone replacement therapy may be needed.
CPHON Late Effects of Childhood Cancer Treatment 1
A childhood cancer survivor who received anthracycline chemotherapy is at greatest risk for which late effect?