Long-term follow-up and late effects of childhood cancer: SAQ

This young woman is a childhood cancer survivor at the age of twenty-two, fifteen years after the treatment of a Hodgkin lymphoma with a high cumulative anthracycline dose and a significant chest radiation exposure. Her exercise intolerance is the symptom that demands the assessment for the anthracycline cardiomyopathy, and her treatment exposures demand the comprehensive risk-stratified surveillance across the cardiac, the endocrine and the oncologic domains. The task is to recognise the late effects, to order the surveillance, and to build the transition plan. [5]

Question 1 (10 marks)

Outline your approach to the risk-stratified assessment and the surveillance for this survivor's late effects. [2]

A full-mark answer covers the treatment-summary-driven surveillance, the targeted history and examination, and the organ-system investigations mapped to the specific exposures. [1]

The treatment summary and the risk stratification (2 marks). The assessment begins with the treatment summary, which documents the diagnosis, the cumulative anthracycline dose of three hundred milligrams per square metre, the chest radiation of thirty gray, and the other exposures. The summary is mapped onto the Children's Oncology Group guidelines to generate the risk-stratified surveillance plan. The anthracycline dose places her in the high-risk group for the cardiomyopathy, because it exceeds the two hundred and fifty milligrams per square metre threshold, and the chest radiation places her in the high-risk group for the breast cancer, because it exceeds the twenty gray threshold. [2]

The targeted history and examination (2 marks). The history probes the exercise tolerance, the dyspnoea and the palpitations for the cardiomyopathy, the menstrual and the fertility history for the gonadal failure, and the cognitive and the mood status for the neurocognitive late effect. The examination measures the height and the weight, palpates the thyroid, examines the breasts, auscultates the heart for the gallop, and examines the skin. The exercise intolerance is the symptom that directs the urgent cardiac assessment. [3][5]

The cardiac investigation (3 marks). The urgent echocardiogram is the key test, because the exercise intolerance in a survivor with a cumulative anthracycline dose of three hundred milligrams per square metre may be the clinical declaration of the cardiomyopathy. The echocardiogram measures the left ventricular ejection fraction and the shortening fraction, and the electrocardiogram looks for the arrhythmia. The troponin and the natriuretic peptide are the adjuncts, and the cardiac magnetic resonance imaging is considered if the echocardiogram is discordant. The cardio-oncology team is involved early. [5]

The endocrine and the oncologic surveillance (3 marks). The endocrine panel includes the thyroid function, the hypothalamic-pituitary hormone panel, and the bone density, because the chest radiation and the chemotherapy carry the endocrine late-effect risk. The breast surveillance is the annual mammography and the breast magnetic resonance imaging, beginning at eight years after the radiation or at age twenty five, whichever occurs later, and because she is twenty-two and fifteen years post-radiation she has already entered the surveillance window. The thyroid examination and the ultrasound are performed for the thyroid cancer risk, and the neuropsychological assessment is considered for the cognitive late effect. [3][9]

Question 2 (10 marks)

Discuss the definitive management across the cardiac, endocrine and oncologic domains, and the transition to the adult late-effects service. [5]

A full-mark answer reproduces the management of each late-effect domain and the structured transition, and it cites the guidelines. [2]

The cardiac management (4 marks). If the echocardiogram shows the falling ejection fraction or the subclinical dysfunction, the angiotensin-converting-enzyme inhibitor or the angiotensin-receptor blocker is started to reduce the afterload and to slow the progression, and the survivor is referred to the cardio-oncology service. If she has the decompensated heart failure, the standard heart failure therapy is delivered, with the diuretics, the afterload reduction and the inotrope, and the heart transplantation is the option for the end-stage cardiomyopathy. The avoidance of the additional cardiotoxicity, the blood-pressure control and the lifestyle form the preventive layer, and the lifelong echocardiographic surveillance continues. [5]

The endocrine management (3 marks). The growth hormone deficiency, if present, is treated with the recombinant growth hormone with the oncology clearance. The hypothyroidism is treated with the levothyroxine. The gonadal failure is treated with the sex-steroid replacement for the bone health and the reproductive function, and the fertility is addressed with the reproductive medicine referral. The adrenal insufficiency, if present, is treated with the hydrocortisone and the sick-day plan, and the survivor is taught the stress-dose hydrocortisone and the emergency intramuscular injection. [3]

The oncologic surveillance and the transition (3 marks). The breast surveillance is the annual mammography and the breast magnetic resonance imaging, and any abnormality is referred to the breast service. The thyroid surveillance continues annually. The structured transition to the adult late-effects service is prepared, with the survivor taught the diagnosis, the exposures, the surveillance plan and the late-effect symptoms, and the written treatment summary and the care plan are handed to the named adult provider. The fellow who builds the transition plan and the patient-held summary is the one who keeps the survivor in the lifelong care. [9][11]