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

Framing the case

This twenty-two-year-old woman is a childhood cancer survivor at the age of fifteen years post-treatment, and her presentation brings together the three highest-yield late effects of the childhood cancer therapy. The cumulative anthracycline dose of three hundred milligrams per square metre places her in the high-risk group for the cardiomyopathy, because it exceeds the two hundred and fifty milligrams per square metre threshold, and her exercise intolerance with the third heart sound is the clinical declaration of the cardiac late effect. The chest radiation of thirty gray places her in the high-risk group for the breast cancer, because it exceeds the twenty gray threshold. The treatment exposures and the symptoms together frame the case as the risk-stratified survivorship assessment, and the first priority is the urgent cardiac assessment. [1][5]

The immediate assessment and the cardiac investigation

The assessment begins with the treatment summary, which documents the diagnosis, the cumulative anthracycline dose, the chest radiation dose and the other exposures. The summary is mapped onto the Children's Oncology Group guidelines to generate the surveillance plan. The urgent echocardiogram is the key test, because the exercise intolerance with the third heart sound in a survivor with the high anthracycline dose is the anthracycline cardiomyopathy until proven otherwise. 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 cardiac management

The echocardiogram confirms the reduced left ventricular ejection fraction, and the management begins at the subclinical dysfunction. The survivor is referred to the cardio-oncology service, and the angiotensin-converting-enzyme inhibitor or the angiotensin-receptor blocker is started to reduce the afterload and to slow the progression. The symptomatic heart failure is managed with the standard therapy, and the end-stage cardiomyopathy may require the heart transplantation, which carries an excellent outcome in the selected survivor. The avoidance of the additional cardiotoxic exposure, the blood-pressure control and the lifestyle form the preventive layer, and the lifelong echocardiographic surveillance continues. The anthracycline cardiomyopathy carries a poorer prognosis than the idiopathic, because the anthracycline injures the terminally differentiated cardiomyocyte irreversibly. [5]

The endocrine and the fertility assessment

The endocrine panel includes the thyroid function, the hypothalamic-pituitary hormone panel and the bone density. The gonadal function is assessed with the luteinising hormone, the follicle-stimulating hormone and the oestradiol, because the alkylating agent and the radiation exposure carry the gonadotoxicity risk. The premature ovarian failure and the infertility are the late effects that the survivor reports as the most distressing, and the fertility is addressed with the reproductive medicine referral. The fertility preservation, the oocyte or the embryo cryopreservation, is offered before any further gonadotoxic therapy, and the reproductive medicine referral is made for the assessment and the counselling. The growth hormone deficiency and the hypothyroidism, if present, are treated with the replacement, and the adrenal insufficiency is treated with the hydrocortisone and the sick-day plan. [3]

The oncologic surveillance

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. Because she is twenty-two and fifteen years post-radiation, she has entered the surveillance window, and the breast magnetic resonance imaging is arranged. The breast tissue of the pubertal girl is exquisitely sensitive to the radiation, so that the chest radiation of thirty gray during the childhood carries the breast cancer risk that approaches that of the BRCA carrier by the age of fifty. The thyroid examination and the ultrasound are performed for the thyroid cancer risk after the neck and the chest radiation, and the annual full blood count is considered for the therapy-related myelodysplasia risk. [9]

Contrasting the second malignancy

The examiner asks the candidate to discuss the risk of the second malignancy. The breast cancer after the chest radiation is the commonest subsequent malignancy in the female survivor, and the surveillance mammography and the magnetic resonance imaging are designed to catch it at the earliest stage. The thyroid cancer after the neck radiation is the second commonest, and the annual thyroid examination and the ultrasound are the surveillance. The central nervous system tumour after the cranial radiation and the therapy-related myelodysplasia after the chemotherapy complete the list, and each carries the surveillance that the treatment summary drives. The second malignancy is the leading cause of the premature death in the survivor, which is why the oncologic surveillance is among the most heavily weighted. [9]

Communication, the transition and the family

The family is counselled honestly and comprehensively. The candidate names the late effects, the surveillance plan and the management for each domain, and she is introduced to the multidisciplinary team. 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 transition is a clinical act, and the fellow who builds the transition plan demonstrates the care that extends the surveillance beyond the paediatric service. The loss to the follow-up is the greatest threat to the late-effect outcome, and the transition plan and the patient-held summary are the interventions that keep the survivor in the lifelong care. [11]