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The Hypothalamic–Pituitary–Adrenal Axis in Childhood Cancer Survivors

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The Hypothalamic–Pituitary–Adrenal Axis in Childhood Cancer Survivors 25 10 Endocrine-Related C Wei and E C Crowne HPAA in childhood cancer 25:10 R479–R496 Cancer survivors REVIEW The hypothalamic–pituitary–adrenal axis in childhood cancer survivors Christina Wei1 and Elizabeth C Crowne2 1St George’s University Hospital, NHS Foundation Trust, London, UK 2Bristol Royal Hospital for Children, University Hospitals Bristol, NHS Foundation Trust, Bristol, UK Correspondence should be addressed to E C Crowne: [email protected] Abstract Endocrine abnormalities are common among childhood cancer survivors. Abnormalities Key Words of the hypothalamic–pituitary–adrenal axis (HPAA) are relatively less common, f ACTH but the consequences are severe if missed. Patients with tumours located and/or f cortisol had surgery performed near the hypothalamic–pituitary region and those treated f HPA axis with an accumulative cranial radiotherapy dose of over 30 Gy are most at risk of f radiotherapy adrenocorticotrophic hormone (ACTH) deficiency. Primary adrenal insufficiency may occur in patients with tumours located in or involving one or both adrenals. The effects of adjunct therapies also need to be considered, particularly, new immunotherapies. High-dose and/or prolonged courses of glucocorticoid treatment can result in secondary adrenal insufficiency, which may take months to resolve and hence reassessment is important to ensure patients are not left on long-term replacement steroids inappropriately. The prevalence and cumulative incidences of HPAA dysfunction are difficult to quantify because of its non-specific presentation and lack of consensus regarding its investigations. The insulin tolerance test remains the gold standard for the diagnosis of central cortisol deficiency, but due to its risks, alternative methods with reduced diagnostic sensitivities are often used and must be interpreted with caution. ACTH deficiency may develop many years after the completion of oncological treatment alongside other pituitary hormone deficiencies. It is essential that health professionals involved in the long-term follow-up of childhood cancer survivors are aware of individuals at risk of developing HPAA dysfunction and implement appropriate Endocrine-Related Cancer monitoring and treatment. (2018) 25, R479–R496 Introduction Improvement in paediatric oncology treatment and support cancer survivor (Campbell et al. 2004, Oeffingeret al. 2006). have overseen the dramatic increase in overall 5-year Unfortunately, survival comes with a cost, and there is survival rates of all childhood cancers from less than 30% ample evidence that childhood cancer survivors experience in the 1960s to the current >80% in the United Kingdom adverse physical, psychological and social health problems (https://www.cancerresearchuk.org/health-professional/ later in life, as well as premature mortality. Data from cancer-statistics/childrens-cancers/long-term-follow- the United States, United Kingdom and Northern Europe up#heading-Two). An estimated 1 in 640–700 young adults have demonstrated a high relative risk of death with a in the United States and United Kingdom is a childhood standardised mortality rate of 8.3–10.8 in childhood cancer http://erc.endocrinology-journals.org © 2018 Society for Endocrinology https://doi.org/10.1530/ERC-18-0217 Published by Bioscientifica Ltd. Printed in Great Britain Downloaded from Bioscientifica.com at 09/25/2021 11:58:02AM via free access -18-0217 Endocrine-Related C Wei and E C Crowne HPAA in childhood cancer 25:10 R480 Cancer survivors survivors (Oeffinger et al. 2006, Mertens et al. 2008, Diller ο Type of radiotherapy et al. 2009, Reulen et al. 2010, Garwicz et al. 2012). About • Chemotherapy three-quarters of patients were reported to have at least one • Glucocorticoid therapy chronic health condition and over two-fifths were affected • Immunotherapy by severe, disabling or life-threatening conditions or have • Oncology treatment and autoimmune diseases died from chronic conditions within 30 years of diagnosis 3. Patient factors (Oeffingeret al. 2006). • Assessment of HPAA in childhood cancer In the United Kingdom, approximately 1800 new cases survivors of cancer are diagnosed in children per year. Leukaemia • Relationship between HPAA function and wider is the most common childhood malignancy, followed aspects of health by intracranial central nervous system (CNS) tumours • Future directions and lymphoma. Other malignant conditions specific to children include neuroblastoma and nephroblastoma Risk factors for HPAA dysfunction (Wilms’ Tumour). As the population of childhood cancer survivors increases, emphasis on the management of Table 1 summarises key publications with data concerning the long-term health becomes more important with HPAA dysfunction in childhood cancer survivors. The aims to prevent early morbidity and mortality. Around relevant studies are categorised according to the location of 50% of childhood cancer survivors develop one or more the primary tumour. However, the majority of the studies endocrine disorders with a higher risk among those are limited by heterogeneity in patient characteristics treated for tumours of the CNS and the prevalence such as the primary diagnosis, treatment modalities, age increases over time (Patterson et al. 2012, Brignardello of treatment and follow-up time post treatment, as well et al. 2013, Mostoufi-Moab et al. 2016). Abnormalities as the method of assessment and definition of HPAA of the hypothalamic–pituitary–adrenal axis (HPAA) are dysfunction. relatively less common in childhood cancer survivors compared with other endocrine disorders such as growth The primary diagnosis: location and type of cancer hormone deficiency, gonadal failure and hypothyroidism, but there can be a significant impact on morbidity and Unsurprisingly, tumours within or close to the HP region, mortality risk (Rose et al. 2004). Under physiological and those of the adrenal glands can have a direct impact stress, undiagnosed cortisol deficiency may present with on HPAA function either as a result of the primary tumour life-threatening adrenal crisis. On a day-to-day basis, or its treatment. patients with adrenal insufficiency can experience subtle and non-specific symptoms such as fatigue, which are CNS tumours at the HP region easily dismissed and result in a reduced quality of life. Intracranial tumours involving the HP region either This review provides an overview of risk factors, directly or in its vicinity, such as the optic pathway, surveillance methods and future directions needed in may result in central HPAA dysfunction at diagnosis as the research of HPAA dysfunction in childhood cancer a result of local damage and/or after neurosurgery, with survivors under the following headings: potentials for evolving effects over time from possible Risk factors for HPAA dysfunction: further tumour growth or after cranial irradiation (Fig. 1). 1. The primary diagnosis: location and type of cancer Tumours at the suprasellar and intrasellar regions account • CNS tumours at the hypothalamic–pituitary (HP) for 10% of all CNS tumours in childhood (Arora et al. region 2009). Abnormalities in endocrine function are often • Adrenal tumours already present at diagnosis (Tan et al. 2017). The most 2. Treatment modality common forms of childhood HP tumours are gliomas and • Radiotherapy craniopharyngiomas. ο Accumulative dose and fractionation schedule Gliomas – tumours derived from glial cells – are ο Location – cranial irradiation to tumours in classified according to grade (low and high) and HP region and its proximity location. There is limited research distinguishing long- ο Location – cranial irradiation for malignancies term sequelae caused by the tumour location from its not specifically involving the HP region or its treatment. Low-grade brain tumours such as HP low- proximity grade astrocytoma may have an indolent course for many http://erc.endocrinology-journals.org © 2018 Society for Endocrinology https://doi.org/10.1530/ERC-18-0217 Published by Bioscientifica Ltd. Printed in Great Britain Downloaded from Bioscientifica.com at 09/25/2021 11:58:02AM via free access Endocrine-Related C Wei and E C Crowne HPAA in childhood cancer Cancer survivors https://doi.org/ Table 1 Summary of studies with data on HPAA function in childhood cancer survivors treated with radiotherapy. Patient characteristics and Main outcomes on HPAA Location Reference Primary diagnosis treatment HPAA diagnosis dysfunction 10.1530/ERC I. CNS tumours at Gan et al. (2015) Low-grade gliomas of optic N = 166 (76M) ITT, SSST or LDST • ACTH deficiency: 13.3% HP region and pathway, hypothalamus, Dx: 4.9 (0.2–15.4) year • Hypothalamic involvement vicinity suprasellar FU: 8.3 (0.04–26.8) year implicated more than RT in RT: in all, CI: 48–55 Gy, 25–30# onset of -18-0217 endocrinopathy and RT more of its density II. CNS tumour Clement et al. Low/high-grade glioma, DNET, N = 718 (389M) ACTH deficiency • ACTH deficiency: 4.3%, outside the HP (2016b) medulloblastoma, sPNET, Dx: 7.7 (0–17.7) year definition: ‘use of 1% at diagnosis region ependymoma, choroid plexus FU: 6.6 (2–13.4) year hydrocortisone • Median time of onset = 2.5 tumours, germ cell tumour, ATRT, Age at FU: 15.1 maintenance or (0.01–7.0) year others, without histology (3.0–29.3) year substitution • 5-year cumulative RT: in 35.9% documented’ incidence: 2.9 (0.4–10.6) CI: 54.0 (12.5–60.0) Gy CS: 24.0 (18.0–39.7) Gy Eaton et al. Medulloblastoma Proton vs Photon respectively: 08:00 h cortisol and • Proton RT may reduce risk (2016) N = 40 (21M) vs 37
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