Craniopharyngiomas are common but complex paediatric brain lesions that present interesting management challenges. Quality of life is an important consideration while choosing management options. In this review, we have discussed the existing literature on various aspects of quality of life in patients treated for craniopharyngioma, assessed by variety of measurement tools

Abdullah, Umme Hani

Baqai, Muhammad Waqas Saeed

Gillani, Mishal

Hassan, Sheza

Shamim, Muhammad Shahzad

English

eCommons@AKU

eCommons@AKU Medical College Documents Medical College, Pakistan 11-2020 Quality of life in children treated for craniopharyngiomas Mishal Gillani Sheza Hassan Umme Hani Abdullah Muhammad Waqas Saeed Baqai Muhammad Shahzad Shamim Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_mc  Part of the Neurology Commons, Neurosurgery Commons, and the Oncology Commons 2072Vol. 70, No. 11, November 2020AbstractCraniopharyngiomas are common but complex paediatricbrain lesions that present interesting managementchallenges. Quality of life is an important considerationwhile choosing management options. In this review, wehave discussed the existing literature on various aspects ofquality of life in patients treated for craniopharyngioma,assessed by variety of measurement tools.Keywords: Craniopharyngioma; Parasellar lesion; Sellarlesion; Quality of life; Hypothalamic lesionIntroductionCraniopharyngiomas are rare embryonic malformations ofsellar and parasellar areas of low histological malignancy,thought to be derived from Rathke’s pouch epithelium. Thedisease is treated with surgery and often adjuvanttreatment such as chemotherapy and radiotherapy. Due tothe location of pathology and its proximity to vitalstructures, both the growth of CP as well as the treatment,are associated with high risks for disturbing importantneurological and endocrine functions such as visualimpairment, stroke, obesity, pituitary insufficiency, etc.,thus impairing the quality of life (QoL) in long-termsurvivors. In this review, we have described how the CP andits treatment may affect patients’ QoL despite good diseasecontrol.Review of literatureWe have presented the literature, grouping the variousoutcomes related to childhood onset craniopharyngiomaand its treatment into five major categories; obesity, visualand neurological outcomes, endocrine imbalance,psychological and psychosocial outcomes, and otherdeterminants of QoL. There is a large body of literature onthis topic, and we have only discussed a few publicationsfor each outcome. Some other notable references on thetopic are mentioned in the Table.I. ObesityHypothalamic involvement (HI) either due to the CP, or itstreatment has significant effect on long term prognosis.Childhood onset CPs with HI were associated with overallreduced survival and increased Body Mass Index (BMI) atdiagnosis and follow up when compared with CPs withoutHI. Children with hypothalamic lesion extending into thenuclei of posterior hypothalamus, frequently develophypothalamic obesity (HO) syndrome which ischaracterized by hyperphagia, diminished satiety,decreased physical activity and consequential obesity. HOrisk assessment can be done using hypothalamic lesionscoring (HLS) system which can predict the risk fordeveloping HO and may allow early initiation ofintervention strategies. After resection of the tumour,patients with childhood onset CP had 50% increased rateof obesity. Obese patients are at further increased risk ofdeveloping subsequent atherosclerotic cardiovasculardiseases, metabolic syndromes and type 2 diabetesmellitus (DM).1II. Visual and neurological outcomesOptic neuropathy may be observed in children with CPdespite few cases presenting with visual symptoms.2Longstanding papilloedema, direct compression due totumour, radiotherapy and handling during surgery can allinjure the optic pathway, although, direct compression isdescribed as the major cause of visual loss.3 Pre-chiasmatictumours and critical pre-operative visual impairment arerisk factors for post-surgical visual disturbances. Surgery viatrans-sphenoidal route has shown better ophthalmologicaloutcomes, although it can be carried out in only a selectgroup of patients.4 Prevalence of persistent neurologicaldeficits is reported at around 8%, but may rise up to 36%for larger tumours. Treatment related neurological deficitsinclude cranial nerve injury, stroke, and epilepsy.5III. Endocrine imbalanceAround 40-80% children present with at least onehormonal deficiency at the time of diagnosis. Diabetesinsipidus (DI) is found in 17-27% of patients.6,7 Tumourextension and involvement of hypothalamic-pituitary axisalso affect the risks of post-operative endocrineimbalance.5 Hoffman et al., assessed long term endocrinestatus of 46 patients with childhood onset CP, and foundthat all patients were using some form of hormonereplacement. Ninety three percent of these patients wereon 1-desamine-8-D-arginine vasopressin (DDAVP)replacement, 89% cortisone, 83% thyroid, 30% sexhormones and 20% were on growth hormoneEVIDENCE BASED NEURO-ONCOLOGY   Quality of Life in Children Treated for CraniopharyngiomasMishal Gillani, Sheza Hassan, Umme Hani Abdullah, Muhammad Waqas Saeed Baqai, Muhammad Shahzad ShamimThe Aga Khan University Hospital, Karachi, Pakistan.Correspondence: Muhammad Shahzad Shamim. e-mail: shahzad.shamim@aku.edureplacements.6 One of the early manifestation of paediatricCP includes impaired growth, which often occurs yearsbefore diagnosis. At the time of diagnosis, growth hormone(GH) insufficiency has been recorded in 26-75% of CPpatients. On the other hand, GH deficiency was also presentin 70-92% of CP patients after treatment.7,8 The percentageof post-operative DI is between 40-93% while transientpost-surgical DI develops in up to 80-100% of total cases.9IV. Psychological and psychosocial wellbeingMemmesheimer et al., predicted loss of concentration,visual field defects, variable BMI and female sex asstrongest predictors for depression in patients treated forCP. For anxiety, BMI, negative life incidents and sleepinesswere considered the main predictors.10 Sterkenburg et al.,investigated psychosocial aspects such as education, job,marriage, offspring, and having a driver’s license in 89 CPpatients and reported that only 2 of the 89 patientssurviving to adulthood had offspring (both without HI).11V. Other determinants of QoLImpaired hypothalamic-pituitary axis (HPA) in patients withchildhood onset CP predispose them to QoL consequencesthat are more severe than adult onset CP, because theyhaven’t established their career, professional lives andfamily yet. Various studies report some clinical parameterssuch as female sex, recurrent surgery, obesity, visual fielddefects and radiotherapy to have adverse effects on QoL.Majority of young adults with childhood onset CP havedifficulty developing relationships, separating from theirparents and may prefer continuing family support over anindependent life.10 Several studies suggest that theneurobiological cause of the low QoL is involvement ofhypothalamus.7,11 The most common issues in paediatricpatients’ everyday life include incapability to controlemotions, low self-confidence, problems in pursuingeducation and unease related to physical appearance.Other hardships these patients face include decreasedmobility, less self-care, depression, anxiety, withdrawal andsomatic complaints such as pain.12 Neurocognitive deficitssuch as reduced attention span, memory related problems,sluggish cognitive speed and behavioural instability areconsidered as precursors to weak academic performance.Considering that intellectual stability has been describedin around 82% of patients, visual memory is till defectivedespite normal visual spatial functioning.13 Mehren et al.,suggested that childhood onset CP patients are atincreased risk for apathy, a condition associated withreduced activities of daily living and poor treatmentoutcomes due to lack of compliance. Apathetic symptomsvary in individuals depending on the tumour extension andinvolvement of motivational brain circuits.14 However,interventions such as efficient psychosocial assistance andself-care guidance may decrease the severity of disease.10The importance of improved diagnostic facilities, newtechniques and early treatment has also been emphasizedas it may improve the chances of better QoL in thesepatients.ConclusionQuality of life in craniopharyngioma patients is significantlyimpaired even after surgical resection and besides a closeendocrine follow up, requires physical, psychological andpsychosocial rehabilitation.References 1. Srinivasan S, Ogle GD, Garnett SP, Briody JN, Lee JW, Cowell CT. Fea-tures of the metabolic syndrome after childhood craniopharyn-gioma. The J. Clin. Endocrinol. Metab. 2004;89:81-6.2. Harbert MJ, Yeh-Nayre LA, O'Halloran HS, Levy ML, Crawford JR. Un-recognized visual field deficits in children with primary central nerv-ous system brain tumors. J Neurooncol. 2012;107:545-9.3. Goldenberg-Cohen N, Ehrenberg M, Toledano H, Kornreich L, Snir M,Yassur I, et al. Preoperative Visual Loss is the Main Cause of Irre-versible Poor Vision in Children with a Brain Tumor. Front. Neurol2011;2:62.4. Elliott RE, Jane JA, Jr., Wisoff JH. Surgical management of cranio-pharyngiomas in children: meta-analysis and comparison of tran-scranial and transsphenoidal approaches. Neurosurg.2011;69:630-43.5. Elliott RE, Wisoff JH. Surgical management of giant pediatric cranio-pharyngiomas.  J Neurosurg Pediatr. 2010;6:403-16.6. Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, BlaserSI. Aggressive surgical management of craniopharyngiomas in chil-dren. J Neurosurg. 1992;76:47-52.2073J Pak Med AssocTable: Studies on childhood onset craniopharyngioma and its treatment.Author Outcomes assessed Scale used1 Srinivasan et al1 Obesity BMI-SD, Percentage body fat formulaa2 Hoffman et al6 Intellectual, Emotional function Full scale intelligence quotient scores.3 Memmesheimer et al10 Psychological well-being HADSb, World Health Organization well-being index (WHO-5)4 Sterkenburg et al11 Obesity, Functional capacity, Psychosocial status, QoL BMI-SD, EORTC-QLQ-C301 questionnaire, MFI-2025 Kendall et al15 Obesity BMI-SD, MRI-score6 Riva et al16 Neurological, neurocognitive and behavioural development WCSTc, Benton visual retention test, Trail making test, Raven’s test.7 Muller et al17 Functional capacity, Obesity BMI-SD, FMHd8 Ozyurt et al18 Memory, Executive functions Emotional face recognition task, functional MRI (fMRI)a  Percentage body fat = DXA measured fat/ DXA measured soft tissue plus bone mineral content; b HADS (Hospital Anxiety and Depression Scale); c WCST:  Wisconsin Card Scoring Test; d  FMH: Fertigkeitenskala Munster/Heidelberg.M. Gillani, S. Hassan, U.H. Abdullah, et al.2074Vol. 70, No. 11, November 20207. Muller HL. Childhood craniopharyngioma. Recent advances in diag-nosis, treatment and follow-up. Hormon Res. 2008;69:193-202.8. Muller HL, Emser A, Faldum A, Bruhnken G, Etavard-Gorris N, Geb-hardt U, et al. Longitudinal study on growth and body mass indexbefore and after diagnosis of childhood craniopharyngioma.  J ClinEndocrinol Metab  2004;89:3298-305.9. Caldarelli M, Massimi L, Tamburrini G, Cappa M, Di Rocco C. Long-term results of the surgical treatment of craniopharyngioma: the ex-perience at the Policlinico Gemelli, Catholic University, Rome. Child'snervous system : ChNS : (official journal of the International Societyfor Pediatric Neurosurgery). 2005;21:747-57.10. Memmesheimer RM, Lange K, Dölle M, Heger S, Mueller I. Psycho-logical well‐being and independent living of young adults withchildhood‐onset craniopharyngioma. DEV MED CHILD NEUROL.2017;59:829-36.11. Sterkenburg AS, Hoffmann A, Gebhardt U, Warmuth-Metz M,Daubenbuchel AM, Muller HL. Survival, hypothalamic obesity, andneuropsychological/psychosocial status after childhood-onset cran-iopharyngioma: newly reported long-term outcomes. Neuro-oncol.2015;17:1029-38.12. Poretti A, Grotzer MA, Ribi K, Schonle E, Boltshauser E. Outcome ofcraniopharyngioma in children: long-term complications and qualityof life. DEV MED CHILD NEUROL 2004;46:220-9.13. Crom DB, Smith D, Xiong Z, Onar A, Hudson MM, Merchant TE, et al.Health Status in Long-Term Survivors of Pediatric Craniopharyn-giomas. J Neurosc Nursing. 2010;42:323-8.14. Mehren A, Özyurt J, zu Klampen P, Boekhoff S, Thiel CM, Müller HL.Self- and informant-rated apathy in patients with childhood-onsetcraniopharyngioma. J Neuro-Oncol,. 2018;140:27-35.15. Kendall BE, Neville BG, de Vile CJ, Grant DB, Stanhope R, Hayward RD.Obesity in childhood craniopharyngioma: relation to post-operativehypothalamic damage shown by magnetic resonance imaging.J.Clin. Endocrinol. Metab.. 1996;81:2734-7.16. Riva D, Pantaleoni C, Devoti M, Saletti V, Nichelli F, Giorgi C. Late neu-ropsychological and behavioural outcome of children surgicallytreated for craniopharyngioma. Child's nervous system : ChNS : offi-cial journal of the International Society for Pediatric Neurosurgery.1998;14:179-84.17. Muller HL, Gebhardt U, Etavard-Gorris N, Korenke E, Warmuth-MetzM, Kolb R, et al. Prognosis and sequela in patients with childhoodcraniopharyngioma -- results of HIT-ENDO and update on KRANIO-PHARYNGEOM 2000. Klinische Padiatrie. 2004;216:343-8.18. Ozyurt J, Thiel CM, Lorenzen A, Gebhardt U, Calaminus G, Warmuth-Metz M, et al. Neuropsychological outcome in patients with child-hood craniopharyngioma and hypothalamic involvement. J Pediat..2014;164:876-81.e4.Quality of Life in Children Treated for ………………….

Quality of life in children treated for craniopharyngiomas

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Quality of life in children treated for craniopharyngiomas

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