Optimal management of craniopharyngioma is challenging and requires multidisciplinary efforts for a successful outcome. The debate over radical versus a conservative resection followed by radiotherapy, continues. Current literature reports no difference in disease freedom and overall survival between the two surgical viewpoints, and favours conservative resection and radiotherapy, leading to lower morbidity and superior functional outcomes

Bakhshi, Saqib Kamran

Mirza, Farhan A

Pervez, Wardah

Shamim, Muhammad Shahzad

English

eCommons@AKU

eCommons@AKU Section of Neurosurgery Department of Surgery 2-1-2021 Complete versus subtotal resection of paediatric craniopharyngioma Wardah Pervez Saqib Kamran Bakhshi Farhan A. Mirza Muhammad Shahzad Shamim Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurg  Part of the Neurology Commons, Neurosurgery Commons, Oncology Commons, and the Surgery Commons 564J Pak Med AssocAbstractOptimal management of craniopharyngioma ischallenging and requires multidisciplinary efforts for asuccessful outcome. The debate over radical versus aconservative resection followed by radiotherapy,continues. Current literature reports no difference indisease freedom and overall survival between the twosurgical viewpoints, and favours conservative resection andradiotherapy, leading to lower morbidity and superiorfunctional outcomes. Keywords: Paediatric craniopharyngioma, extent ofresection, radiotherapyIntroductionCraniopharyngioma is a benign, epithelial tumour, or assome authors have mentioned, an embryonicmalformation in the sellar/suprasellar location. It ispredominantly seen in children and adolescents1 andcomprises around 10% of all paediatric central nervoussystem tumours.2 The close proximity ofcraniopharyngioma to eloquent structures such as pituitarygland, optic chiasm, hypothalamus and carotid arteries,makes surgical resection as well as adjuvant radiationchallenging.3Significant controversy mars the optimal treatmentstrategy for managing craniopharyngioma. Even thoughthese are non-malignant lesions, recurrence or diseaseprogression often blemishes the aim of long-term diseasecontrol. Gross total resection (GTR) reduces the risk ofrecurrence and has traditionally been proposed as the beststrategy.4,5 But with the high rates of endocrinological andvisual complications associated with GTR the pendulumhas swung towards subtotal resection (STR) followed byradiotherapy.5,6 However, the intellectual impedance,mental retardation, and risk of secondary tumours inducedby radiotherapy has again lead  surgeons and oncologiststo rethink their approach towards this lesion. This has beenfurther supported by the advent of extended endonasalapproaches (EEA) providing a direct approach to thesechallenging tumours, without transgression of normalbrain.During last few decades, our understanding of thepathogenesis and natural history of craniopharyngiomahas improved immensely. Although extensive literature hasbeen published and surgeons have been modifying theirapproach to these tumours, there is still a lack of consensuson the standard treatment for craniopharyngiomas inchildren.7 We have reviewed the existing literaturepublished on the surgical management ofcraniopharyngioma, and have aimed to summarize theavailable recommendations for an ideal approach to thesetumours.Review of evidenceRecent advancement in surgical techniques, includingincreased use of endoscopic approach, and imagingtechnology has reduced the overall risk of surgicalcomplications during resection of craniopharyngioma. Itwould be fair to say that trans-nasal endoscopic approachor EEA is now the standard for these tumours. Othercommon surgical approaches include microscopic andendoscope-assisted trans-cranial approaches, as well astrans-ventricular endoscopic approaches.8,9Surgical ApproachThe goal of surgery in most cases is to relievehydrocephalus and/or decompress the optic apparatus.The surgical approach should be tailored to themorphology of the lesion. Predominantly cystic tumoursextending into the third ventricle are amenable to a trans-ventricular endoscopic approach for cyst decompressionand biopsy. This can be accompanied by insertion of anOmmaya reservoir for repeat cyst drainage if it enlarges andbecomes symptomatic. This also allows instillation ofchemotherapeutic agents directly into the cyst. Solidtumours in the sellar/suprasellar region with superiorextension into the third ventricle are generally amenableto extended endonasal approaches which provide themost direct corridor to tumour resection. Tumourspredominantly in the third ventricle with limited suprasellarpresence are difficult to access via the trans-nasal route andoften require pituitary transposition maneuvers. Advancedmaneuvers may be required to mobilize the pituitary glandand protect the chiasm if the tumour is completely retro-chiasmatic. These tumours are better served with atrans-callosal approach or a sub-frontal translaminarterminalis approach. Regardless of approach, the tumour-hypothalamic plane should be respected at all costs tominimize hypothalamic transgression.EVIDENCE BASED NEURO-ONCOLOGY   Complete versus Subtotal Resection of Paediatric CraniopharyngiomaWardah Pervez1, Saqib Kamran Bakhshi2, Farhan A. Mirza3, Muhammad Shahzad Shamim41,2,4Section of Neurosurgery, Aga Khan University Hospital, Karachi-Pakistan;3Kentucky Neuroscience Institute (KNI), Department of Neurosurgery, University of Kentucky, Lexington, KY, USA.Correspondence: Muhammad Shahzad Shamim. e-mail: shahzad.shamim@aku.eduExtent of ResectionSarkar et al. retrospectively studied 37 children (mean age10 years and median follow-up of 79 months) 16 (43.2%)underwent GTR, 6 (16.2%) had near-total resection and 15(40.5%) had STR.10 Extent of resection was defined on post-op MRI done 3-6 months after surgery, and patients withSTR had also received adjuvant radiotherapy.10 Childrenwith STR and radiotherapy had a better survival ascompared to those who underwent radical surgery, and theextent of resection did not influence long-term functionaloutcome.10Vile et al. studied 75 paediatric craniopharyngioma casestreated at their hospital over  a 20 year period.11 Detailedendocrinological, visual, psychological, neurological,hypothalamic and radiological evaluations were completedin all  patients pre- and post-operatively. GTR was achievedin 29 (39.7%) children while 44 (60.3%) had STR. Attemptsto remove the tumour adherent to the hypothalamusresulted in greater hypothalamic dysfunction after surgery.STR was associated with less post-operative morbidity andthere was no statistically significant difference in thetumour recurrence rate between patients who achievedGTR versus those who achieved STR with adjuvantradiotherapy. In a follow up study by the same group, 59children were treated with a less aggressive approach withSTR/cyst aspiration followed by radiotherapy.12 This studyproposed that use of image guidance and endoscopydecreased surgical morbidity, and targeted radiotherapyusing proton-beam resulted in better functional outcomesas compared to the earlier cohort study performed at theircenter. This study also noted that children who present ata younger age (≤ 5 years) are poised to develop higherlong-term morbidity. As radiotherapy cannot be safelyadministered in this young group, they are at a higher riskof tumour recurrence therefore more radical surgicalresection should be considered if safely possible.12In another series of 28 children, Liu et al. reported GTR in11 (39%), STR in 14 (50%), and biopsy in 3 (11%) patients.13Seven patients with STR and 2 patients with GTR receivedadjuvant radiotherapy, whereas the 3 patients who had565Vol. 71, No. 2-A, February 2021Complete versus Subtotal Resection of Paediatric ………Figure-1: T2 weighted axial section, and T1-post contrast coronal and sagittal sections showing a predominantly solid, heterogenously enhancing sellar and suprasellar cranio-pharyngioma which is extending into the sphenoid sinus and 3rd ventricle, and is causing hydrocephalus.Figure-2: T2 weighted axial section, and T1-post contrast coronal and sagittal sections showing a predominantly cystic, sellar and suprasellar craniopharyngioma which is ringenhancing and has a small enhancing solid component. It is displacing the floor of 3rd ventricle, and is causing hydrocephalus.566J Pak Med Assocundergone biopsy, received intra-cystic interferon therapyvia Omaya reservoir. In their cohort, extent of resectionalone was not a significant predictor of survival, however,patients who had received adjuvant radiotherapy hadbetter progression free and overall survival, as comparedto those who underwent surgery alone or surgery withinterferon therapy.In another series of 25 children 21 (84%) underwent GTR.They had proposed GTR to be associated with morefavourable outcome.14 However, in an extensive systematicreview of 377 patients, Clark et al. concluded that STR andradiotherapy offers similar tumour control rate as GTR, andtherefore, should be the aim of surgical treatment.15ConclusionCraniopharyngioma should be considered and treated asa chronic disease. Goal of treatment should be aimed atmaximizing preservation of pituitary, hypothalamic, andvisual function. Current evidence predominantly suggeststhat the extent of surgical resection does not predictprogression free survival or overall survival in paediatriccraniopharyngioma, as GTR and STR with radiotherapyhave similar survival outcomes. Aggressive resection carriesa higher risk of morbidity and thus, should be reserved foronly those cases where the tumour is easily resectable andthe tumour-hypothalamic plane can be preserved. Inyounger children in whom radiotherapy is not a feasibleoption, maximizing safe resection and use of intra-cystictherapy should be considered. References 1. DiPatri AJ, Prabhu V. A history of the treatment of craniopharyn-giomas.  Childs Nerv Syst. 2005;21:606-21.2. Soldozy S, Yeghyayan M, Yağmurlu K, Norat P, Taylor DG, Kalani MY,Jane JA, Syed HR. Endoscopic endonasal surgery outcomes for pe-diatric craniopharyngioma: a systematic review. Neurosurg. Focus.2020;48:E6.3. Karavitaki N, Cudlip S, Adams CB, Wass JA. Craniopharyngiomas. En-docr. Rev. 2006;27:371-97.4. De Vile CJ, Grant DB, Kendall BE, Neville BG, Stanhope R, Watkins KE,Hayward RD. Management of childhood craniopharyngioma: canthe morbidity of radical surgery be predicted? J Neurosurg.1996;85:73-81.5. Stripp DC, Maity A, Janss AJ, Belasco JB, Tochner ZA, Goldwein JW,Moshang T, Rorke LB, Phillips PC, Sutton LN, Shu HK. Surgery with orwithout radiation therapy in the management of craniopharyn-giomas in children and young adults"Int. J. Radiat. Oncol. Biol. Phys.2004;58:714-20.6. Yang I, Sughrue ME, Rutkowski MJ, Kaur R, Ivan ME, Aranda D, BaraniIJ, Parsa AT. Craniopharyngioma: a comparison of tumor control withvarious treatment strategies.  Neurosurg. Focus.. 2010;28:E5.7. Clark AJ, Cage TA, Aranda D, Parsa AT, Sun PP, Auguste KI, Gupta N. Asystematic review of the results of surgery and radiotherapy ontumor control for pediatric craniopharyngioma Childs Nerv Syst..2013;22:231-8.8. Wijnen M, van den Heuvel-Eibrink MM, Janssen JA, Catsman-Berrevoets CE, Michiels EM, van Veelen-Vincent MC, Dallenga AH,van den Berge JH, Van Rij CM, van der Lely AJ, Neggers SJ. Very long-term sequelae of craniopharyngioma. Eur J Endocrinol.2017;176:755-67.9. Park HR, Kshettry VR, Farrell CJ, Lee JM, Kim YH, Won TB, Han DH, DoH, Nyguist G, Rosen M, Kim DG. Clinical outcome after extended en-doscopic endonasal resection of craniopharyngiomas: two-institu-tion experience. World Neurosurg.. 2017;103:465-74.10. Sarkar S, Chacko SR, Korula S, Simon A, Mathai S, Chacko G, ChackoAG. Long-term outcomes following maximal safe resection in a con-temporary series of childhood craniopharyngiomas. Acta Neurochir. 2020;19:1-1.11. De Vile CJ, Grant DB, Kendall BE, Neville BG, Stanhope R, Watkins KE,Hayward RD. Management of childhood craniopharyngioma: canthe morbidity of radical surgery be predicted? J Neurosurg.1996;85:73-81.12. Marcus HJ, Rasul FT, Hussein Z, Baldeweg SE, Spoudeas HA, HaywardR, Thompson D, Grieve JP, Dorward NL, Aquilina K. Craniopharyn-gioma in children: trends from a third consecutive single-center co-hort study. J Neurosurg: Paed. 2019;25:242-50.13. Liu AP, Tung JY, Ku DT, Luk CW, Ling AS, Kwong DL, Cheng KK, Ho WW,Shing MM, Chan GC. Outcome of Chinese children with cranio-pharyngioma: a 20-year population-based study by the Hong KongPediatric Hematology/Oncology Study Group. Childs Nerv Syst.2020;36:497-505.14. Bidur KC, Prasad DU. Outcome following surgical resection of cran-iopharyngiomas: A case series. Asian J. Neurosurg.2017;12:514.15. Clark AJ, Cage TA, Aranda D, Parsa AT, Sun PP, Auguste KI, Gupta N. Asystematic review of the results of surgery and radiotherapy ontumor control for pediatric craniopharyngioma. Childs Nerv Syst.2013;29:231-8.W. Pervez, S.K. Bakhshi, F.A. Mirza, et al.

Complete versus subtotal resection of paediatric craniopharyngioma

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Complete versus subtotal resection of paediatric craniopharyngioma

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