对生活质量产生重大影响。最应将颅咽管瘤看作多维慢性疾病。[47]Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16:56-67.http://rd.springer.com/article/10.1007%2Fs11102-012-0401-0http://www.ncbi.nlm.nih.gov/pubmed/22678820?tool=bestpractice.com 此外,颅咽管瘤成人患者相对于一般人群,心血管疾病风险显著升高,它将加重这些患者已普遍存在的健康不佳状况。[48]Erfurth EM. Endocrine aspects and sequel in patients with craniopharyngioma. J Pediatr Endocrinol Metab. 2015;28:19-26.http://www.ncbi.nlm.nih.gov/pubmed/25514328?tool=bestpractice.com
肿瘤完全切除
复发风险范围为 10%-35%。虽然经碟入路的手术死亡率约为 1%,但经碟入路全切肿瘤后的复发率最低。[10]Dhellemmes P, Vinchon M. Radical resection for craniopharyngiomas in children: surgical technique and clinical results. J Pediatr Endocrinol Metab. 2006;19(suppl 1):329-335.http://www.ncbi.nlm.nih.gov/pubmed/16700308?tool=bestpractice.com[57]Fahlbusch R, Honegger J, Paulus W, et al. Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg. 1999;90:237-250.http://www.ncbi.nlm.nih.gov/pubmed/9950494?tool=bestpractice.com 肿瘤复发大多数发生在初次手术后第一个 5 年内。在术后和长期随访中,>70% 的患者功能保留。[20]Sorva R, Heiskanen O, Perheetupa J. Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst. 1998;4:97-99.http://www.ncbi.nlm.nih.gov/pubmed/3401877?tool=bestpractice.com[39]Poretti A, Grotzer MA, Ribi K, et al. Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol. 2004;46:220-229.http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2004.tb00476.x/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/15077699?tool=bestpractice.com[40]Kiehna EN, Mulhem RK, Li C, et al. Changes in attentional performance of children and young adults with localized primary brain tumors after conformal radiation therapy. J Clin Oncol. 2006;24:5283-5290.http://jco.ascopubs.org/cgi/content/full/24/33/5283http://www.ncbi.nlm.nih.gov/pubmed/17114662?tool=bestpractice.com[58]Dekkers OM, Biermasz NR, Smit JW, et al. Quality of life in treated adult craniopharyngioma patients. Eur J Endocrinol. 2006;154:483-489.http://www.eje-online.org/content/154/3/483.full.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/16498063?tool=bestpractice.com[59]Gupta DK, Ojha BK, Sarkar C, et al. Recurrence in craniopharyngiomas: analysis of clinical and histological features. J Clin Neurosci. 2006;13:438-442.http://www.ncbi.nlm.nih.gov/pubmed/16678722?tool=bestpractice.com[60]De Vile CJ, Grant DB, Kendall BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg. 1996;85:73-81.http://www.ncbi.nlm.nih.gov/pubmed/8683285?tool=bestpractice.com
根治性手术增加下丘脑损伤的风险(饮食过量、肥胖、神经系统行为异常),是长期随访中出现并发症的主要原因。[17]Puget S, Garnett M, Wray A, et al. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg. 2007;106:3-12.http://www.ncbi.nlm.nih.gov/pubmed/17233305?tool=bestpractice.com[28]Hoffman HJ, De Silva M, Humphreys RP, et al. Aggressive surgical management of craniopharyngiomas in children. J Neurosurg. 1992;76:47-52.http://www.ncbi.nlm.nih.gov/pubmed/1727168?tool=bestpractice.com[51]Müller HL, Gebhardt U, Teske C, et al. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after three year follow-up. Eur J Endocrinol. 2011;165:17-24.http://www.eje-online.org/content/165/1/17.full.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/21490122?tool=bestpractice.com[60]De Vile CJ, Grant DB, Kendall BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg. 1996;85:73-81.http://www.ncbi.nlm.nih.gov/pubmed/8683285?tool=bestpractice.com
术后需注意有些患者视力改善,而有些恶化。然而,多达 80% 的患者仍有视觉损伤。[20]Sorva R, Heiskanen O, Perheetupa J. Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst. 1998;4:97-99.http://www.ncbi.nlm.nih.gov/pubmed/3401877?tool=bestpractice.com[39]Poretti A, Grotzer MA, Ribi K, et al. Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol. 2004;46:220-229.http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2004.tb00476.x/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/15077699?tool=bestpractice.com
大多数 (>80%) 患者表现为多种内分泌功能紊乱,需要激素替代治疗。[38]Müller HL. Consequences of craniopharyngioma surgery in children. J Clin Endocrinol Metab. 2011;96:1981-1991.http://jcem.endojournals.org/content/96/7/1981.longhttp://www.ncbi.nlm.nih.gov/pubmed/21508127?tool=bestpractice.com[61]Honegger J, Buchfelder M, Fahlbusch R. Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg. 1999 Feb;90(2):251-7.http://www.ncbi.nlm.nih.gov/pubmed/9950495?tool=bestpractice.com[62]Duff JM, Meyer FB, Ilstrup DM, et al. Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery. 2000;46:291-305.http://www.ncbi.nlm.nih.gov/pubmed/10690718?tool=bestpractice.com
肿瘤不完全切除后放射治疗
复发风险为 25%-50%。[31]Weiss M, Sutton L, Marcial V, et al. The role of radiation therapy in the management of childhood craniopharyngioma. Int J Radiat Oncol Biol Phys. 1989;17:1313-1321.http://www.ncbi.nlm.nih.gov/pubmed/2689398?tool=bestpractice.com[32]Habrand JL, Ganry O, Couanet D, et al. The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys. 1999;44:255-263.http://www.ncbi.nlm.nih.gov/pubmed/10760417?tool=bestpractice.com[33]Varlotto JM, Flickinger JC, Kondziolka D, et al. External beam irradiation of craniopharyngioma: long-term analysis of tumor control and morbidity. Int J Radiat Oncol Biol Phys. 2002;54:492-499.http://www.ncbi.nlm.nih.gov/pubmed/12243827?tool=bestpractice.com[36]Scarzello G, Buzzaccarini MS, Perilongo G, et al. Acute and late morbidity after limited resection and focal radiation therapy in craniopharyngiomas. J Pediatr Endocrinol Metab. 2006 Apr;19 Suppl 1:399-405.http://www.ncbi.nlm.nih.gov/pubmed/16700317?tool=bestpractice.com 肿瘤复发大多数发生在初次手术后第一个 5 年内。尽管部分临床医师认为手术全切和不完全切除后放疗相比,其长期预后相当,但是长期随访的数据更加支持手术全切除肿瘤。