20-Year experience in childhood craniopharyngioma

doi:10.1016/0360-3016(93)90227-M

International Journal of Radiation OncologyBiologyPhysics

Volume 27, Issue 2, 30 September 1993, Pages 189-195

https://doi.org/10.1016/0360-3016(93)90227-M Get rights and content

Abstract

$Purpose$ : The management of craniopharyngioma is controversial, and surgery alone is frequently advocated. The purpose of this study was to assess the long-term impact of various treatments in childhood craniopharyngioma.

$Methods and Materials$ : Sixty-one children ≤ 21 years of age at diagnosis were treated for craniopharyngioma at Children's Hospital and the Joint Center for Radiation Therapy in Boston from 1970 to 1990. The median age was 7.5 years (range 10 months–21 years). There were 33 females and 28 males. The median follow-up was 10 years (range 2–20.5 years). Neuroimaging was available for detailed review in 53. Nine children were treated with radiotherapy alone, 15 were treated with surgery alone, and 37 were treated with both surgery and radiotherapy. All patients in the radiotherapy and surgery plus radiotherapy groups were treated with megavoltage radiation with a median dose of 5464 cGy.

$Results$ : All nine of the children treated with radiation therapy alone are alive; none have recurred. Nine of the 15 children treated with surgery alone have recurred (p = 0.007 Fisher exact test). Two are alive with disease, and seven are alive without disease after treatment at relapse with radiation therapy, surgery, or both. Seven of the 37 patients treated with surgery plus radiotherapy have recurred. Three of the seven patients are dead of disease, three patients are alive with disease, and one patient is alive without disease after further treatment. The 10-year actuarial overall survival was 91% for all patients. The 10-year actuarial freedom from progression for the surgery group was 31% compared with 100% for patients treated with radiation therapy only (log rank p = 0.01), and 86% for patients treated with surgery plus radiotherapy at diagnosis (p = 0.001). There were two treatment related deaths, both in the surgery plus radiotherapy group. A higher incidence of visual loss and diabetes insipidus was associated with the use of aggressive surgery. The size of the tumor at presentation correlated with an increased risk of recurrence; 5 of 6 patients with tumors ≥ 5 cm experienced recurrences while only 6 of 30 recurred when the tumor was < 5 cm.

$Conclusions$ : Overall survival in childhood craniopharyngioma is excellent. However, patients treated with surgery alone have a significantly worse freedom from progression when compared to patients treated with surgery and radiation therapy or radiation therapy alone.

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This study was retrospectively performed on 46 patients suffering from craniopharyngioma referred to Loghman Hakim Hospital sparing from 2011 to 2016. In patients with magnetic resonance imaging (MRI) and computerized tomography (CT) scans, endocrinology tests, other related tests, and complete neuromuscular examinations were performed before and after surgery. All patient information was collected descriptively according to their case, and quantitative data are displayed using mean, Standard Deviation, intermediate, and Interquartile range, and qualitative data are displayed using frequency and percentage. For analytical analysis, the data were calculated from independent samples t, Mann-Whitney statistical tests, K2 test, and, if necessary, Fisher's precise P-Value. A significant level was considered for the 5 % statistical tests, and the SPSS statistical software version 25 was used to analyze the data.
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Magnetic resonance imaging (MRI) is now the established imaging modality used to diagnose and treat paediatric brain tumours, the leading cause of cancer-related death in children. Optimal use of MRI deployed independently, or in combination with other modalities, such as computed tomography, single-photon emission computed tomography and positron emission tomography, provides exquisite detail of structural, anatomical and physiologic information that facilitates accurate diagnosis, timely intervention and improved patient outcomes. The role of functional imaging sequences continues to evolve, and helps grade tumours, plan surgical approaches, assess biology of tumour relapse and helps monitor response to surgical and non-surgical treatment. The aim of this chapter is threefold: First, to review the neuroimaging features of a broad spectrum of paediatric brain tumours with a focus on MR imaging; second, to discuss specific paediatric tumour types grouped by location and third, highlight changes in the most recent revision of the WHO classification of paediatric brain tumours. This chapter further describes (1) the utility of MR perfusion imaging in characterising tumour hemodynamics; (2) MR spectroscopy in assessing brain tumour metabolism and physiology; (3) diffusion imaging in characterising tumour, grading, assessing cellularity and evaluating treatment response and (4) diffusion tensor imaging in visualising and quantifying brain tumours, especially gliomas. Finally, MRI's precision in localising and targeting brain tumours has vastly improved surgical intervention techniques using image guidance. With increased reliance on MRI in evaluating and treating paediatric brain tumours and the ability to better correlate imaging findings with recent advances in molecular genetics of brain tumours, physicians are now better equipped to battle this devastating disease.
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Craniopharyngiomas are difficult to treat because of their dense adherence to surrounding structures and the frequent presence of cysts which obscure the anatomy. The introduction of endonasal endoscopic approaches has enabled microsurgery to be performed without mortality. The high recurrence rate requires other forms of treatment of which GKNS has currently been shown to be perhaps the most effective. Difficulties include poorly visualized visual pathways despite which a high degree of tumor control is achievable with surprisingly little damage to vision.
Characteristics of ectopic recurrence of craniopharyngioma: Case report and literature review
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Craniopharyngiomas makes up between 5 and 15% of brain tumors in children and 1% in adults. While these lesions are considered to be benign, they are characteristically infiltrative and have a high rate of local recurrence. Although rare, ectopic recurrences have been documented in case reports with a total of 61 cases available. Here we analyze the available literature for trends in patients with ectopic recurrence as well as present a case of an ectopic recurrence which was resected 11 years after its initial diagnosis. Ectopic recurrence is thought to occur due to seeding along the surgical tract or spread along the CSF pathway. These lesions occur at a later time point after initial treatment compared to local recurrences. All pediatric cases were of adamantinomatous histology, while 27% of adult cases showed papillary histology, matching the distribution of general adult craniopharyngioma. Half of all cases recurred in the frontal lobes with an even distribution in the rest of the cited locations, likely reflecting the surgical approach. We found a significantly longer time period to ectopic recurrence in patients who underwent radiotherapy for subtotal tumor resection compared to those who either had a gross total resection or subtotal resection with no further treatment. Further, the Ki-67 labeling indices, which can serve as a marker of the proliferative potential of tumors was lower in the ectopic recurrences when compared to available data on local recurrences, possibly explaining the longer time between initial diagnosis and ectopic recurrence. In some of the cases, local recurrence preceded ectopic recurrence and we looked at how and if the local recurrences are different from those reported in the literature, which we did not find to be significantly different. In light of longer time to recurrence in these cases, prolonged follow-up is warranted in patients post craniopharyngioma resection.
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Craniopharyngiomas can invade surrounding structures, including the optic chiasm and hypothalamus. In such cases, subtotal resection is often preferred to limit perioperative morbidity and mortality; however, subtotal resection is associated with high rates of recurrence. Recurrent craniopharyngioma is typically treated with another subtotal resection and adjuvant radiotherapy. We present a case of a patient found to have a large craniopharyngioma compressing the optic chiasm, hypothalamus and left cavernous sinus. She underwent surgical debulking but developed recurrence shortly thereafter. Subsequently, she underwent a second debulking surgery, followed by fractionated stereotactic radiotherapy (SRT). Results show that she exhibited an impressive response to SRT with further tumor shrinkage, while remaining clinically well. This case demonstrates the efficacy of SRT in salvage of recurrent craniopharyngioma.
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Citation Excerpt :
Though optimal management strategy had been a matter of debate for long, the general consensus is that the conservative resection with adjuvant therapy carries equally good outcome in terms of local tumor control with less postoperative morbidities Many authors believe that the postoperative morbidities often result from the radical excision and prefer a conservative approach over radical resection [4–8]. Neither the proponents of total excision (TE) nor those of subtotal excision (STE) combined with adjuvant radiotherapy (RT) have been able to prove the superior outcome of one over the other [4,5,7,8].
The optimal therapeutic approach to craniopharyngioma has not been established conclusively. The surgical outcome following radical excision and conservative resection with adjuvant radiotherapy are comparable or even better with the later in pediatric or mixed populations. This study is aimed at reviewing the role of extent of resection in local tumor control and long-term outcome in adults with craniopharyngioma.
Ninety-five adults operated between 2001 and 2013 were included. Progression-free survival (PFS) and overall survival (OS) were calculated. Predictors of various outcome parameters were analyzed.
The predominant presenting symptom was visual impairment (78 %) followed by symptoms of hypothalamic involvement (29 %). Total and subtotal excisions (TE, STE) were achieved in 63 %, and 47 % of patients, respectively. Vision improved in 62 % of patients in the early postoperative period. Thirteen patients (14 %) experienced vision deterioration postoperatively. On multivariate analysis, tumor >3 cm and optic atrophy predicted poor visual outcome. Hormonal replacement for hypopituitarism was required in 83 % during follow-up. Diabetes insipidus was seen in 73 %. Imaging evidence of 3rd ventricular floor destruction by tumor emerged as an independent predictor of postoperative hypothalamic morbidities. The recurrence rate following TE and STE was 11.6 % and 72 %, respectively. Unlike radical excision, PFS following STE was significantly shorter (p- 0.02). TE was not associated with increased visual impairment or hypothalamic-pituitary dysfunction postoperatively as compared to STE. Most of the patients (85 %) were independent and able to return to the premorbid occupation.
Subtotal resection provides equally good long-term visual, endocrinological, and hypothalamic outcomes as radical surgery. When used with adjuvant radiotherapy, it also gives a better local control of the tumor. Hence, subtotal resection with adjuvant radiotherapy should be considered as an effective alternative strategy to radical excision.

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^☆: Presented at the 34th Annual Meeting of the American Society for Therapeutic Radiology and Oncology, San Diego, CA, 12 November 1992.

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Clinical original contribution20-Year experience in childhood craniopharyngioma☆

Abstract

Int. J. Radiat. Oncol. Biol. Phys.

Int. J. Radiat. Oncol. Biol. Phys.

Int. J. Radiat. Oncol. Biol. Phys.

Neurological and psychophysiological sequelae following different treatments of craniopharyngioma in children

J. Neurosurg.

Factors affecting intellectual outcome in pediatric brain tumor patients

Neurosurg.

Craniopharyngiomas in children: long-term effects of conservative surgical procedures combined with radiation therapy

J. Neurosurg.

Aggressive surgical management of craniopharyngiomas in children

J. Neurosurg.

Surgery and radiation therapy in the management of craniopharyngiomas

Int. J. Radiat. Oncol. Biol. Phys.

Clinical original contribution
20-Year experience in childhood craniopharyngioma☆