Craniopharyngioma — long-term results following limited surgery and radiotherapy

Rajan, Balakrishnan; Ashley, S.; Gorman, Christine; Jose, C.C.; Horwich, A.; Bloom, H. J. G.; Marsh, Henry; Brada, Michael

doi:10.1016/0167-8140(93)90019-5

Cited by 301 publications

(218 citation statements)

References 54 publications

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“…Morbidity and mortality in this patient group results from injury to the critical structures surrounding the tumor, inflicted by the tumor itself and the treatment. Recent series reports 81% to 91% 10-year recurrence-free survival rates after subtotal tumor removal followed by radiotherapy, with 46% to 58% of the survivors living a normal independent life (40,41). Still, a substantial part of the patients are carrying considerable morbidity.…”

Section: Discussionmentioning

confidence: 99%

Expression and Growth Dependency of the Insulin-Like Growth Factor I Receptor in Craniopharyngioma Cells: A Novel Therapeutic Approach

Ulfarsson¹,

Karström

Yin

et al. 2005

Clinical Cancer Research

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Craniopharyngioma is a rare benign intracranial epithelial tumor that, however, often recurs and sometimes kills the affected patients, one-third of which are children. In many cases, the patients acquire growth hormone deficiency and postoperatively need substitution. Generally, growth hormone promotes local release of insulin-like growth factor I (IGF-I), which in turn activates the IGF-I receptor (IGF-IR) if present. Together, these circumstances raise the question whether IGF-IR may be involved in craniopharyngioma growth. To address this issue, we analyzed phenotypically well-characterized primary low-passage craniopharyngioma cell lines from nine different patients for IGF-IR expression and IGF-I dependency. Two of the cell lines showed no/very low expression of the receptor and was independent on IGF-I, whereas five cell lines exhibited a strong expression and was clearly contingent on IGF-I. The two remaining cell lines had low receptor expression and IGF-I dependency. Upon treatment with an IGF-IR inhibitor, cells with high IGF-IR expression responded promptly with decreased Akt phosphorylation followed by growth arrest. These responses were not seen in cells with no/very low receptor expression. Growth of cell lines with low IGF-IR expression was only slightly affected by IGF-IR inhibition. Taken together, our data suggest that IGF-IR may be involved in the growth of a subset of craniopharyngiomas and points to the possibility of the involvement of IGF-IR inhibitors as a treatment modality to obtain complete tumor-free conditions before growth hormone substitution.

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Section: Discussionmentioning

confidence: 99%

Expression and Growth Dependency of the Insulin-Like Growth Factor I Receptor in Craniopharyngioma Cells: A Novel Therapeutic Approach

Ulfarsson¹,

Karström

Yin

et al. 2005

Clinical Cancer Research

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“…The OS rates (which reflect effect of multiple treatments) described in an exclusively pediatric series ranged from 83 to 96% at 5 years (14, 15, 17, 30, 31, 32, 33) and 65 to 100% at 10 years (15, 26, 30, 31, 32, 34, 35, 36, 37, 38, 39, 40, 41), averaging 62% at 20 years (42). In adults or a broad age-range population (adults and children) series, the OS rates ranged from 54 to 96% at 5 years (13,16,25,29,32,43,44,45,46), 40 to 93% at 10 years (13,16,25,28,29,32,43,44,45,46), and 66 to 85% at 20 years (29,45,46). The lower limits of survival rates usually reflected data from earlier series that occurred before modern advances in microsurgery, neuroimaging, and radiotherapy.…”

Section: European Journal Of Endocrinologymentioning

confidence: 99%

“…What remains unclear is whether the age at diagnosis represents a survival prognostic factor because some studies have demonstrated that the youngest patients have better survival rates (28,32,44), others have found better outcome in older patients (12,46), whereas still other studies report no difference between pediatric and adult populations (13,16,45,47). The role of sex as a prognostic factor is not established; some authors report a higher mortality among females (28,29), but others have not found any sex differences (13,15,16).…”

Section: European Journal Of Endocrinologymentioning

confidence: 99%

“…The standardized overall mortality rate varies from 2.88 to 9.28 in cohort studies covered in this review. According to the review, patients with childhood CP have a threeto 19-fold higher cardiovascular mortality in comparison with the general population, and female childhood CP patients have an even higher risk (46). Tumor size is likely to be a prognostic factor because increased survival rates have been shown in tumors with a diameter smaller than 3 cm (47).…”

Section: European Journal Of Endocrinologymentioning

confidence: 99%

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Hydrocephalus and hypothalamic involvement in pediatric patients with craniopharyngioma or cysts of Rathke's pouch: impact on long-term prognosis

Daubenbüchel

Hoffmann

Gebhardt

et al. 2015

European Journal of Endocrinology

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Objective: Pediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke's pouch (CRP) frequently suffer disease-and treatment-related sequelae. We analyzed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP. Subjects and methods: Using retrospective analysis of patient records, presence of initial HY or HI was assessed in 177 pediatric patients (163 CP and 14 CRP). Twenty-year overall survival (OS) and progression-free survival (PFS), FC, and BMI were analyzed with regard to initial HY, degree of resection, or HI. Results: Of the 177 patients, 105 patients (103/163 CP and 2/14 CRP) presented with initial HY and 96 presented with HI. HY at diagnosis was associated (PZ0.000) with papilledema, neurological deficits, and higher BMI at diagnosis and during followup. OS, PFS, and FC were not affected by HY at initial diagnosis. HI at diagnosis (96/177) had major negative impact on longterm prognosis. Sellar masses with HI were associated with lower OS (0.84G0.04; PZ0.021), lower FC (PZ0.003), and higher BMI at diagnosis and last follow-up (PZ0.000) when compared with sellar masses without HI (OS: 0.94G0.05). PFS was not affected by HI or degree of resection. Conclusions: Initial HY has no impact on outcome in patients with sellar masses. OS and FC are impaired in survivors presenting with initial HI. PFS is not affected by HY, HI, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial HI to prevent further hypothalamic damage.

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“…Mortality rates are affected by initial treatment. For example, for patients treated between 1966 and 1992, the 5-year progression-free survival was 89% for patients treated with radiotherapy and conservative surgery compared with 81% after complete excision and 53% after partial excision (10). The comparable figures at 10 years were 77%, 69% and 37% respectively.…”

Section: Discussionmentioning

confidence: 99%

‘Do no harm’: management of craniopharyngioma

Gleeson¹,

Amin²,

Maghnie

2008

European Journal of Endocrinology

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Craniopharyngioma management is challenging. Although histology is benign, the tumour can be clinically aggressive with local invasion and frequent recurrences. Extensive morbidity may be present at diagnosis and furthermore, occurs as a consequence of neurosurgery and radiotherapy. Hypothalamic symptoms can have a devastating effect on quality of life and may reduce life expectancy. This case highlights both the challenge of managing hyperphagia and morbid obesity and the importance of initial treatment preserving existing hypothalamic function and the need to avoid tumour recurrence and further surgery.A 11-year old boy presented with hydrocephalus secondary to a craniopharyngioma (he had visual failure and hypopituitarism but few hypothalamic symptoms). He underwent radical resection followed by radiotherapy. Following this treatment, he developed psychological and behavioural problems and hyperphagia. Weight gain in the first year (an increase from C1.4 to C3.7 S.D.) resulted in poor mobility and a fall which caused a slipped femoral epiphysis. In the next year, there was a 6-month period of unexpected weight loss (C4.2 to C3.8 S.D.) that culminated in emergency treatment for diabetic ketoacidosis secondary to severe insulin resistance. He developed a left hemiplegia, and a subsequent cerebral angiogram identified multiple stenoses of the Circle of Willis with a Moyamoya appearance secondary to radiotherapy. Weight gain has continued (C3.8 to C5.5 S.D.) so that bariatric surgery is a management option.European Journal of Endocrinology 159 S95-S99

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Craniopharyngioma — long-term results following limited surgery and radiotherapy

Cited by 301 publications

References 54 publications

Expression and Growth Dependency of the Insulin-Like Growth Factor I Receptor in Craniopharyngioma Cells: A Novel Therapeutic Approach

Expression and Growth Dependency of the Insulin-Like Growth Factor I Receptor in Craniopharyngioma Cells: A Novel Therapeutic Approach

Hydrocephalus and hypothalamic involvement in pediatric patients with craniopharyngioma or cysts of Rathke's pouch: impact on long-term prognosis

‘Do no harm’: management of craniopharyngioma

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