Craniopharyngioma in childhood: our evidence-based approach to management

Thompson, Dominic; Phipps, Kim; Hayward, Richard

doi:10.1007/s00381-005-1210-9

Cited by 88 publications

(52 citation statements)

References 7 publications

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“…68 Likewise, operative mortality for pediatric craniopharyngiomas has steadily declined with time. 50,88,94,95 Nevertheless, most authors continue to report increased mortality, 94 morbidity, 14,22,94 and tumor recurrence 14,22,29,34,83,91 and decreased extent of resection 14,16,23,27,28,39,46,74,86 for larger tumors. Yaşargil et al 94 reported a direct correlation between increasing size and higher operative morbidity and mortality.…”

Section: Surgical Success and Functional Outcomementioning

confidence: 99%

“…Prior work by our group demonstrated decreased overall survival (OS) in patients with larger craniopharyngiomas. 19 Other authors report increased mortality, 94 increased neurological and hypothalamic morbidity, 14,22,94 decreased extent of resection and local disease control, 14,16,23,27,28,39,46,68,74,86,91 and higher rates of tumor recurrence 14,22,29,34,83,91 in larger tumors. Some authors, however, report tumor size or volume to have no impact on extent of resection, 2,4,50,88,95 rate of recurrence 49,63 or hypothalamic morbidity.…”

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confidence: 99%

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Surgical management of giant pediatric craniopharyngiomas

Elliott¹,

Wisoff

2010

PED

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Object Prior work by the authors' group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter). Methods Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment. Results Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%. Conclusions In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.

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Section: Surgical Success and Functional Outcomementioning

confidence: 99%

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confidence: 99%

Surgical management of giant pediatric craniopharyngiomas

Elliott¹,

Wisoff

2010

PED

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“…However, longer followup studies are needed to confirm its long-term effectiveness. [10][11][12][13][14]19,23,24,30,32,[35][36][37]40,41,57,60 implications for cP treatment strategy…”

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confidence: 99%

Growth patterns of craniopharyngiomas: clinical analysis of 226 patients

Pan¹,

Qi²,

Liu³

et al. 2016

PED

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OBJECT Craniopharyngiomas (CPs) are rare epithelial tumors that are often associated with an enigmatic and unpredictable growth pattern. Understanding the growth patterns of these tumors has a direct impact on surgical planning and may enhance the safety of radical tumor removal. The aim of this study was to analyze the growth patterns and surgical treatment of CPs with a focus on the involvement of the hypothalamopituitary axis and the relationship of the tumor to the arachnoid membrane and surrounding structures. METHODS Clinical data from 226 consecutive patients with primary CP were retrospectively reviewed. Tumor location and the relationship of the tumor to the third ventricle floor and the pituitary stalk were evaluated using preoperative MRI and intraoperative findings. A topographic classification scheme was proposed based on the site of tumor origin and tumor development. The clinical relevance of this classification on patient presentation and outcomes was also analyzed. RESULTS The growth of CPs can be broadly divided into 3 groups based on the site of tumor origin and on tumor-meningeal relationships: Group I, infrasellar/infradiaphragmatic CPs (Id-CPs), which mainly occurred in children; Group II, suprasellar subarachnoid extraventricular CPs (Sa-CPs), which were mainly observed in adults and rarely occurred in children; and Group III, suprasellar subpial ventricular CPs (Sp-CPs), which commonly occurred in both adults and children. Tumors in each group may develop complex growth patterns during vertical expansion along the pituitary stalk. Tumor growth patterns were closely related to both clinical presentation and outcomes. Patients with Sp-CPs had more prevalent weight gain than patients with Id-CPs or Sa-CPs; the rates of significant weight gain were 41.7% for children and 16.7% for adults with Sp-CPs, 2.2% and 7.1% for those with Id-CPs, and 12.5% and 2.6% for those with Sa-CPs (p < 0.001). Moreover, patients with Sp-CPs had increased hypothalamic dysfunction after radical removal; 39% of patients with Sp-CPs, 14.5% with Id-CPs, and 17.4% with Sa-CPs had high-grade hypothalamic dysfunction in the first 2 postoperative years (p < 0.001). CONCLUSIONS The classification of CPs based on growth pattern may elucidate the best course of treatment for this formidable tumor. More tailored, individualized surgical strategies based on tumor growth patterns are mandatory to provide long-term tumor control and to minimize damage to hypothalamic structures. Differences in the distribution of growth patterns between children and adults imply that hierarchical comparison is necessary when investigating outcomes and survival across treatment paradigms in patients with CP.

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“…The paediatric patients present with bigger tumours in a more acute fashion and often have significant and devastating hypothalamic problems related to their therapy. As a consequence, most paediatric neurosurgeons have abandoned the aggressive approach advocated by Hoffman [2] for a much more cautious one, as is demonstrated in the guidelines for management from the Great Ormond Street Children's Hospital neurosurgical group in London [6], which has the highest throughput of these paediatric cases in the UK.…”

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confidence: 99%