Minimal residual calcification and recurrence after gross-total resection of craniopharyngioma in children

Elliott, Robert E.; Moshel, Yaron A.; Wisoff, Jeffrey H.

doi:10.3171/2009.1.peds08335

Cited by 22 publications

(13 citation statements)

References 83 publications

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“…It should be noted that some patients in this series were included in other reports on various aspects of the management of craniopharyngiomas in children. [20][21][22][23]58,66,70,71 A GTR was the primary aim of treatment in all children and was defined as the lack of residual tumor on visual inspection intraoperatively and no residual soft-tissue mass or enhancement suspected to be tumor on postoperative imaging, as determined by an independent neuroradiologist. The outcome variables measured included BMI (weight [in kilograms]/height 2 [in square meters]) with Z scores (SDs), 56 endocrine replacement regimen, VA and VF testing performed pre-and postoperatively by pediatric ophthalmologists, neurological examination pre-and postoperatively, subsequent seizures requiring medications, and highest level of education attained and need for educational assistance.…”

Section: Patient Populationmentioning

confidence: 99%

Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

Elliott¹,

Sands

Strom³

et al. 2010

FOC

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Object Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise. Methods The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction). Results There was a significant increase in pituitary dysfunction following treatment—consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas—and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus. Conclusions Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors.

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Section: Patient Populationmentioning

confidence: 99%

Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

Elliott¹,

Sands

Strom³

et al. 2010

FOC

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“…Of note, some patients in this series were included in other reports on various aspects of the management of craniopharyngiomas in children. 30,93,119,122 Fourteen patients (16%) were from foreign countries. Followup data after 6 months were missing for an in ternational and a domestic patient and, therefore, they were excluded from longterm analyses.…”

Section: Methodsmentioning

confidence: 99%

Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children

Elliott¹,

Hsieh²,

Hochm

et al. 2010

PED

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158

148

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Object Optimal treatment of primary and recurrent craniopharyngiomas remains controversial. Radical resection and limited resection plus radiation therapy yield similar rates of disease control and overall survival. The data are much less clear for recurrent tumors. The authors report their experience with radical resection of both primary and recurrent craniopharyngiomas in children and compare the outcomes between the 2 groups. Methods A retrospective analysis was performed in 86 children younger than 21 years of age who underwent a total of 103 operations for craniopharyngioma between 1986 and 2008; these were performed by the senior author. The goal was resection with curative intent in all patients. Two patients were lost to follow-up and were excluded from analysis. The mean age at the time of surgery was 9.6 years, and the mean follow-up was 9.0 years. Results All 57 children with primary tumors underwent gross-total resection (GTR). A GTR was achieved in significantly fewer children with recurrent tumors (18 [62%] of 29). There were 3 perioperative deaths (3%). Tumor recurred after GTR in 14 (20%) of 71 patients. Overall survival and progression-free survival were significantly better in patients with primary tumors at time of presentation to the authors' institution. There were no significant differences in the neurological, endocrinological, visual, or functional outcomes between patients with primary and those with recurrent tumors. Factors negatively affecting overall survival and progression-free survival include subtotal resection (recurrent tumors only), tumor size ≥ 5 cm, or presence of hydrocephalus or a ventriculoperitoneal shunt. Prior radiation therapy and increasing tumor size were both risk factors for incomplete resection at reoperation. Conclusions In the hands of surgeons with experience with craniopharyngiomas, the authors believe that radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity. While GTR does not preclude recurrence and is more difficult to achieve in recurrent tumors, especially large and previously irradiated tumors, radical resection is still possible in patients with recurrent craniopharyngiomas with morbidity similar to that of primary tumors.

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“…Of note, some patients in this series were included in other reports on various aspects of the management of craniopharyngiomas in children. [19][20][21]92,93 Twenty-six patients had tumors greater than or equal to 5 cm in largest diameter in any plane on imaging and were included in this study. Gross-total resection was the primary aim of treatment in all children; GTR was defined as lack of residual tumor by visual inspection intraoperatively and no residual soft-tissue mass or enhancement suspicious for tumor on postoperative imaging as determined by an independent neuroradiologist.…”

Section: Methodsmentioning

confidence: 99%

“…Minimal residual calcification on postoperative CT without residual bulky tumor or contrast enhancement was still considered GTR. 20 Outcome variables measured included body mass ]) with Z-scores, 67 endocrine replacement regimen, status of pituitary stalk determined intraoperatively, results of visual acuity and visual field testing performed pre-and postoperatively by pediatric ophthalmologists, results of pre-and postoperative neurological examinations, complications, operative mortality (defined as death within 30 days of operation), subsequent seizures requiring medications, and the highest level of education and need for educational assistance. In addition to tumor size, the extent of tumor extension outside the suprasellar region (determined by the number of intracranial compartments involved) was also analyzed to determine its effect on outcome variables.…”

Section: Methodsmentioning

confidence: 99%

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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Minimal residual calcification and recurrence after gross-total resection of craniopharyngioma in children

Cited by 22 publications

References 83 publications

Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children

Surgical management of giant pediatric craniopharyngiomas

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