Management and outcome of focal low-grade brainstem tumors in pediatric patients: the St. Jude experience

Klimo, Paul; Panandiker, Atmaram S. Pai; Thompson, C.; Boop, Frederick A.; Qaddoumi, Ibrahim; Gajjar, Amar; Armstrong, Gregory T.; Ellison, David W.; Kun, Larry E.; Ogg, Robert J.; Sanford, Robert A.

doi:10.3171/2012.11.peds12317

Cited by 50 publications

(47 citation statements)

References 30 publications

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“…9 The present report summarizes our surgical experience in 15 consecutive pediatric cases of low-grade midbrain tumors treated over a 22-year period. Sixty-six of our 100 consecutive low-grade cerebellar astrocytomas reported on previously were operated on after the introduction of MRI-that is, within the same time period as the present series of midbrain tumors.…”

Section: Discussionmentioning

confidence: 99%

Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Lundar¹,

Due-Tønnessen²,

Egge³

et al. 2014

PED

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Object The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma. Methods A series of 15 consecutive patients (age range 0–15 years) who underwent primary tumor resection for a low-grade midbrain glioma during the years 1989–2010 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index. Results Of the 15 patients, 10 were in their 1st decade (age 0–9 years) and 5 were in their 2nd decade of life (age 10–15 years) at the time of surgery. The male/female ratio was 0.50 (5:10). No patients were lost to follow-up. One patient died in the postoperative period (32 days posttreatment). Another 2 patients died during follow-up. One patient succumbed to acute bleeding in the resection cavity 8 months after surgery, and the other died of shunt failure 21 years after initial treatment. Twelve patients are alive at the time of this writing, with follow-up periods from 3 to 24 years (median 8 years). Among the 12 survivors, the Barthel Index scores were normal (100) in 11 patients and 80 in 1 patient. A total of 25 tumor resections were performed. In 1 patient, further resection was performed 5 days after initial resection due to MRI-confirmed residual tumor. Another 5 patients underwent repeat tumor resection after MRI-confirmed progressive tumor disease and clinical deterioration ranging from 3 months to 4 years after the initial operation. Three of these 5 patients also underwent a third resection, and 1 of the 3 underwent a fourth operation. Six children received adjuvant therapy: local radiotherapy in 2 patients, chemotherapy in 3 patients, and both in 1 patient. Twelve (80%) of the 15 patients needed treatment for persistent hydrocephalus. Conclusions Selected cases of low-grade midbrain gliomas may clearly benefit from resection with favorable results, even for prolonged periods. Three patients in the present series died, one of whom had a prolonged survival period of 21 years. Among the 12 survivors, stable long-term results appeared obtainable in at least 9. One patient died of acute hemorrhage 8 months after initial resection; otherwise, rapid tumor progression and death were not observed. Forty percent of the patients received adjuvant treatment, with local radiotherapy, chemotherapy, or both.

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Section: Discussionmentioning

confidence: 99%

Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Lundar¹,

Due-Tønnessen²,

Egge³

et al. 2014

PED

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“…Gliomas are the most common brain tumor in children with brainstem gliomas accounting for about 10-20% of all pediatric central nervous system (CNS) tumors [1-3]. Children are typically less than ten years of age at diagnosis and the incidence is similar across genders.…”

Section: Introductionmentioning

confidence: 99%

Cognitive outcomes among survivors of focal low-grade brainstem tumors diagnosed in childhood

et al. 2016

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Objective Pediatric focal low-grade brainstem tumors are associated with excellent prognosis. Surgical resection and conformal radiation therapy are front-line treatment options; radiation therapy (RT) serves as an excellent treatment for disease progression. Given high survival rates and limited research regarding functional outcomes, the current study examined neurocognitive outcomes in a group of low-grade brainstem glioma survivors. Methods Forty-three survivors of focal low-grade brainstem gliomas underwent neurocognitive assessment (58% male; median=6.9 years at diagnosis; median=14.9 years at latest assessment). Treatment included combinations of surgery, chemotherapy, and RT with 70% ultimately receiving RT. Neurocognitive outcomes were evaluated through retrospective chart review. Results Intellectual and academic performance were significantly different from normative expectations (Full scale IQ=86.5±16.8; Reading Comprehension=91.3±16.4; Math Reasoning=88.2±18.9; reference group=100±15). Further, the percentage performing below average exceeded the expected 16% in the normative sample (Full scale IQ=43%; Reading Comprehension=37%; Math Reasoning=50%). Mean parent ratings did not reflect concerns regarding internalizing and externalizing behaviors or executive functioning (Internalizing=54.9±12.7; Externalizing=51.6±14.6, Global Executive Composite=57.1±16.0; reference group=50±10); however, the proportion with clinically elevated scores was higher than the expected 16% (Internalizing=42%; Externalizing=26%; Global Executive Composite=38%). Mean performance fell below average for visual-motor coordination (81.8±13.2) and parent ratings of adaptive functioning (73.4±24.2), with 65% and 62% falling outside the average range, respectively. There were no significant differences between those receiving and not receiving RT. Conclusions Multiple cognitive domains were significantly different from normative expectations. Despite focal disease and treatment targeting subcortical brain regions, neurocognitive risks exist that may impact treatment planning and caregiver education.

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“…Similar to our experience with focal brainstem tumors, RT is an excellent treatment option for CMTs. 26 It can be the primary or salvage treatment for low-grade tumors or it can be used in conjunction with chemotherapy for the primary treatment of malignant tumors. Among our patients with low-grade tumors who received RT (n = 16), there were only 2 failures.…”

Section: Discussionmentioning

confidence: 99%

Cervicomedullary tumors in children

McAbee

Modica

Thompson

et al. 2015

PED

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OBJECT Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. METHODS The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. RESULTS Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. CONCLUSIONS Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

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Management and outcome of focal low-grade brainstem tumors in pediatric patients: the St. Jude experience

Cited by 50 publications

References 30 publications

Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Cognitive outcomes among survivors of focal low-grade brainstem tumors diagnosed in childhood

Cervicomedullary tumors in children

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