Low-grade astrocytoma: a decade of experience at St. Jude Children's Research Hospital.

Gajjar, Amar; Sanford, Robert A.; Heideman, Richard L.; Jenkins, Jesse J.; Walter, Andrew W.; Li, Yulan; Langston, J. William; Muhlbauer, Michael S.; Boyett, James M.; Kun, Larry E.

doi:10.1200/jco.1997.15.8.2792

Cited by 206 publications

(150 citation statements)

References 29 publications

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“…9,13,22 Several authors who discuss the impact of age on prognosis, however, refer to series that include infratentorial as well as supratentorial low-grade astrocytomas. In our series, 32 children were 0-4 years old at the primary surgery.…”

Section: Surgical Resultsmentioning

confidence: 99%

Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients

Due-Tønnessen¹,

Lundar²,

Egge³

et al. 2013

PED

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Object The objective of this study was to delineate the long-term results of surgical treatment of pediatric low-grade cerebellar astrocytoma. Methods One hundred consecutive children and adolescents (0–19 years old) who underwent primary tumor resection for a low-grade cerebellar astrocytoma during the years 1980–2011 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 100 patients, 61 children were in the 1st decade, and 39 were 10–19 years old. The male/female ratio was 1.13:1 (53 males, 47 females). No patients were lost to follow-up. There were no deaths in this series and all 100 patients are currently alive. In 29 patients, the follow-up duration was less than 10 years, in 37 it was between 10 and 19 years, and in 34 it was between 20 and 31 years. The Barthel Index was 100 (normal) in 97 patients, 90 in 2 patients, and 40 in the last patient. A total of 113 tumor resections were performed. Two patients underwent further tumor resection due to MRI-confirmed residual tumor demonstrated on the immediate postoperative MR image (obtained the day after the initial procedure). Furthermore, 9 children underwent repeat tumor resection after MRI-confirmed progressive tumor recurrence up to 10 years after the initial operation. Two of these patients also underwent a third resection, without subsequent radiation therapy, and have experienced 8 and 12 years of tumor-free follow-up thereafter, respectively. A total of 15% of the patients required treatment for persistent hydrocephalus. Conclusions Low-grade cerebellar astrocytoma is a surgical disease, in need of long-term follow-up, but with excellent long-term results. Nine percent of the children in this study underwent repeated surgery due to progressive tumor recurrence, and 15% were treated for persistent hydrocephalus.

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Section: Surgical Resultsmentioning

confidence: 99%

Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients

Due-Tønnessen¹,

Lundar²,

Egge³

et al. 2013

PED

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“…21,26 Although it is accepted that radiotherapy can often be effective in controlling growth in these tumors, the late side effects of radiotherapy have led to a reduction in its use, particularly as most of these patients should be expected to survive into adulthood. There remains controversy over the precise role of neurosurgery, 16,18,19,24,35,[39][40][41][42]47 with many teams regarding this as an intervention of last resort with potentially unacceptable morbidity.…”

Section: Discussionmentioning

confidence: 99%

“…Historically, there has not been clear consensus over the role of biopsy at the start of therapy, with controversy as to whether biopsy is necessary at all (independent of NF1 status) if the imaging appearances are typical. 16,17,26,29,39 Histopathological diagnosis can certainly allow an informed discussion of the therapeutic options and prognosis, especially with the need to distinguish between pilocytic astrocytoma and pilomyxoid astrocytoma. It is therefore not unreasonable to consider an initial biopsy if the imaging appearances are atypical and if the biopsy can be performed with a satisfactory degree of safety.…”

Section: Neurosurgerymentioning

confidence: 99%

The role of surgery in optic pathway/hypothalamic gliomas in children

Goodden¹,

Pizer

Pettorini³

et al. 2014

PED

105

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Object Optic pathway/hypothalamic gliomas (OPHGs) are generally benign tumors situated in an exquisitely sensitive brain region. The location and natural history of OPHGs has led to much debate about optimal treatment. This paper revisits the role of and optimal timing of debulking surgery in OPHG. Methods This paper presents a series of cases managed by the neuro-oncology team at Alder Hey Children's Hospital and a single surgeon. Data were collected retrospectively for periods prior to 2009 and prospectively thereafter. Tailored treatment strategies were used, including observation and combinations of surgery, chemotherapy, and radiotherapy. Tumor control rates and outcomes are reviewed. Results Forty-two patients were treated between 1998 and 2011. Their median age at diagnosis was 5 years 7 months. Nineteen patients were positive for neurofibromatosis Type 1 (NF1) and 23 patients were negative for NF1. The median duration of follow-up was 77 months (range 21.8–142.3 months). Presenting symptoms included visual impairment (in 50% of cases), headache (in 24%), and hypothalamic/pituitary dysfunction (in 29%). Twenty-two debulking procedures were performed in 21 patients. Four biopsies (3 open, 1 endoscopic) were also performed. The histological diagnosis was pilocytic astrocytoma in 21 patients and pilomyxoid astrocytoma in 2 patients. Ten patients (Group 1) had primary surgical debulking alone and were then observed. Four patients (Group 2) had surgical debulking, plus planned chemotherapy within 3 months. Seven patients (Group 3) required surgical debulking for progressive disease following a variety of treatments. Patient age had the greatest impact on subsequent tumor progression. In total, 13 patients received chemotherapy, 4 on initial presentation, 4 in combination with surgery, and 5 for further tumor progression. Five patients were treated with radiotherapy, 3 prior to referral to Alder Hey. Eleven patients required shunt insertion for hydrocephalus. Vision was stabilized for 74% of patients. The number of patients with hypothalamic/pituitary dysfunction increased from 12 at presentation to 16 by the end of treatment. The overall survival rate was 93%. Three patients died—1 from tumor progression, 1 from infective complications from tumor biopsy, and 1 from a spontaneous posterior fossa hemorrhage. NF1 was associated with improved outcome—fewer patients required active intervention and rates of visual impairment and/or or hypothalamic/pituitary dysfunction were lower. Conclusions Good long-term survival and functional outcomes can be achieved in children with OPHG. Tumor control was achieved through an individualized approach using surgery, chemotherapy, or radiotherapy in varied combinations. The authors aim to limit radiotherapy to cases involving older children in whom other therapies have failed, due to the well-described and often devastating late effects associated with midline cranial irradiation. Surgery has a clear role for diagnosis, tumor control, and relief of mass effect. In particular, primary surgical debulking of tumor (without adjuvant therapy) is safe and effective. Recent advances in intraoperative MRI may add value and need further assessment.

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“…For patients with low-grade gliomas who can safely undergo resection, macroscopic total resection remains the preferred treatment. 2,33,34 For patients who have tumors that are not amenable to resection, the choice of therapy is more controversial. Although radiotherapy provides durable disease control with 5-year OS rates of 75-90% in patients with hypothalamic-optic pathway gliomas, 6,[35][36][37] concerns for the long-term neurocognitive and neuroendocrine sequelae of radiotherapy have led investigators to explore the use of chemotherapy, especially in younger patients.…”

Section: Outcomementioning

confidence: 99%

Outcome of children with centrally reviewed low‐grade gliomas treated with chemotherapy with or without radiotherapy on Children's Cancer Group high‐grade glioma study CCG‐945

Fouladi

Hunt

Pollack

et al. 2003

Cancer

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BACKGROUNDThe objectives of the current study were to determine the outcome of children who were treated with chemotherapy and radiotherapy on the Children's Cancer Group (CCG) high‐grade glioma protocol (CCG‐945) who were diagnosed with low‐grade gliomas on post hoc central pathologic review and to identify clinical and biologic features associated with prognosis.METHODSBetween 1985 and 1991, 250 children with institutionally classified high‐grade gliomas were enrolled on CCG‐945. Patients older than 24 months with intracranial lesions were assigned randomly to receive either lomustine, vincristine, and prednisone (control regimen) or the 8‐drugs‐in‐1‐day regimen (experimental regimen); younger patients and those with primary spinal cord tumors were assigned nonrandomly to the experimental regimen. Central independent review by 5 neuropathologists led to a reclassification of low‐grade glioma in 70 patients, who were the focus of the current study.RESULTSThe study involved 42 males and 28 females (median age, 7.7 years) with a median follow‐up of 10.4 years. At 5 years, the progression‐free survival (PFS) rate was 63% ± 6%, and the overall survival (OS) rate was 79% ± 5%, compared with a PFS rate of 19% ± 3% (P < 0.0001) and an OS rate of 22% ± 3% (P < 0.0001) in the remainder of the cohort. Significantly poorer 5‐year PFS was seen in children younger than 24 months, those with fibrillary astrocytoma, and those with posterior fossa tumors. Patients demonstrated a modest improvement in PFS but no improvement in OS compared with children with low‐grade gliomas who were treated with contemporary chemotherapy‐alone approaches.CONCLUSIONSThe current report calls attention to the importance of central pathologic review in large multiinstitutional trials of children with gliomas and suggests that aggressive front‐line combined chemoradiotherapy does not confer a survival advantage in this highly selected population of patients. Cancer 2003;98:1243–52. © 2003 American Cancer Society.DOI 10.1002/cncr.11637

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Low-grade astrocytoma: a decade of experience at St. Jude Children's Research Hospital.

Cited by 206 publications

References 29 publications

Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients

Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients

The role of surgery in optic pathway/hypothalamic gliomas in children

Outcome of children with centrally reviewed low‐grade gliomas treated with chemotherapy with or without radiotherapy on Children's Cancer Group high‐grade glioma study CCG‐945

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