Spontaneous Regression of Low-Grade Gliomas in Pediatric Patients without Neurofibromatosis

Rozen, Warren M.; Joseph, Samuel; Lo, Patrick

doi:10.1159/000134925

Cited by 60 publications

(25 citation statements)

References 43 publications

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“…Although their clinical behavior can vary, the majority of low-grade gliomas are indolent and do not undergo malignant transformation. Case reports have even described spontaneous regression of some tumors 1,2. This is in contrast to adult low-grade gliomas that have a more aggressive phenotype.…”

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confidence: 99%

Pediatric Low-Grade Gliomas

2009

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Pediatric low-grade gliomas encompass a heterogeneous set of tumors of different histologies. Cerebellar pilocytic astrocytomas occur most frequently followed by supratentorial diffuse fibrillary astrocytomas. Recent research has implicated activation of the RAS/RAF/MEK pathway in tumorigenesis of these tumors. Surgery is the mainstay of therapy. Overall survival rates for patients whose tumors are completely resected are 90% or greater, 10 years from diagnosis. Conversely, most optic pathway/hypothalamic, deep midline, and brain stem gliomas have minimal potential for resection; these tumors can be difficult to treat and deserve special attention. Combination chemotherapy is currently recommended as front-line adjuvant treatment for progressive or recurrent tumors. Second-line radiotherapy can also improve overall survival but is associated with more frequent and significant neurocognitive, endocrine, and other long-term toxicities.Keywords low-grade; glioma; astrocytoma; pilocytic; fibrillary Pediatric low-grade gliomas are a heterogeneous set of tumors. They encompass tumors of astrocytic, oligodendroglial, and mixed glial-neuronal histology. Although their clinical behavior can vary, the majority of low-grade gliomas are indolent and do not undergo malignant transformation. Case reports have even described spontaneous regression of some tumors. 1,2 This is in contrast to adult low-grade gliomas that have a more aggressive phenotype. One reason for the differences between the 2 populations may be the different frequencies of histological subtypes. Pilocytic astrocytomas infrequently occur in adults but are the leading histology in children. Conversely, diffuse gemistocytic astrocytomas, which have been associated with an increased tendency toward malignant progression, are rarely found in children. 3 Low-grade gliomas are estimated to account for anywhere from 30% to 50% of central nervous system tumors in children. [4][5][6] Discussion of gliomas can be confusing as it is a descriptive and not a pathological term. Tumors are classified according to the World Health Organization (WHO) criteria, most recently published in 2007, which describes their histological features and also provides a grading or "malignancy scale." 3 Low-grade gliomas encompass grade 1 and grade 2 tumors (Table 1). 3 Grading is based on a number of factors including presence of necrosis, giant cells, mitosis, endothelial proliferation, hyperchromatic nuclei, and pleomorphic cells. These findings can be subjective, and retrospective studies have documented significant Pediatric low-grade gliomas can be difficult to categorize as they can occur anywhere in the central nervous system and comprise multiple different tumor histologies. Historically, the cerebellum is the most prevalent location, and cerebellar low-grade gliomas account for 15% to 25% of all pediatric central nervous system tumors. They are followed by hemispheric (cerebral) gliomas (10%-15%), gliomas of the deep midline structures (10%-15%), optic pathway gliomas (5%)...

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confidence: 99%

Pediatric Low-Grade Gliomas

2009

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“…Furthermore, spontaneous regression of low-grade glial lesions [27] is a rare but well recognized phenomenon [28], already described in children with [29] or without NF1 [30,31] and mainly after surgical intervention of biopsy/resection. Unfortunately, main limit of our description is the absence of the histopathologic characterization of the lesion in the cerebellum.…”

Section: Discussionmentioning

confidence: 99%

Congenital cystic eye associated with a low-grade cerebellar lesion that spontaneously regressed

et al. 2014

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BackgroundCongenital cystic eye is an exceedingly rare ocular malformative disease, originated from the failure in the invagination of the optic vesicle during the fetal period and it can be associated with other ocular and non-ocular abnormalities. Diagnosis is based on clinical, radiological and histological features.Case presentationWe report a case of a congenital cystic eye associated with a cerebellar lesion accidentally detected at magnetic resonance imaging. Biopsy of the mass has not been performed due to parental rejection. Based on radiologic features and absence of clinical signs, a low-grade glioma diagnosis was hypothesized, but histological characterization was not obtained. Follow-up neuro-imaging 6 months after diagnosis showed that intracranial lesion spontaneously regressed without any treatment.ConclusionOur report stresses the importance of early MRI in children with ocular malformations, in order to detect associated intracranial defects, also of non-malformative origin. Additionally, we debate the clinic-radiological features of the intracranial lesions that could allow a wait-and-see policy. We also recommend a strict clinical and neuro-imaging follow-up for these lesions. Finally, biological mechanisms at the base of spontaneous regression of the brain lesions are discussed.

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“…Case reports have even described spontaneous regression of some tumors (Rozen, Joseph & Lo, 2008;Parsa, Hoyt, Lesser, Weinstein, Strother, Hoyt, 2001). This is in contrast to adult…”

Section: Childhoodmentioning

confidence: 98%

Primary CNS Tumors and Leptomeningeal, Disseminated and/or Multicentric Disease in Children Treated in Phase II Studies with Antineoplastons A10 and AS2-1

Burzynski¹,

Janicki²,

Burzynski³

2016

CCO

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It is estimated that as many as 30% of patients with primary CNS tumors have leptomeningeal, disseminated, and/or multicentric disease (LDM). These patients respond poorly to conventional therapy. Fifty-seven children with LDM (median age of 7.1 years) were treated in multiple prospective phase II clinical studies of high-and low-grade primary CNS tumors with Antineoplastons A10 and AS2-1 (ANP). Their inclusion in this analysis was based on MRI imaging. Patients with glioblastoma were excluded. The patients received ANP therapy 6 times daily; A10: 8.77 g/kg/d; AS2-1: 0.35 g/kg/d. The response to ANP was monitored by MRIs every 8 weeks.Patients evaluable for efficacy (N = 40) received 12 or more weeks of ANP or developed progressive disease (PD) before 12 weeks. 10 patients (17.5%) achieved an objective response (OR) with 4 (7%) achieving a complete response (CR) and 6 (10.5%) had a partial response (PR). Stable disease (SD) was maintained in 7 patients (12.3%) and PD developed in 23 patients (40.4%). Survival analysis of the 57 children showed 2-and 5-year overall survival (OS) were both 28% while 10-and 15-year OS were both 26%. One of the patients achieving an OR had atypical teratoid/rhabdoid tumor (AT/RT) while nine had low-grade gliomas (LGGs). Grade 3 and 4 toxicities included hypokalemia (14.0%); fatigue, anemia, hypernatremia and leukopenia (3.5% each); diarrhea, hypertension, joint pain, thrombocytopenia, and somnolence (1.8% each). These findings suggest the need for a single-arm, phase II study of ANP in children with LDM.

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Spontaneous Regression of Low-Grade Gliomas in Pediatric Patients without Neurofibromatosis

Cited by 60 publications

References 43 publications

Pediatric Low-Grade Gliomas

Pediatric Low-Grade Gliomas

Congenital cystic eye associated with a low-grade cerebellar lesion that spontaneously regressed

Primary CNS Tumors and Leptomeningeal, Disseminated and/or Multicentric Disease in Children Treated in Phase II Studies with Antineoplastons A10 and AS2-1

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