Management of Primary Tectal Plate Low-Grade Glioma in Pediatric Patients: Results of the Multicenter Treatment Study SIOP-LGG 2004

Kaufmann, Ariane; Gerber, Nicolas U.; Kandels, Daniela; Azizi, Amedeo A.; Schmidt, René; Warmuth‐Metz, Monika; Pietsch, Torsten; Kortmann, Rolf‐Dieter; Gnekow, Astrid; Grotzer, Michael A.

doi:10.1055/s-0038-1660503

Cited by 18 publications

(40 citation statements)

References 37 publications

(61 reference statements)

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“…Incompletely resected tumors tend to progress independent of location, but time to progression and proportion of patients progressing relate to the size of the postoperative residue with smaller residues progressing later and less frequently [41,134]. As an example, the probability not to experience tumor progression during observation or start of non-surgical treatment (event-free survival, EFS) is above 50 % at 10 years for small tumors of the tectal plate, while it declines to 12 % for the larger ones [67]. If repeat-surgery is possible in case of relapse/progression, observation-only is again justified for patients without significant tumor-related symptoms.…”

Section: Observation Groupmentioning

confidence: 99%

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SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

et al. 2019

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Low grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Around 40% of all LGG patients can be cured by complete neurosurgical resection and are followed by close observation. In case of relapse, second resection often is possible. Following incomplete resection observation is recommended, as long as there is no radiologic tumor growth and the patient does not suffer from significant, tumor-related symptoms. This also applies to patients with a diagnosis of LGG on the basis of radiological criteria. By contrast, clinical worsening and / or radiologic progression are an indication to treatment with either chemo- or radiotherapy. Overall survival is around 90%, and many patients survive with residual tumor, i. e. they suffer from chronic disease. All patients need comprehensive neuro-oncological care, the principles and details of which are summarized in the current guidelines. These represent standard of care for diagnostic work-up (including neuroimaging and neuropathology), and for therapeutic decisions (including the indications to non-surgical treatment) as well as concepts for neurosurgical intervention, chemotherapy and radiotherapy as well as surveillance and rehabilitation. The current treatment algorithm was compiled by members of the LGG working group of the SIOP-E brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports.

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Section: Observation Groupmentioning

confidence: 99%

“…However, transient increase of tumor size with stable disease thereafter is possible in all regions of the brain. For patients with tumors of the brain stem or tectal plate it was seen in 10 % [67]. Therefore, it may be necessary to confirm continuous progression by follow-up scans.…”

Section: Radiologic Progressionmentioning

confidence: 99%

SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

et al. 2019

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“…and Sandri et al . to the exclusion of mesencephalic tumors where aqueduct obstruction causes symptoms of increased cranial pressure in more than 80% …”

Section: Discussionmentioning

confidence: 99%

“…The natural course of LGG disease is more favorable for cerebral and cerebellar tumor location and after complete resection . In contrast, OS of supratentorial midline and brainstem tumors depends on multi‐modal treatment strategies . Long‐term prognosis for brainstem LGG disease was found to be lower than for cerebral or cerebellar sites in some cohorts .…”

Section: Introductionmentioning

confidence: 99%

Favorable prognosis in pediatric brainstem low‐grade glioma: Report from the German SIOP‐LGG 2004 cohort

Holzapfel

Kandels

Schmidt

et al. 2019

Intl Journal of Cancer

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Reports on pediatric low-grade glioma (LGG) of the caudal brainstem are retrospective with heterogeneous cohorts, variable treatments and inconsistent outcome data. We analyzed their natural history and asked whether brainstem location proved unfavorable for survival within the framework of the comprehensive SIOP-LGG 2004 management strategy. Within the prospectively registered, population-based German SIOP-LGG 2004 cohort 116 patients (age 0.2-16.5 years, 10% Neurofibromatosis NF1) were diagnosed with LGG of the pons (27%) and medulla oblongata (73%). After biopsy (23%), variable resection (63%) or radiologic diagnosis only (14%), 59 patients received no adjuvant treatment. Radiologic progression or severe neurologic symptoms prompted chemo-(n = 39) or radiotherapy (n = 18). After further progression (28/57), salvage treatments included multiple treatment lines for 12/28 patients. Five-years event-free survival dropped to 0.40, while 5-years overall survival was 0.95 (median observation time 6.8 years). Higher extent of resection yielded lower progression rate (p = 0.001), but at a cost of 21/100 patients suffering from new postsurgical complications including respiratory insufficiency. Central review confirmed pilocytic astrocytoma (56%), diffuse astrocytoma (8%) or glioneuronal histology (16%) (others 4%, no histology 17%). Malignant evolution was documented in five patients associated with Histone3 mutation in 2/5. Our treatment algorithm conveyed high overall survival for pediatric brainstem LGG. Extensive neurosurgical resection did increase additional postoperative neurologic deficits but not overall survival in this often-chronic disease. More than half of all patients can be Additional Supporting Information may be found in the online version of this article.safely followed by observation, while multimodal adjuvant treatment can control progressive tumors. Molecular assessment should confirm low-grade diagnosis and may detect patterns prognostic for malignant evolution. What's new?Outcome studies for pediatric low-grade gliomas (LGG) of the brainstem have generally been small and of low quality, making it difficult to determine prognosis. In this prospective, multicenter study, the authors developed a detailed description of the natural history, treatment response, and prognostic factors associated with favorable survival when a comprehensive treatment strategy was used. This treatment algorithm resulted in high overall survival for pediatric brainstem LGG. The study also emphasizes the importance of the predictive value of molecular patterns, as malignant evolution can occur even in lowgrade tumors.Favorable prognosis in pediatric brainstem LGG Int.

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“…[11,[13][14][15][16]Thus, for most of these patients, the treatment of hydrocephalus is enough. [4,17] ETV and shunt placement are currently the most widely used methods to relieve hydrocephalus. However, the risk of postoperative complications with shunt surgery is significantly higher than that of ETV.…”

Section: Etv Can Effectively Relieve the Symptoms Of Obstructive Hydrmentioning

confidence: 99%

Surgical Management and Prognostic Factors Based on 50 Tectal Plate Gliomas

Kang

Zhao

et al. 2020

Preprint

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BackgroundThe management and prognostic factors of tectal glioma (TG) remain ambiguous, because it is an extremely rare neoplasm that occurs predominantly in the pediatric population. The objective of this study was to evaluate the risk factors for progression-free survival (PFS) in TG patients after ETV operation, elucidate the radiological features of TG, and propose a treatment protocol.MethodsFrom 2002 to 2018, 50 patients that preoperative imaging manifestations were low-grade TGs were treated at our institute. Clinical features, treatments, radiologic findings, biopsies, and pertinent risk factors were evaluated.ResultsA total of 50 patients with a diagnosis of TG were identified. Twenty-six (52%) patients were males. The median age at diagnosis was 11.5 years (range 0.5–19 years). All patients had symptoms related to obstructive hydrocephalus and were treated with endoscopic third ventriculostomy (ETV). After a median follow-up duration of 59 months (range 11.0–208.0 months), progression occurred in six patients (12%), with a median PFS time of 18.0 months (range 4.0–56.0 months). Twelve patients (24%) underwent a biopsy, one patient (8.3%) was diagnosed with anaplastic oligodendroglioma, one patient (8.3%) was diagnosed with astrocytoma (WHO grade II-III), five patients (41.7%) were diagnosed with pilocytic astrocytoma, and the type of tumor could not be confirmed in five patients (41.7%) due to the small amount of tumor sample, thus, these patients were diagnosed with gliosis. PFS rates at 1 and 5 years were 91.2% ± 4.2% and 84.9% ± 5.9%, respectively. A multivariate model demonstrated that a large tumor size and cystic changes are risk factors for progression.ConclusionETV has been uniformly successful in the management of hydrocephalus caused by TG. A large tumor size and cystic changes are risk factors for progression. Under the condition of safety, a biopsy should be performed. For patients with low-grade TG, ETV is often the only surgical procedure that most patients require.HighlightsTectal gliomas are generally low-grade gliomas with a favorable prognosis.The only surgical procedure that most patients with tectal glioma require is ETV.Under the condition of safety, neuroendoscopy for a pathological diagnosis should be performed.A large tumor size and cystic changes are risk factors for progression.

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Management of Primary Tectal Plate Low-Grade Glioma in Pediatric Patients: Results of the Multicenter Treatment Study SIOP-LGG 2004

Abstract: More than half of patients were managed without tumor treatment. Favorable prognostic factors for EFS were small initial tumor volume (≤3cm) and the absence of initial contrast enhancement on MRI. Overall survival was excellent.

Cited by 18 publications

References 37 publications

SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

Favorable prognosis in pediatric brainstem low‐grade glioma: Report from the German SIOP‐LGG 2004 cohort

Surgical Management and Prognostic Factors Based on 50 Tectal Plate Gliomas

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