Recurrent pediatric central nervous system low-grade gliomas: the role of surveillance neuroimaging in asymptomatic children

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Pediatric low-grade glioma (LGG) is the most common brain tumor of childhood. Except for the known association of gross-total resection and improved survival rates, relatively little is known about the clinical and radiographic predictors of recurrent disease and the optimal frequency of surveillance MRI. The authors sought to determine the clinical and radiographic features associated with recurrent or progressive disease in a single-institutional series of children diagnosed with primary CNS LGG.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors performed a retrospective analysis of data obtained in 102 consecutive patients diagnosed at Rady Children's Hospital–San Diego between 1994 and 2010 with a biopsy-proven LGG exclusive of a diagnosis of neurofibromatosis. Tumor location, patient age, sex, and symptomatology were correlated with tumor progression or recurrence. Magnetic resonance imaging characteristics and neuroimaging surveillance frequency were analyzed in those children with progressive or recurrent disease.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Forty-six of 102 children diagnosed with an LGG had evidence of recurrent or progressive disease between 2 months and 11 years (mean 27.3 months) after diagnosis. In the larger group of 102 children, gross-total resection was associated with improved progression-free survival (p = 0.012). The location of tumor (p = 0.26), age at diagnosis (p = 0.69), duration of symptoms (p = 0.72), histological subtype (p = 0.74), sex (p = 0.53), or specific chemotherapeutic treatment regimen (p = 0.24) was not associated with tumor progression or recurrence. Sixty-four percent of children with recurrent or progressive disease were asymptomatic, and recurrence was diagnosed by surveillance MRI alone. All children less than 2 years of age in whom the tumor was diagnosed were asymptomatic at the time of progression (p = 0.04). Thirteen percent (6 of 46) of the children had disease recurrence 5 years after initial diagnosis; all of them had undergone an initial subtotal resection. Tumor progression was associated with either homogeneous or patchy T1-weighted post–Gd administration MRI enhancement in 94% of the cases (p = 0.0001).</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Children diagnosed with recurrent LGG may be asymptomatic at the time of recurrence. The authors' findings support the need for routine neuroimaging in a subset of children with LGGs, even when gross-total resection has been achieved, up to 5 years postdiagnosis. The authors found that T1-weighted MR images obtained before and after Gd administration alone may be sufficient to diagnose LGG recurrence and may represent an effective strategy worthy of further validation in a larger multiinstitutional cohort.</jats:p></jats:sec>

The utility of routine surveillance screening with magnetic resonance imaging (MRI) to detect tumour recurrence in children with low-grade central nervous system (CNS) tumours: a systematic review

“…The definition for radiographic recurrence was provided in all but two studies [ 10 , 12 ] but again definitions varied between studies. All but one study [ 13 ] reported details of the change in patient management/treatment post-recurrence. Definitions of survival outcome measures were provided in only half of the studies reporting survival, with one study failing to define OS [ 8 ] and one RFS [ 12 ] (see Online Resource 5 for quality assessment table).…”

“…The seven studies were published between 2001 and 2016 with six published since 2009 [ 9 – 14 ]. Five studies were undertaken in the USA [ 8 , 9 , 11 – 13 ], one in Canada [ 14 ] and one in Australia [ 10 ]. The studies (overall n = 469 patients) were comprised of six low grade tumour studies [ 9 – 14 ] and one with a mix of low and high-grade tumours [ 8 ].…”

“…Median follow-up ranged from 5.6 to 7 years. Three studies [ 9 , 11 , 13 ] explicitly excluded children with Neurofibromatosis Type 1, with the remaining studies providing no details of the NF1 status of their study populations. At the commencement of surveillance imaging, none of the patients had relapsed disease (See Table 1 ).…”

SIOP PODC Adapted treatment guidelines for low grade gliomas in low and middle income settings

“…Repeated relapse/progressive disease in children with LGG is common in incompletely resected/unresectable tumors. Children whose tumour involves the optic tract, those with multifocal tumors, those below 1 year of age and/ or those with evidence of dissemination at initial diagnosis tend to have a higher rate of progression than those with a single lesion …”

The impact of routine surveillance screening with magnetic resonance imaging (MRI) to detect tumour recurrence in children with central nervous system (CNS) tumours: protocol for a systematic review and meta-analysis

“…The impact of surveillance imaging has been assessed in children with different types of malignant CNS tumours including low- and high-grade gliomas [5–7], medulloblastoma [6, 8–10], ependymoma, [11] and central nervous system primitive neuroectodermal tumours (CNS-PNETs) [12]. However, differences in imaging schedules and modalities (including both computerised tomography (CT) and MRI), and a reliance upon case series studies, mean no consensus has been reached regarding the utility of surveillance imaging, the optimal interval for undertaking scans or the length of surveillance following diagnosis.…”