Application of the Rotterdam postoperative cerebellar mutism syndrome prediction model in patients undergoing surgery for medulloblastoma in a single institution

Savannah Bush University of Tennessee, School of Medicine, Memphis;

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Paul Klimo Jr. Department of Neurosurgery, University of Tennessee, Memphis;

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Arzu Onar-Thomas Departments of Biostatistics,

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Jie Huang Departments of Biostatistics,

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Frederick A. Boop Pediatric Global Medicine, and

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Amar Gajjar Division of Neuro-Oncology, and

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Giles W. Robinson Division of Neuro-Oncology, and

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Raja B. Khan Division of Neurology, St. Jude Children’s Research Hospital, Memphis, Tennessee

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OBJECTIVE

Postoperative cerebellar mutism syndrome (CMS) develops in up to 40% of children with medulloblastoma. The Rotterdam model (RM) has been reported to predict a 66% risk of CMS in patients with a score of ≥ 100. The aim of this study was to retrospectively apply the RM to an independent cohort of patients with newly diagnosed medulloblastoma and study the applicability of the RM in predicting postoperative CMS.

METHODS

Participants had to have their first tumor resection at the authors’ institution and be enrolled in the SJMB12 protocol (NCT01878617). All participants underwent structured serial neurological evaluations before and then periodically after completing radiation therapy. Imaging was reviewed by the study neurologist who was blinded to CMS status when reviewing the scans and retrospectively applied RM score to each participant.

RESULTS

Forty participants were included (14 females and 26 males). Four (10%) patients had CMS. The median age at tumor resection was 11.7 years (range 3.5–17.8 years). Tumor location was midline in 30 (75%), right lateral in 6 (15%), and left lateral in 4 (10%). The median Evans index was 0.3 (range 0.2–0.4), and 34 (85%) patients had an Evans index ≥ 0.3. Five participants required a ventricular shunt. The median tumor volume was 51.97 cm3 (range 20.13–180.58 cm3). Gross-total resection was achieved in 35 (87.5%) patients, near-total resection in 4 (10%), and subtotal in 1. The median RM score was 90 (range 25–145). Eighteen participants had an RM score of ≥ 100, and of these 16.7% (n = 3) had CMS. Of the 22 patients with an RM score < 100, 1 child developed CMS (4.5%, CI 0.1%–22.8%); 3 of the 18 patients with an RM score ≥ 100 developed CMS (16.7%, CI 3.6%–41.4%). The observed rate of CMS in the cohort of children with an RM score ≥ 100 was significantly lower than the observed rate in the original RM cohort (66.7%, CI 51%–80.0%, p < 0.001). A greater risk of CMS in patients with an RM score ≥ 100 could not be confirmed (p = 0.31).

CONCLUSIONS

At the authors’ institution, the incidence of CMS in patients who had an RM ≥ 100 was significantly lower than the RM cohort. These findings raise questions regarding generalizability of RM; however, fewer cases of CMS and a relatively small cohort limit this conclusion.

ABBREVIATIONS

CMS = cerebellar mutism syndrome; CMS1 = type 1; CMS2 = CMS type 2; GTR = gross-total resection; d(sagittal) = depth of invasion and/or compression of the brainstem by tumor; D(sagittal) = line connecting the upper and lower points of the brainstem invaded by tumor; MCP = middle cerebellar peduncle; NTR = near-total resection; NWNS = non-WNT/non-SHH; RM = Rotterdam model; SCP = superior cerebellar peduncle; SHH = sonic hedgehog; STR = subtotal resection.
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Figure from Gupta et al. (pp 127–136).

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