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Dawn E. Saunders, Kim P. Phipps, Angela M. Wade, and Richard D. Hayward

Object

The authors sought to evaluate surveillance strategies for the detection and monitoring of residual and recurrent disease in children with cerebellar low-grade astrocytomas (CLGAs) treated surgically or with radiotherapy. Patients were divided into three groups: 1) those in whom a “complete” resection was achieved; 2) those with residual disease with no immediate adjuvant therapy; and 3) those who received radiotherapy for residual/recurrent disease.

Methods

Magnetic resonance (MR) imaging studies and clinical data obtained in children with CLGA who presented between January 1988 and September 1998 were reviewed. Eighty-four children were followed for a mean period of 73 months (range 2–159 months). One child died. Of the 70 children in whom a complete resection was achieved, nine (13%) developed a recurrence detected by surveillance imaging at 6, 8, 9, 9, 13, 27, 39, 44, and 47 months, respectively. Following an incomplete resection, radiologically detected tumor progression leading to further treatment was detected at 7, 9, 12, 13, and 20 months, respectively, and an additional six tumors regressed or stablized. In 11 of 12 children treated with radiotherapy, stabilization/regression occurred radiologically at a mean of 14.9 months.

Conclusions

The authors recommend surveillance MR imaging in children treated for CLGA at 6 months and 1, 2, 3.5, and 5 years following a complete resection and after radiotherapy performed either initially or following recurrence. For follow up of residual tumor, 6-month interval imaging for at least 3 years, yearly images for another 2 years, and subsequent 2-year imaging is recommended.

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Dawn E. Saunders, Kim P. Phipps, Angela M. Wade, and Richard D. Hayward

Object. The authors sought to evaluate surveillance strategies for the detection and monitoring of residual and recurrent disease in children with cerebellar low-grade astrocytomas (CLGAs) treated surgically or with radiotherapy. Patients were divided into three groups: 1) those in whom a “complete” resection was achieved; 2) those with residual disease with no immediate adjuvant therapy; and 3) those who received radiotherapy for residual/recurrent disease.

Methods. Magnetic resonance (MR) imaging studies and clinical data obtained in children with CLGA who presented between January 1988 and September 1998 were reviewed. Eighty-four children were followed for a mean period of 73 months (range 2–159 months). One child died. Of the 70 children in whom a complete resection was achieved, nine (13%) developed a recurrence detected by surveillance imaging at 6, 8, 9, 9, 13, 27, 39, 44, and 47 months, respectively. Following an incomplete resection, radiologically detected tumor progression leading to further treatment was detected at 7, 9, 12, 13, and 20 months, respectively, and an additional six tumors regressed or stablized. In 11 of 12 children treated with radiotherapy, stabilization/regression occurred radiologically at a mean of 14.9 months.

Conclusions. The authors recommend surveillance MR imaging in children treated for CLGA at 6 months and 1, 2, 3.5, and 5 years following a complete resection and after radiotherapy performed either initially or following recurrence. For follow up of residual tumor, 6-month interval imaging for at least 3 years, yearly images for another 2 years, and subsequent 2-year imaging is recommended.

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Dawn E. Saunders, Richard D. Hayward, Kim P. Phipps, Wui Khean Chong, and Angela M. Wade

Object. The goal of this paper was to review brain and spine images obtained in children with medulloblastomas to determine the risk factors for tumor recurrence and to assess the impact of surveillance imaging on patient outcomes among patients who remain alive 1 month postsurgery.

Methods. Imaging studies and clinical data obtained in children with medulloblastomas, who presented between January 1987 and August 1998, were retrospectively reviewed. Images were termed surveillance if they were follow-up studies and symptom prompted if they were obtained to investigate new symptoms.

One hundred seven patients (mean age 6 years and 3 months, range 2 months–15 years and 6 months) were entered into the study. Fifty-three children experienced tumor recurrence; 41 had one recurrence, nine had two, and three had three recurrences. Surveillance imaging revealed 10 of the first 53 recurrences and 15 of all 68 recurrences. When the first recurrence was identified by the emergence of symptoms (42 patients), the children tended to survive for a shorter time (hazard ratio 3.72, 95% confidence interval 1.42–9.76, p = 0.008) than children in whom the first recurrence was detected before symptoms occurred (10 patients). The median survival time following symptomatic tumor recurrence was 4 months and that after surveillance-detected tumor recurrence was 17 months. The median increased survival time among patients whose recurrence was asymptomatic and identified by imaging studies was 13 months, more than half the mean time between surveillance imaging sessions. Incomplete tumor resection was associated with a significantly reduced time to recurrence (p = 0.048) and to death (p = 0.002). The number of recurrences that were experienced was associated with a reduced time to death (p < 0.001).

Conclusions. Surveillance imaging is associated with an increase in survival in children with medulloblastomas. More frequent surveillance imaging in children with incomplete tumor excision and recurrent disease may further improve the length of survival.

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Catriona D. Good, Angela M. Wade, Richard D. Hayward, Kim P. Phipps, Anthony J. Michalski, William F.J. Harkness, and W.K. Chong

Object. The authors examined images obtained in 52 children with intracranial ependymomas to determine risk factors for tumor recurrence and to assess the impact of surveillance imaging on patient outcome.

Methods. Data obtained in all children with intracranial ependymomas were prospectively entered into a database from January 1987 to June 2000. The imaging and clinical details in all patients were reviewed. Fifty-two children with histologically proven intracranial ependymomas were treated at the authors' institution; recurrences developed in 28 (54%) of them, with a median time from surgery to first recurrence of 14.5 months (range 3–65 months). Of these tumor recurrences, 43% were asymptomatic and were noted on surveillance imaging. Seventeen children died, all of whom had recurrences. Incomplete excision of the primary tumor was significantly associated with reduced time to recurrence (p = 0.0144) and time to death (p = 0.0472). The age of the patient, location of the primary tumor, histological findings, and the presence or absence of spinal metastases on preoperative imaging were not significantly associated with outcome. The risk of death at any given time was 12-fold greater in patients in whom a recurrence was identified due to symptoms rather than on surveillance images (p = 0.016).

Conclusions. Recurrent childhood ependymoma has a poor prognosis. The extent of the initial local tumor resection is the factor most closely associated with outcome. Surveillance imaging reveals a substantial number of asymptomatic recurrences, and survival appears to be improved in these patients compared with those identified by symptoms. The improvement in survival is thought to be greater than that expected just from earlier diagnosis.

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Sebastian M. Toescu, Gargi Samarth, Hugo Layard Horsfall, Richard Issitt, Ben Margetts, Kim P. Phipps, Noor-ul-Owase Jeelani, Dominic N. P. Thompson, and Kristian Aquilina

OBJECTIVES

The goal of this study was to characterize the complications and morbidity related to the surgical management of pediatric fourth ventricle tumors.

METHODS

All patients referred to the authors’ institution with posterior fossa tumors from 2002 to 2018 inclusive were screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.

RESULTS

Three hundred fifty-four children with posterior fossa tumors were treated during the study period; of these, 185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis. One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30 cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases), with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ventricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventriculostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.

Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%); however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS; 28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant difference in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression modeling did not reveal any statistically significant relationships between CMS and surgical approach.

CONCLUSIONS

Surgical management of pediatric fourth ventricle tumors continues to evolve, and resection is increasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient population.