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Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Frič, Petter Brandal and Paulina Due-Tønnessen

OBJECTIVE

Ependymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.

METHODS

The authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.

RESULTS

The authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.

Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.

CONCLUSIONS

Pediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.

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Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Frič, Bård Krossnes, Petter Brandal, Einar Stensvold and Paulina Due-Tønnessen

OBJECTIVE

The authors conducted a study to delineate the long-term results of the surgical treatment of pediatric pleomorphic xanthoastrocytomas (PXAs).

METHODS

All consecutive children and adolescents (0–20 years) who underwent primary tumor resection for a PXA during the years 1972–2015 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.

RESULTS

Of the 12 patients, 8 patients were in the 1st decade of life and 4 in the 2nd. The male/female ratio was 6:6. No patient was lost to follow-up. One patient presented with severe progressive tumor disease and died within 3 months after repeated resection. Another child died 3 days following a second surgical procedure involving gross-total resection (GTR) 8 years after the initial operation. The other 10 patients were alive at the latest follow-up when they reached the median age of 34 years (range 11–60 years). The median follow-up duration was 22 years (range 2–41 years). Barthel Index score was 100 in all 10 survivors. A total 18 tumor resections were performed. Five patients underwent a second tumor resection after MRI/CT confirmed recurrent tumor disease, from 6 months up to 17 years after the initial operation. Only one of our patients received adjuvant therapy: a 19-year-old male who underwent resection (GTR) for a right-sided temporal tumor in 1976. This particular tumor was originally classified as astrocytoma WHO grade IV, and postoperative radiotherapy (54 Gy) was given. The histology was reclassified to that of a PXA. Seven of 8 children whose primary tumor resection was performed more than 20 years ago are alive as of this writing—i.e., 88% observed 20-year survival. These are long-term survivors with good clinical function and all are in full- or part-time work.

CONCLUSIONS

Pediatric patients with PXA can be treated with resection alone with rewarding results. Recurrences are not uncommon, but repeated surgery is well tolerated and should be considered in low-grade cases before adjuvant therapy is implemented. Follow-up including repeated MRI is important during the first postoperative years, since individual patients may have a more aggressive tumor course.

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Tryggve Lundar, Bernt Johan Due-Tønnessen, Arild Egge, David Scheie, Petter Brandal, Einar Stensvold and Paulina Due-Tønnessen

Object

The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma.

Methods

A series of 15 consecutive patients (age range 0–15 years) who underwent primary tumor resection for a low-grade midbrain glioma during the years 1989–2010 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.

Results

Of the 15 patients, 10 were in their 1st decade (age 0–9 years) and 5 were in their 2nd decade of life (age 10–15 years) at the time of surgery. The male/female ratio was 0.50 (5:10). No patients were lost to follow-up. One patient died in the postoperative period (32 days posttreatment). Another 2 patients died during follow-up. One patient succumbed to acute bleeding in the resection cavity 8 months after surgery, and the other died of shunt failure 21 years after initial treatment. Twelve patients are alive at the time of this writing, with follow-up periods from 3 to 24 years (median 8 years).

Among the 12 survivors, the Barthel Index scores were normal (100) in 11 patients and 80 in 1 patient. A total of 25 tumor resections were performed. In 1 patient, further resection was performed 5 days after initial resection due to MRI-confirmed residual tumor. Another 5 patients underwent repeat tumor resection after MRI-confirmed progressive tumor disease and clinical deterioration ranging from 3 months to 4 years after the initial operation. Three of these 5 patients also underwent a third resection, and 1 of the 3 underwent a fourth operation. Six children received adjuvant therapy: local radiotherapy in 2 patients, chemotherapy in 3 patients, and both in 1 patient. Twelve (80%) of the 15 patients needed treatment for persistent hydrocephalus.

Conclusions

Selected cases of low-grade midbrain gliomas may clearly benefit from resection with favorable results, even for prolonged periods. Three patients in the present series died, one of whom had a prolonged survival period of 21 years. Among the 12 survivors, stable long-term results appeared obtainable in at least 9. One patient died of acute hemorrhage 8 months after initial resection; otherwise, rapid tumor progression and death were not observed. Forty percent of the patients received adjuvant treatment, with local radiotherapy, chemotherapy, or both.