Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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).</jats:p> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p></jats:sec>

The Epidemiological Characteristics and Prognostic Factors of Low-Grade Brainstem Glioma: A Real-World Study of Pediatric and Adult Patients

“…Teo and Siu (23) reported a 100% 5-year OS rate of 23 pediatric LGBSG patients treated with endoscope-assisted microsurgery. Lundar et al (14) performed resections on 15 pediatric patients diagnosed with low-grade midbrain glioma. They reported prolonged survival period of the patients.…”

“…And no study focusing on adult LGBSG has been published to date. Regarding its treatment modalities, surgical resection has improved with advancing imaging and neurosurgery techniques (12)(13)(14). At the same time, efforts were devoted to investigate the adjuvant therapy including radiotherapy (RT) and chemotherapy (CT) for LGBSG patients (15)(16)(17)(18)(19).…”

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

“…Anatomical site determines resectability, which, in turn, when complete, is associated with better survival outcomes in both low‐ and high‐grade glioma . Improvement of surgical techniques, use of intraoperative adjuncts, especially neurophysiological monitoring, and referral to specialized pediatric neurosurgical centers contributed to reduced postoperative morbidity after resection for brainstem LGG . Complete and subtotal resection rates of 16–53% and 24–56%, respectively, were reported in small monocentric series, but were clearly lower in our cohort with 10% and 16%, respectively.…”

Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas

“…With the exception of tectal gliomas, tissue diagnosis is necessary and resection is often a treatment option along with chemotherapy or radiation therapy. 25,30 Although tumor progression may occur, these children have an excellent chance of enjoying a long and functional life. 25,30 Non-DIPG malignant brainstem tumors represent the third group of tumors that are exceedingly rare, can be focal or diffuse, exophytic or wholly intrinsic, and represent a spectrum of pathologies.…”

“…25,30 Although tumor progression may occur, these children have an excellent chance of enjoying a long and functional life. 25,30 Non-DIPG malignant brainstem tumors represent the third group of tumors that are exceedingly rare, can be focal or diffuse, exophytic or wholly intrinsic, and represent a spectrum of pathologies. There are but a few case reports or small case series in the literature of embryonal tumors, with the most commonly described example of this group being a primitive neuroectodermal tumor (PNET).…”