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Lateral-type posterior fossa ependymomas in pediatric patients: a national collaborative study

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Abstract

Purpose

To review a multicentric series of lateral-type posterior fossa ependymomas operated in the last ten years and to analyze the factors related to clinical evolution and tumor survival.

Methods

Descriptive, retrospective study. Active members of the Spanish Society of Pediatric Neurosurgery were invited to participate in this multicentric study. Clinical and radiological data were incorporated to an open database. The role of histologic grade, grade of resection, postoperative morbidities, and clinical follow-up was evaluated through bivariate associations (chi-square), Kaplan–Meier’s curves (log-rank test), and multivariate analysis (binary logistic regression).

Results

Fourteen centers entered the study, and 25 cases with a minimum follow-up of 6 months were included. There were 13 boys and 12 girls with a mean age close to 3 years. Mean tumor volume at diagnosis was over 60 cc. A complete resection was achieved in 8 patients and a near-total resection in 5 cases. Fifteen tumors were diagnosed as ependymoma grade 2 and ten as ependymoma grade 3. Major morbidity occurred postoperatively in 14 patients but was resolved in twelve within 6 months. There were six cases of death and 11 cases of tumor progression along the observation period. Mean follow-up was 44.8 months. Major morbidity was significantly associated with histologic grade but not with the degree of resection. Overall and progression-free survival were significantly associated with complete surgical resection. At the last follow-up, 16 patients carried a normal life, and three displayed a mild restriction according to Lansky’s scale.

Conclusions

Lateral-type posterior fossa ependymomas constitute a specific pathologic and clinical tumor subtype with bad prognosis. Gross total resection is the goal of surgical treatment, for it significantly improves prognosis with no additional morbidity. Neurological deficits associated to lower cranial nerve dysfunction are common, but most are transient. Deeper genetic characterization of these tumors may identify risk factors that guide new treatments and stratification of adjuvant therapies.

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Data Availability

Data are available through the secretary of the spanish pediatric of neurosurgery.

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Funding

No funding was obtained for this study.

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Authors and Affiliations

  1. Unidad de Neurocirugía Pediátrica, Hospital Universitario y Politécnico La Fe, Avda Fernando Abril Martorell, 106, 46026, Valencia, Spain

    Pablo Miranda-Lloret, Estela Plaza-Ramírez, Antonio López-Guerrero, Diego López-Bermeo, Teresa García-Campos, Silvia Vázquez-Sufuentes, Pablo M. Munárriz, Elena López-García, Alejandra Londoño-Quiroz, Cristina Ferreras-García, Mario García-Conde, Javier Saceda-Gutiérrez, Jorge Giménez-Pando, Giovanni Pancucci & Sara Iglesias-Moroño

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  1. Pablo Miranda-Lloret
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Contributions

Pablo Miranda, Estela Plaza and Giovanni Pancucci contributed with 7 cases to the series; Antonio López-Guerrero and Diego López-Bermeo contributed each with 3 cases to the series; Teresa García Campos and Silvia Vázquez Sufuentes contributed with 2 cases to the series; Pablo M. Munárriz, Elena López-García, Alejandra Londoño-Quiroz, Cristina Ferreras-García, Mario García-Conde, Javier Saceda-Gutiérrez, Jorge Giménez-Pando and Sara Iglesias-Moroño contributed each with one case to the series. Pablo Miranda gathered the information and wrote the manuscript. All authors reviewed and approved the text. Pablo M. Munárriz made significant corrections to the manuscript text.

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Correspondence to Pablo Miranda-Lloret.

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Miranda-Lloret, P., Plaza-Ramírez, E., López-Guerrero, A. et al. Lateral-type posterior fossa ependymomas in pediatric patients: a national collaborative study. Childs Nerv Syst 40, 407–416 (2024). https://doi.org/10.1007/s00381-023-06194-7

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