Pediatric Extraspinal Sacrococcygeal Ependymoma: A Structured Narrative Review of Published Cases and Diagnostic Challenges
- 1Pediatric Surgery Unit, Maternity and Children Department, University of Pisa, 56126 Pisa, Italy
- 2Division of Pediatric Surgery, Department of Medical Sciences, Surgical Sciences and Neurosciences, University of Siena, 53100 Siena, Italy
- 3Histopathology Department, Pisa University Hospital, 56126 Pisa, Italy
Abstract
Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often incorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies, and outcomes. Materials and Methods: A structured literature search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by the World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.
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BibTeX
@article{nativi2026pediatric,
title = {{Pediatric Extraspinal Sacrococcygeal Ependymoma: A Structured Narrative Review of Published Cases and Diagnostic Challenges}},
author = {Eleonora Nativi and Vittorio Maria Virgone and Elisa Bassan and Bianca Stefani and Chiara Cordola and Angela Pucci and Marco Ghionzoli},
journal = {Cancers},
year = {2026},
month = oct,
doi = {10.3390/cancers18213459},
url = {https://www.mdpi.com/2072-6694/18/21/3459},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Pediatric Extraspinal Sacrococcygeal Ependymoma: A Structured Narrative Review of Published Cases and Diagnostic Challenges
AU - Eleonora Nativi
AU - Vittorio Maria Virgone
AU - Elisa Bassan
AU - Bianca Stefani
AU - Chiara Cordola
AU - Angela Pucci
AU - Marco Ghionzoli
PY - 2026
DA - 2026/10/26
JO - Cancers
DO - 10.3390/cancers18213459
UR - https://www.mdpi.com/2072-6694/18/21/3459
AB - Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often incorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies, and outcomes. Materials and Methods: A structured literature search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by the World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.
SN - 2072-6694
ER -