ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2018
Pediatric extraspinal sacrococcygeal ependymoma (ESE): an Italian AIEOP experience of six cases and literature review.
Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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13 citing papers in PubMed, 19 citations in OpenAlex.
- Extraspinal sacrococcygeal anaplastic ependymoma: A case report.International journal of surgery case reports · 2025Article
- Intraocular Myxo-Papillary Ependymoma versus Retinal Gliosis Mimicking Uveal Melanoma: Report of 2 Adult Patients with Clinical, Imaging, and Histopathologic Features.Ocular oncology and pathology · 2025Article
- Paediatric extra-axial myxopapillary ependymoma: what to expect? Case report and literature review.Frontiers in oncology · 2025Article
- Epidemiological characteristics of myxopapillary ependymoma and factors affecting overall survival: a SEER-based analysis.Translational cancer research · 2024Article
- When a dermatopathologist encounters the ultra-rare: A case series of superficial soft tissue/cutaneous myxopapillary ependymomas.Journal of cutaneous pathology · 2024Article
- Co-occurrence of subcutaneous myxopapillary ependymoma, dermal sinus tract, and filum terminale lipoma: a review of the pathobiology of caudal spinal cord development and spinal cord tethering. Illustrative case.Journal of neurosurgery. Case lessons · 2023Article
- Rare sacral extradural grade II ependymoma: a comprehensive review of literature.BMJ case reports · 2021Article
- Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review.Diagnostics (Basel, Switzerland) · 2021Article
- Article
- Surgical management of a rare myxopapillary ependymoma of the gluteal region: A case report.Surgical neurology international · 2021Article
- Diagnosis: subcutaneous myxopapillary ependymoma.Skeletal radiology · 2020Article
- An unexpected diagnosis of paediatric subcutaneous sacrococcygeal extraspinal ependymoma: lessons learnt and review of the literature.Journal of surgical case reports · 2020Article
- Clinicopathologic features of anaplastic myxopapillary ependymomas.Brain pathology (Zurich, Switzerland) · 2019Article
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7 authors at 5 institutions in 1 country.
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Abstract
backgroundPrimary pediatric extraspinal sacrococcygeal ependymoma (ESE) is a very rare disease, poorly described in literature, whose diagnostic, therapeutic, and follow-up approach is still controversial.
methodsWe describe six cases of pediatric ESE treated at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers in Italy since 1983, with a review of the literature.
resultsAll six patients had primary sacrococcygeal disease (two presacral and four subcutaneous) with median age of 10 years. Three patients were males, and two of them are metastatic at diagnosis; 3/6 had myxopapillary ependymoma grade I and 3/6 had classic ependymoma grade II. Five patients underwent surgical resection with complete removal only in one case with coccygectomy. Adjuvant chemoradiotherapy was administered to one metastatic patient obtaining a complete remission. Two patients relapsed at 3 and 8 years from diagnosis: they were treated with salvage chemotherapy (high-dose sequential chemotherapy with myeloablative regimen in one case), surgery, and radiotherapy achieving complete remission (CR). All six patients are in complete continuous remission (CCR) at a median follow-up of 12.8 years.
conclusionsPediatric patients with this peculiar disease need to be referred to specialized pediatric cancer centers that can provide multidisciplinary treatment after a centralized pathology review. Our experience highlights the role of chemotherapy and radiotherapy in adjuvant and relapse setting. The final prognosis is relatively optimistic, but with a careful follow-up due to the high risk of recurrence.
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