Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review
Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-09-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/11/9/1680 |
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| author | Francesco Fabozzi Silvia Ceccanti Antonella Cacchione Giovanna Stefania Colafati Andrea Carai Alessandro Crocoli Angela Mastronuzzi Denis A. Cozzi |
| author_facet | Francesco Fabozzi Silvia Ceccanti Antonella Cacchione Giovanna Stefania Colafati Andrea Carai Alessandro Crocoli Angela Mastronuzzi Denis A. Cozzi |
| author_sort | Francesco Fabozzi |
| collection | DOAJ |
| description | Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years. |
| first_indexed | 2024-03-10T07:45:21Z |
| format | Article |
| id | doaj.art-4c95687072be4af4ac96d436fa44ba83 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-10T07:45:21Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-4c95687072be4af4ac96d436fa44ba832023-11-22T12:40:45ZengMDPI AGDiagnostics2075-44182021-09-01119168010.3390/diagnostics11091680Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature ReviewFrancesco Fabozzi0Silvia Ceccanti1Antonella Cacchione2Giovanna Stefania Colafati3Andrea Carai4Alessandro Crocoli5Angela Mastronuzzi6Denis A. Cozzi7Department of Hematology/Oncology, Cell and Gene Therapy, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyPediatric Surgery Unit, Sapienza University of Rome, AOU Policlinico Umberto I, 00161 Rome, ItalyDepartment of Hematology/Oncology, Cell and Gene Therapy, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyNeuroradiology Unit, Department of Imaging, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyNeurosurgery Unit, Department of Neurosciences, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyDepartment of Pediatric Surgery, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyDepartment of Hematology/Oncology, Cell and Gene Therapy, IRCCS Bambino Gesù Children’s Hospital, 00165 Rome, ItalyPediatric Surgery Unit, Sapienza University of Rome, AOU Policlinico Umberto I, 00161 Rome, ItalyPrimary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years.https://www.mdpi.com/2075-4418/11/9/1680pediatric ependymomaextra-CNS ependymomamyxopapillary ependymomapilonidal disease |
| spellingShingle | Francesco Fabozzi Silvia Ceccanti Antonella Cacchione Giovanna Stefania Colafati Andrea Carai Alessandro Crocoli Angela Mastronuzzi Denis A. Cozzi Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review Diagnostics pediatric ependymoma extra-CNS ependymoma myxopapillary ependymoma pilonidal disease |
| title | Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review |
| title_full | Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review |
| title_fullStr | Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review |
| title_full_unstemmed | Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review |
| title_short | Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review |
| title_sort | pediatric extraspinal sacrococcygeal ependymoma report of two cases and literature review |
| topic | pediatric ependymoma extra-CNS ependymoma myxopapillary ependymoma pilonidal disease |
| url | https://www.mdpi.com/2075-4418/11/9/1680 |
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