Diagnosis: Subcutaneous myxopapillary ependymoma

Thejeel, Bashiar; Rajakulasingam, Ramanan; Balogh, Petra; Saifuddin, Asif

doi:10.1007/s00256-020-03407-x

Cited by 3 publications

(2 citation statements)

References 8 publications

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“…More than a century ago, Mallory et al first described the case of an ependymoma that developed in the subcutaneous tissue of the sacrococcygeal region [ 9 ]. Since then, only a few cases have been reported in literature with less than 50 reported cases occurring in childhood during the last 35 years ( Table 1 ) [ 4 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ]. They occur more frequently in the sacrococcygeal region, followed by the pelvic region, while cases of onset in the liver, lung, or mediastinum are rarer and mainly described in adulthood [ 3 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Extra CNS ependymomas arising in childhood have a tendency for both local and metastatic recurrences, the inguinal nodes being the most common localization. Relapse occurs in about 20% of cases described in the last 35 years, even more than 10 years after the initial remission [ 4 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ]. Despite this, the prognosis is good: in the past 35 years, only one death in 39 cases has been reported in the literature [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

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Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

et al. 2021

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

et al. 2021

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Extra‐axial sacral soft tissue giant cell ependymoma affecting a child: Case report and review of the literature

et al. 2021

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An otherwise healthy eight-year-old girl presented with a mass in the soft tissue of the sacral region. The lesion was diagnosed as a vascular malformation on imaging studies, for which percutaneous sclerotherapy was attempted. The mass continued to grow and a complete resection was performed after four years. The pathological diagnosis was giant cell ependymoma (GCE). GCE is a term used to describe a rare histologic variant of ependymoma characterized by malignancy-like morphologic phenotype and indolent behavior. To the best of our knowledge, this is the first case of extra-axial soft tissue sacral GCE reported in a child.

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Subcutaneous Sacro Coccygeal Myxopapillary Ependymoma: A Case Report and a Comprehensive Review of the Literature Reappraising Its Current Diagnostic Approach and Management

Ramkumar¹,

Wanniang²,

Wahlang³

et al. 2021

Cureus

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Sacrococcygeal myxopapillary ependymoma (MPE) is an uncommon type I glial tumor detected most frequently in the lumbosacral area of adolescents and children. It is usually presented as an intradural ependymal tumor that originates from the filum terminale and other locations within the ventricular system along the craniospinal axis. In rare cases, however, MPE may develop as a primary subcutaneous tumor in the sacrococcygeal area. Tumors can also appear as a dorsal sacrococcygeal growth or subcutaneous nodule. In this case report, we describe a rare case presenting as a subcutaneous sacrococcygeal mass in an elderly female that was subsequently resected and confirmed as subcutaneous MPE. The current standard treatment for MPE is maximal surgical resection with or without postoperative radiotherapy based on the locoregional extent and histological grading. However, there is limited evidence that radiotherapy for oligometastatic foci improves longevity or extends the time to recurrence. In addition to this case report, we provide a comprehensive review of similar cases and case series in the medical literature. Prospective studies evaluating the efficacy of resection and/or radiotherapy are required for improved management of extradural MPE.

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Diagnosis: Subcutaneous myxopapillary ependymoma

Abstract: A 16-year-old girl presented with a 2 month history of a painless lump in the buttock.

Cited by 3 publications

References 8 publications

Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

Extra‐axial sacral soft tissue giant cell ependymoma affecting a child: Case report and review of the literature

Subcutaneous Sacro Coccygeal Myxopapillary Ependymoma: A Case Report and a Comprehensive Review of the Literature Reappraising Its Current Diagnostic Approach and Management

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