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

Abstract

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.

When a dermatopathologist encounters the ultra‐rare: A case series of superficial soft tissue/cutaneous myxopapillary ependymomas

“…Anaplastic transformation, although uncommon, has been described in MPEs, marked by the presence of pronounced cytologic atypia, increased mitotic activity, and areas of necrosis and endothelial proliferation, 20 particularly seen in areas of hypercellularity and reduced extracellular mucin 22 . A few case reports have described sacrococcygeal soft tissue ependymomas with prominent cellular and nuclear pleomorphism, including giant cell features, as well as a solid and trabecular architectural pattern of growth 25–27 . Although these lesions have been named “giant cell ependymoma,” a term reflective of their morphologic features, it is unclear whether they represent a distinct clinicopathologic entity or a mere morphologic variation within the spectrum of MPE.…”

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

“…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 ].…”

“…Conversely, classical ependymomas become more frequent in the pelvic or mediastinal region, while more aggressive histological subtypes have been described less frequently, sometimes as areas of undifferentiation associated with a myxopapillary component [ 27 , 29 ]. Recently, the first case of giant cell ependymoma of the sacrococcygeal region occurring in childhood was reported by Planas and colleagues [ 36 ].…”

“…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 ].…”

“…Conversely, classical ependymomas become more frequent in the pelvic or mediastinal region, while more aggressive histological subtypes have been described less frequently, sometimes as areas of undifferentiation associated with a myxopapillary component [27,29]. Recently, the first case of giant cell ependymoma of the sacrococcygeal region occurring in childhood was reported by Planas and colleagues [36]. While it is universally accepted that CNS ependymomas develop from the ependymal cells lining in the ventricles and the central canal, the pathogenesis of extraspinal ependymomas is still debated, with three main hypotheses reported in the literature [3,4,6] (Figure 2).…”