Case reportGiant cell ependymoma: A case report
Introduction
GCE is a particularly rare variant of ependymoma. To the best of our knowledge, to date, only seven cases of GCE have been described in the literature [1]. The cases reported so far are summarized in Table 1. Because of such a low number of the reported cases it is impossible to draw definite conclusions as for the biological and clinical behavior of the tumor (see Section 4).
Ependymomas are neuroepithelial neoplasms occurring mainly in childhood and in young adults. The ventricular system and its vicinity is a typical site of these slow-growing tumors however they appear along the whole neuroaxis including the spinal cord and also extraventricularly: the latter probably originating from embryonic ependymal remnants. There is a poor association between the biological behavior and morphological presentation of the tumor, in consequence, it is difficult to predict the patient's outcome on the grounds of histopathological examination. Age is a well-known factor, with the tumors affecting children being more aggressive. Apart from “standard” ependymomas the WHO classification incorporates some particular variants, such as cellular, papillary, clear cell and tanycytic ependymoma [2]. Other variants, as ependymoma with lipomatous differentiation, signet ring cell ependymoma, melanotic ependymoma and giant cell ependymoma, are extremely rare.
The majority of the reported cases of GCE (see Table 1) were seen in young people. Three tumors were localized in the vertebral canal and five intracranially (that number includes the present case). In the present paper, the authors report a case of GCE arising from the lateral ventricle in 17-year-old subject.
Section snippets
Case report
A 17-year-old patient was admitted to hospital (September 2005) due to vision impairment, which had occurred 1 month earlier. He also had a history of mild, recurrent headaches persisting for 2 years. Physical examination revealed bilateral pupil edema with hemorrhage in the left eye and the paresis of the cranial nerve VI. CT scanning (Fig. 1, Fig. 2) has demonstrated a large tumor localized in the left occipital lobe, arising from the occipital horn of the lateral ventricle.
An occipital
Histopathology
For histological examination the surgically obtained specimens were fixed with 10% buffered formalin and embedded in paraffin. Sections were stained with hematoxylin and eosin, and periodic acid-Shiff (PAS). For immunohistochemistry, antibodies against GFAP (DAKO, 1:100), NSE (DAKO, 1:100), synaptophysin (DAKO, 1:50), topoisomerase II alpha (Novocastra, 1:30) were used.
For electron microscopic examination, fragments of the formalin-fixed tumor were retrieved. They were refixed in glutaraldehyde
Discussion
The bizzare morphological features of the tumor, including gigantocellular morphology with marked pleomorphism, in spite of the occurrence of sporadic structures resembling true ependymal rosettes and perivascular pseudorosettes; necessitate considering not only special variants of ependymoma, such as clear cell ependymoma, anaplastic ependymoma, and giant cell ependymoma but also some other possibilities like: anaplastic oligodendroglioma, subependymal giant cell astrocytoma (SEGA),
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Cited by (21)
Giant Cell Ependymoma of Cervicomedullary Junction: A Case Report of a Long-Term Survivor and Literature Review
2018, World NeurosurgeryCitation Excerpt :In 13 of the low-grade lesions, gross total resection was reported. In 1 case, a subtotal resection was reported with a recurrence after 5 months; in 1 case, only biopsy and radiotherapy were performed; in 1 case, the diagnosis was made at autopsy because the patient died of respiratory insufficiency before surgery.18 We describe the first case to our knowledge of GCE of the cervicomedullary junction; a detailed histologic examination was performed to distinguish the GCE from other high-grade tumors.
Giant Cell Ependymoma of Lateral Ventricle: Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile
2017, World NeurosurgeryCitation Excerpt :At the time of last follow-up, adjuvant proton beam therapy targeting the resection cavity was ongoing. Including our patient, 29 cases of GCE have been described, none of which arose within the lateral ventricle (Supplementary Table S1).6-28 Although the heterogeneity of the preceding reports precludes formal meta-analysis, we have summarized the overarching findings to help clarify the characteristics of this rare tumor.
Multiple chromosomal monosomies are characteristic of giant cell ependymoma
2011, Human PathologyCitation Excerpt :As GCEs seem to have a very clear-cut cytogenetic pattern, it could be argued that cytogenetic analysis should be included in routine diagnostics. The literature has suggested intracerebral GCEs to be more aggressive than those related to the spine [4-6,13-15]. The remarkable similarity between the cytogenetic profiles of this supratentorial tumor and that of the filum terminale [2] suggests no clear pathogenetic backing for this finding.
Giant cell ependymoma of the cauda equina. A case report
2010, Revista Espanola de PatologiaGiant-cell ependymoma: Presentation of a case of the sacral region and literature review
2017, Ultrastructural Pathology