Giant Cell Ependymoma Research Articles

Giant cell ependymoma: Cytology of a rare entity with unusual presentation

Giant cell ependymoma (GCE) is a slow-growing tumor that accounts for 9–13% of all ependymoma and occurs preferably at conus medullaris, cauda equina, and filum terminate. Squash cytology is a fast, simple, and reliable technique for intraoperative diagnosis. We present a case of GCE arising at the L5-S2 level and suprasellar region in a 17-year-old male who initially presented with low backache followed by headache and double vision. Intraoperative squash smears of the lumbosacral lesion were suggestive of high-grade glioma. Histomorphological and immunohistochemical profile favors the diagnosis of giant cell variant of myxopapillary ependymoma. He received craniospinal irradiation followed by a boost to the sellar-suprasellar and lumbosacral region through IGMT. After sometime, he developed papilledema and decreased vision, the suprasellar lesion was decompressed which on histopathology revealed similar features. GCE remains a diagnostic challenge on intraoperative squash smears due to its rarity. It has a good prognosis with a 5-year survival of 98.4% after total resection; however, our patient succumbed to illness due to post-operative residual lumbosacral mass and effects of the suprasellar lesion.

Factors associated with postoperative outcomes in patients with intramedullary Grade II ependymomas: A Systematic review and meta-analysis.

Background:Most of the previous studies combined all types of intramedullary ependymomas without providing accurate pathological subtypes. In addition, it was very difficult to evaluate the factors associated with postoperative outcomes of patients with different pathological subtypes of intramedullary Grade II ependymomas by traditional meta-analysis. This study evaluated the factors related with postoperative outcomes of patients with intramedullary Grade II ependymomas.Methods:Individual patient data analysis was performed using PubMed, Embase, and the Cochrane Central Register of Controlled Trials. The search included articles published up to April 2018 with no lower date limit on the search results. The topics were intramedullary Grade II ependymomas. Progression-free survival (PFS) and overall survival (OS) were analyzed by Kaplan–Meier survival analysis (log-rank test). The level of significance was set at P < .05.Results:A total of 21 studies with 70 patients were included in this article. PFS of patients who underwent total resection was much longer than the PFS of those who received subtotal resection (P < .001). Patients who received adjuvant therapy (P = .005) or radiotherapy and chemotherapy (P < .001) seemed to have shorter PFS than others; PFS of patients who had cerebrospinal fluid disease dissemination (P = .022) or scoliosis (P = .001) were significantly shorter than others. OS of cellular ependymoma patients was less than giant cell ependymoma patients (P < .001).Conclusions:PFS of patients who received total resection was much longer than those who received subtotal resection. Patients treated with adjuvant therapy or radiotherapy and chemotherapy appeared to have shorter PFS than others; PFS of patients with cerebrospinal fluid disease dissemination or scoliosis were significantly shorter than others. Cellular ependymomas would have better OS than giant cell ependymoma. However, giant cell ependymoma patients might have the worst OS.

Survival outcomes and prognostic factors of patients with intramedullary Grade II ependymomas after surgical treatments

This study evaluated survival outcomes of patients with intramedullary Grade II ependymomas and identify prognostic factors. Electronic searches of PubMed, EMBASE, OVID, the Cochrane Central Register of Controlled Trials were performed to identify trials according to the Cochrane Collaboration guidelines. The objects were intramedullary Grade II ependymoma according to 2007 WHO classification. Kaplan–Meier survival analysis with log-rank test was used to analyze progressive free survival (PFS) and overall survival (OS). Cox proportional hazard model was utilized for multivariate analysis with hazard ratio (HR) and 95% confidence interval (CI) calculated. P values <0.05 were considered statistically significant. A total of 28 studies including 138 cases of intramedullary Grade II ependymomas were retrieved. Patients who were classified as cellular ependymomas or papillary ependymomas had higher risks of progression than those who possessed typical Grade II ependymomas. Patients who were treated with adjuvant therapy had a higher risk of progression than those without adjuvant therapy. OS of patients with giant cell ependymoma was significantly shorter than those with typical Grade II ependymoma. Patients who had cellular or papillary subtype, adjuvant therapy would have a shorter estimated value of progression-free time and a higher risk of progression than typical Grade II ependymomas. Giant cell ependymoma patients would have a higher risk of fatality than those with typical Grade II ependymomas. Definite pathology type and appropriate treatments were foundations of intramedullary Grade II ependymomas’ managements.

Supratentorial giant cell ependymoma with extensive tumour cell vacuolization and calcification

Supratentorial giant cell ependymoma with extensive tumour cell vacuolization and calcification

Immunohistochemical features of giant cell ependymoma of the filum terminale with unusual clinical and radiological presentation

BackgroundGiant cell ependymoma of the filum terminale is a rare variant, generally manifested as a well-circunscribed intradural mass with an indolent biological behavior.Case presentationWe describe the case of a 48-year-old Mexican female who non-relevant past medical history, that developed a GCE of the filum terminale. Magnetic resonance imaging and computed tomography revealed the presence of an intra-axial tumor extending from L3 to L5 with extra-medullary invasion. Therefore the tumor was considered unresectable and only incisional biopsy was obtained, establishing the tentative diagnosis of a poorly differentiated neoplasia. A second evaluation of the case revealed the presence of numerous non-cohesive pleomorphic giant cells with intranuclear inclusions and broad eosinophilic cytoplasm, alternating with intermediate size cells with round, hyperchromatic nuclei and forming a perivascular pseudo-rosettes pattern. The ependymal phenotype was supported by light microscopy and corroborated by immunohistochemistry analysis. The patient was subsequently treated with radiotherapy 54Gy. She is alive after a 27-month follow-up, with residual disease, difficulty ambulating and pain.ConclusionsGCE of filum terminale may have an atypical clinical and radiological presentation, albeit with invasive characteristics and anaplasia on histologic analysis. However, its biological behavior is indolent and associated to longer survival. Due to the presence of giant cells, the differential diagnosis of other primary neoplasias at that site were considered, including paraganglioma, malignant peripheral nerve sheath tumors as well as metastatic malignant melanoma, adrenal carcinoma, thyroid gland carcinoma and urothelial carcinoma, that may all harbor giant cells.

Rare Variants of Ependymoma: A Report of Four Cases and a Review of the Literature

Ependymomas are brain tumors that have a wide spectrum of morphological patterns. Rare or unusual patterns of ependymoma can resemble other neoplasms and result in erroneous diagnosis, and management. Four out of 22 ependymoma cases in the pathology archive of King Abdulaziz University Hospital in the last decade had these patterns. The fi rst case was of ependymoma with multinodular growth and diff erent types of ependymal diff erentiation, including subependymoma, classic ependymoma, and astroblastoma. Dysplastic neurons were associated with the neoplastic growth. This combination can be interpreted as ganglioglioma (i.e., with ependymoma representing the glial component, World Health Organization grade I) or alternatively as ependymoma with dysplastic neurons (World Health Organization grade II). The second case was of a rare variant of intracranial extra-axial ependymoma with lobular and papillary architecture. The third case was of a previously unreported combination of clear cell and giant cell ependymoma. The fourth case was of epithelioid ependymoma with fi brillary background and a peculiar arrangement of the tight clustered epithelioid cells. This report expands the morphological spectrum of ependymoma by introducing diff erent, previously unreported combinations of patterns that were observed in individual tumors, as well as rare locations andarchitectures.

31. Giant cell ependymoma of the third ventricle: a case report

Ependymomas are central nervous system (CNS) glial tumours thought to be derived from ependymal lining cells in the ventricles or spinal cord. The 2007 WHO classification of CNS tumours recognises three grades of ependymoma and several subtypes. 1 Giant cell ependymoma is a rare variant of ependymoma characterised by bizarre tumour giant cells with a variable component of more typical ependymoma. It has been described largely as isolated case reports and small series since its first recognition in 1996. 2 Giant cell ependymoma may be either WHO grade II or grade III. It has been described in variety of sites, including: spinal cord, cerebellum, the suprasellar region and cerebral hemispheres. We describe a case of giant cell ependymoma of the third ventricle occurring in a previously healthy 7 year old girl. The morphological features, immunohistochemical findings and ultrastructural features are discussed. As this ependymal subtype is not widely known, it may be confused with several other tumours including: pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, giant cell glioblastoma and atypical teratoid/rhabdoid tumour (ATRT). The differential diagnosis is discussed as confusion with these other entities may have significant treatment and prognostic implications making recognition of this rare ependymoma subtype vital.

Correlative study of squash smear cytology with histopathology in a rare case of anaplastic giant cell ependymoma of the pineal.

Anaplastic giant cell ependymoma (AGCE) is a very rare neoplasm. Its cytological features, helpful for the intraoperative diagnosis, have been reported only once. AGCE is characterized by giant cells with intranuclear inclusions, besides other findings, observable in ependymal neoplasms, such as intracytoplasmic vacuoles, epithelial and glial features of the tumor cells and ependymal pseudorosettes. These findings can be detected also in intraoperative squash smear. Herein we describe a pineal AGCE, highlighting the cytological and histological correlations and underlining some useful diagnostic clues of this unusual entity.

Giant cell ependymoma of the thoracic spinal cord

We report a new case of giant cell ependymoma (GCE) of the thoracic spinal cord. Ependymomas predominate in children and young adults and are frequently intracranial and infra-tentorial. However, a second age peak at 30-40 years is reported for spinal tumours. Microscopically, ependymomas show a large variety of histological features, among which a rare variant with giant cells. This 59-year-old woman presented with a 6-month history of numbness and burning sensation affecting the left lower limb and hemi-trunk. A cervico-thoracic MRI revealed a solid intra-medullary tumour at the level of T1-T3, slightly T1-hypointense, T2-hyperintense and contrast enhancing. A complete surgical resection was carried out through a C7 to T4 laminectomy. Recovery was complete with no sign of recurrence at 18-month follow-up. The initial histological diagnosis of glioblastoma was challenged on the basis of the imaging and operative findings of a well-circumscribed tumour. The case was sent to us for second opinion and we diagnosed a GCE, WHO grade II, with a biphasic pattern including a predominant giant cell component (>90%), with genetic evidence of polyploidy, and a very limited classic component, showing a characteristic loss of chromosome 22. Our report adds to the clinical, imaging, pathological and genetic characterisation of GCE and brings the first genetic evidence that these rare tumours are at least bi-clonal. It also suggests that GCE have a good prognosis after complete surgical resection.

Cytologic Features of Giant Cell Ependymoma: A Case Report and Review of the Literature

Here, we present a case of anaplastic giant cell ependymoma (GCE) occurring in a 15-year-old woman. Squash smear slides for intraoperative frozen section diagnosis revealed oval to round cell clusters with a papillary structure in a fibrillary background. This was occasionally accompanied by the presence of bizarre pleomorphic giant cells with hyperchromatic nuclei and prominent intranuclear inclusions. These intranuclear inclusions were a key clue to diagnosis of ependymoma. Histologic analysis revealed features of a high-grade tumor with perivascular pseudorosettes and bizarre pleomorphic giant cells, which established the diagnosis of GCE. We performed a review of literatures about the cytologic features of GCE, including our case, thus proposing that intraoperative frozen diagnosis of GCE would be established by squash smear preparations featuring the mitosis and necrosis, as well as the high cellularity, and the presence of giant cells showing hyperchromatic nuclei with eosinophilic cytoplasm and intranuclear inclusions/pseudoinclusions.

Rare histological variants in ependymomas: histopathological analysis of 13 cases

Ependymomas are rare brain tumors representing about 3% of all intra-cerebral and spinal neoplasms. The WHO classification recognizes various rare histological ependymoma variants (i.e., lipidized ependymoma, giant cell ependymoma, etc.). However, a detailed analysis of a larger series of such cases is still lacking. We analyzed 13 case of ependymoma presenting unusual histological features. The data analysis of our series and its comparison to the cases published in literature did not reveal any close association between these features and the clinical parameters (such as age or localization). Moreover, some of these features can be found combined in individual tumors, suggesting that these variants may represent a spectrum of differentiation rather than true specific entities. However, awareness on these rare histological patterns in ependymomas is necessary in the differential diagnosis with other primary or secondary brain tumors.

Multiple chromosomal monosomies are characteristic of giant cell ependymoma

Giant cell ependymoma, a rare ependymoma subtype, was recently recognized as a separate diagnostic entity with variations both in malignant potential and course of disease. We analyzed the first supratentorial giant cell ependymoma using G-band karyotyping, DNA ploidy analysis, and array comparative genomic hybridization. The tumor was hypodiploid, and the karyotype showed multiple monosomies. This novel cytogenetic pattern seems specific for giant cell ependymoma as the only previous cytogenetic analysis of a giant cell ependymoma found similar monosomies. We were also able to analyze cytogenetically the subsequent recurrent tumor, phenotypically an anaplastic ependymoma, allowing a first insight into the genetic events involved in disease progression.

Ependimoma de células gigantes de la cauda equina. A propósito de un caso

Los ependimomas constituyen la neoplasia más frecuente de la médula espinal. Se desarrollan a partir de células que revisten el canal ependimario. Un subtipo infrecuente es el ependimoma de células gigantes, del que se han descrito solo 8 casos en dicha localización. Describimos el caso de un varón de 42 años con lumbalgia de varios meses de evolución. La RMN mostró un tumor bien circunscrito intradural en L1, que se resecó en su totalidad. El estudio histológico demostró proliferación fusocelular de distribución perivascular con formación de pseudorrosetas y moderada atipia citológica con células gigantes multinucleadas. El estudio inmunohistoquímico confirmó el diagnóstico de ependimoma de células gigantes. Ependymomas are the most frequent neoplasms of the spinal cord, arising from the cells lining the spinal canal. Among them, giant cell ependymoma is a rare subtype with only 8 cases previously reported to date. We present a further case in a 42-year-old man who presented with a history of lower back pain for several months. The MRI revealed a well-circumscribed interdural mass at L1. The tumour was totally resected and histologically it was seen to be comprised of a proliferation of fusiform cells arranged in a perivascular pattern with pseudorosettes and cytologic atypia with multinucleated giant cells. Immunohistochemistry confirmed the diagnosis of giant cell ependymoma.

Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex

Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare.

GIANT CELL EPENDYMOMA OF THE THORACIC SPINE

INTRODUCTION AND IMPORTANCE: Spinal ependymomas are slow-growing lesions that comprise the majority of primary spinal cord neoplasms. When surgery is indicated, the extent of tumor removal is most prognostic for long-term survival. Unusual histological subtypes can make intraoperative diagnosis spurious, possibly altering the surgical approach from gross total resection for ependymomas to debulking for high-grade astrocytomas. We describe a 67-year-old woman with a thoracic spine intramedullary giant cell ependymoma. She presented with decreased lower extremity sensation leading to unsteadiness and an eventual fall. A physical examination revealed lower extremity hyperreflexia and ankle clonus, but no clear sensory level. Magnetic resonance imaging demonstrated an intramedullary T1 and T2 hypointense, homogenously enhancing lesion at T8 with extensive cephalad and caudal edema. A laminectomy at T8 to T9 afforded gross total resection of the lesion that had a clear cleavage plane with normal spinal cord. Intraoperative pathology suggested a high-grade glioblastoma, but final section showed sporadic giant cells with marked pleomorphism, uniform immunofluorescence staining with both glial fibrillary acidic protein and cluster of differentiation 99, and high MIB-1 index. Electron microscopy showed "zipper-like" junctions. There were no detected genomic abnormalities consistent with glioblastoma. We present this first reported case of thoracic spine giant cell ependymoma alongside scant literature yielding 1 case in the cervical spine and 2 cases at the filum terminale. Those cases had benign courses, whereas ours demonstrates a high degree of proliferation, making the malignant potential difficult to assess.

Giant cell ependymoma of the cervical spinal cord: case report and review of the literature

Ependymomas account for 2-6% of all central nervous system neoplasms. They develop from the ependymal cells that line the ventricular cavities of the brain and the central canal of the spinal cord, as well as from ependymal clusters in the filum terminale. Giant cell ependymoma (GCE) is a rare subtype, with few cases reported, mostly in the brain. We describe the case of a cervical spinal cord ependymoma with pleomorphic giant cells and focal calcifications occurring in a 25-year-old woman. Magnetic resonance imaging revealed a large multicystic and partially enhancing intramedullary tumour extending from C2 to C5. Intraoperative analysis of frozen section tissue fragments suggested a malignant tumour; however, an obvious cleavage plane was present around most of the mass, and a macroscopically complete tumour removal could be achieved. Subsequently, paraffin sections and immunohistochemical investigations revealed unequivocal evidence of a GCE with focal calcifications. This case, the second giant-cell ependymoma to be described in the spinal cord and the first with focal calcifications, highlights the features of GCE and the discrepancy between the worrisome histological appearance, the surgical findings and the clinical relatively good prognosis.

Suprasellar giant cell ependymoma: a rare neoplasm in a unique location

Ependymomas are glial tumors that usually present in the posterior fossa in children and in the spinal cord in adults. Giant cell ependymoma, a rare ependymal subtype only recently recognized as a diagnostic entity in the last decade, demonstrates pleomorphic giant cells admixed with features of typical ependymoma. Although only 8 giant cell ependymomas have been reported to date, none have been reported in the suprasellar space. Moreover, as these neoplasms demonstrate a high incidence of anaplastic grade, recognition of this ependymal subtype is paramount. We describe the presentation and pertinent radiologic, histologic, immunologic, and ultrastructural findings in conjunction with relevant clinical implications of the first reported case of a suprasellar giant cell ependymoma occurring in a 34-year-old female 7 years after an initial diagnosis of a medullary ependymoma with rare atypical giant cells, a potential tumor seeding culprit.

Giant cell ependymoma: A case report

Ependymomas account for 3–9% of all neuroepithelial tumors. A peculiar variant of ependymoma known as “giant cell ependymoma” (“GCE”) is especially rarely reported, it may pose some difficulties for the diagnosing neuropathologist. Here we present a case of a giant cell ependymoma occuring in a 17-year-old patient with the history of 2-year recurrent headaches and a 1-month history of vision impairment. CT scanning demonstrated a mass in the left occipital lobe, arising from the occipital horn of the lateral ventricle. Histological, immunohistochemical and electron microscopic findings were consistent with high-grade ependymoma. Especially striking was the presence of bizzare pleomorphic giant cells which predominated in the tumor tissue. As a result the diagnosis of GCE was established. This type of neoplasm necessitates, at least in theory, differentiation with anaplastic oligodendroglioma, clear cell ependymoma, pleomorphic xanthoastrocytoma, giant cell glioblastoma, and subependymal giant cell astrocytoma. To date giant cell ependymomas (GCEs) were reported in seven cases in the literature. To the best of our knowledge this is the 8th case in the literature. In spite of apparently “worrisome” histology GCE seems to be a neoplasm with a relatively good prognosis.

Giant cell ependymoma: two new cases and review of the literature

Giant cell ependymoma: two new cases and review of the literature

Giant Cell Ependymoma: Two New Cases and Review of the Literature

Giant Cell Ependymoma: Two New Cases and Review of the Literature