A case of an anaplastic ependymoma with gliosarcomatous components

Kano, Tomoaki; Ikota, Hayato; Wada, Hirochiyo; Iwasa, Susumu; Kurosaki, Shuhei

doi:10.1007/s10014-008-0240-x

Cited by 3 publications

(9 citation statements)

References 14 publications

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“…2 was similar to the present case. To date, in English publications about ependymal tumors with sarcomatous changed “ependymosarcomas,” only 19 cases, including the present case, have been reported (Table ) . There were 10 female and nine male patients with ependymosarcoma (median age, 34 years; range, 2–63).…”

Section: Discussionmentioning

confidence: 87%

Ependymosarcoma with eosinophilic granular cells

et al. 2013

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Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. Were port a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle.A 62-year-old man complained of headaches over a 2-month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation.Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplasticglial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot-like epithelial membrane antigen and diffuse cytoplasmic D2-40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10%of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB-crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.

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Section: Discussionmentioning

confidence: 87%

Ependymosarcoma with eosinophilic granular cells

et al. 2013

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“…Reports include 2 cases of subependymoma with sarcomatous change [11, 12, 18], 1 case of mixed subependymoma-rhabdomyosarcoma [20], and 15 cases of sarcomatous change in ependymoma [2, 7,9,10, 18, 21]. The ependymomas described have been classified as WHO grade II or grade III [2, 9, 10, 17, 21]. Seven of the 15 ependymosarcomas were described in the original resection specimens [7, 18], while 8 were described in recurrent tumor specimens [2,9,10,18,21].…”

Section: Discussionmentioning

confidence: 99%

“…The ependymomas described have been classified as WHO grade II or grade III [2, 9, 10, 17, 21]. Seven of the 15 ependymosarcomas were described in the original resection specimens [7, 18], while 8 were described in recurrent tumor specimens [2,9,10,18,21]. Seven of the 15 cases were described after the patient had received radiation therapy [2, 9, 10, 18].…”

Section: Discussionmentioning

confidence: 99%

“…Seven of the 15 ependymosarcomas were described in the original resection specimens [7, 18], while 8 were described in recurrent tumor specimens [2,9,10,18,21]. Seven of the 15 cases were described after the patient had received radiation therapy [2, 9, 10, 18]. Whether these tumors are radiation induced depends on a number of factors, including defining the time interval required between the radiation and the development of the sarcomatous component [18].…”

Section: Discussionmentioning

confidence: 99%

“…In most cases, the glial component is astrocytic [5]. However, there have been reports of the glial component being oligodendroglial [17] or ependymal [2,9,10,18,21]. The terms oligosarcoma and ependymosarcoma have been proposed for such composite tumors [17,18].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Identification of t(1;19)(q12;p13) and ploidy changes in an ependymosarcoma: a cytogenetic evaluation

Tabbarah¹,

Carlson²,

Oviedo³

et al. 2012

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Gliosarcoma, a recognized subtype of glioblastoma, is a biphasic tumor exhibiting distinct glial and sarcomatous components. Ependymosarcomas are rarer, biphasic ependymal tumors exhibiting sarcomatous change. Genetic abnormalities associated with this curious phenotype are not well understood. We are presenting the first karyotype of ependymosarcoma with identification of a clonal t(1;19)(q12;p13). Fluorescence in situ hybridization (FISH) was performed with a probe set targeting 1q23 and 19p13.3. Although the tumor did not show evidence of t(1;19)(q23;p13.3) by FISH, increased ploidy was a feature of the sarcomatous component. On clinical follow-up the patient is doing well without evidence of recurrence 55 months after initial resection, and postoperative treatment with irradiation and temozolomide. The significance of the genetic alterations we describe associated with sarcomatoid change in ependymal neoplasms, and ultimately their prognostic relevance, merits further study.

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Ependymosarcoma harboring C11orf95:RELA fusion transcript: Report of two cases and review of the literature

et al. 2021

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Ependymoma is a relatively rare glial tumor of the central nervous system that arise from the cells lining the ventricles and central canal of the spinal cord. Ependymosarcoma (ES) is a newly introduced tumor entity of uncertain prognosis characterized by a rare phenomenon of a malignant mesenchymal transition arising within an ependymoma. ESs are surgically challenging tumors for diagnosis and therapy with a high incidence of morbidity and mortality. Here, we report two diagnostically challenging cases of primary ES in a 25-year-old female and a 17-year-old male. Both the cases presented with progressive and sequential neurological deficits over a period of five to eight months, and histological examination revealed a biphasic gliomesenchymal architecture comprised of anaplastic ependymomatous and sarcomatous components. Molecular genetic analysis revealed the presence of type 1 C11orf95:RELA fusion transcript. To date, 22 cases of ES have been reported in the literature, and only one case harbored type 1 C11orf95:RELA fusion transcript.

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A case of an anaplastic ependymoma with gliosarcomatous components

Cited by 3 publications

References 14 publications

Ependymosarcoma with eosinophilic granular cells

Ependymosarcoma with eosinophilic granular cells

Identification of t(1;19)(q12;p13) and ploidy changes in an ependymosarcoma: a cytogenetic evaluation

Ependymosarcoma harboring C11orf95:RELA fusion transcript: Report of two cases and review of the literature

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