A Primary Extraosseous Ewing Sarcoma in the Cerebellopontine Angle of a Child: Review of Relevant Literature and Case Report

Rubio, Ellianne J. dos Santos; Harhangi, Biswadjiet S.; Kros, Johan M.; Vincent, Arthur; Dirven, Clemens M.F.

doi:10.1227/neu.0b013e3181f82569

Cited by 16 publications

(8 citation statements)

References 37 publications

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“…Since Stechschulte et al . described the first histologically confirmed right parietotemporal case in 1994, more reports on intracranial EES have appeared in the literature . Primary CNS‐EES affects primarily children and young adults.…”

Section: Discussionmentioning

confidence: 99%

A 36‐year‐old man with fourth ventricular mass

Loharamtaweethong

Sutepvarnon

2015

Neuropathology

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A 36-year-old man presented with a 3-month history of intermittent progressive headache, nausea, vomiting and ataxia. No focal neurologic deficits were detected at the time of presentation. Brain MRI revealed inhomogeneous enhancing, lobulated border, isosignal intensity on T1-weighted image (TIW) ( Fig. 1), hypersignal intensity on fluid-attenuated inversion recovery (FLAIR)/T2W, showing a mass, measuring 4.0 × 3.5 × 3.5 cm. The mass appeared in the dilated fourth ventricle with extension to bilateral foramen of Luschka. The cerebellum appeared normal. The mass caused a pressure effect to the brain stem. The primary radiologic diagnosis was ependymoma. The patient underwent a total resection of the brain tumor. Intraoperative findings show grayish tumor attached to the anterolateral wall of the fourth ventricle. Postoperatively, the patient was completely disabled and totally confined to bed. Palliative radiotherapy was planned instead of chemotherapy due to poor performance status of the patient. Nuclear bone scan and CT scan of chest, abdomen and pelvis were negative for metastatic disease. Fourteen months after diagnosis, the patient is still alive without evidence of tumor recurrence. PATHOLOGICAL FINDINGSMacroscopically, the tumor was resected into multiple fragments of grayish tissues. Microscopically, sheets and compact nests of uniform small blue cells with scant cytoplasm were seen ( Fig. 2A). The nuclei were round to oval with finely dispersed chromatin and a small nucleolus. Mitotic figures were frequent. Immunohistochemically, the tumor demonstrated diffuse, strong membranous positivity for CD99 ( Fig. 2B) and nuclear positivity for Friend leukemia integration 1 transcription factor (FLI-1) (Fig. 2C). Focal positivity for glial fibrillary acidic protein (GFAP), synaptophysin and S100 protein was observed. Diffuse vimentin expression was detected. Integrase interactor 1 (INI-1) expression was preserved. Ki-67 labeling index was observed in approximately 40% of the tumor cells. Expression of AE1/AE3, CD45, CD117, desmin, epithelial membrane antigen (EMA), myogenin, placental alkaline phosphatase (PLAP), and terminal deoxynucleotidyl transferase (TdT) were not detected in tumor specimens. CYTOGENETIC ANALYSISFluorescence in situ hybridization (FISH) analysis revealed rearrangement of the Ewing sarcoma region (EWS) on chromosome 22q12 in 102/200 (51%) of tumor cells ( Fig. 3).

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Section: Discussionmentioning

confidence: 99%

A 36‐year‐old man with fourth ventricular mass

Loharamtaweethong

Sutepvarnon

2015

Neuropathology

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“…The mainstay of local treatment is surgery with or without radiotherapy embedded into neoadjuvant chemotherapy and following local treatment additional chemotherapy to eliminate residual tumor cells [ 9 ]. Recently, tumor-specific genetic alterations have gained interest [ 8 , 11 ]. Mutations in the EWSR-1 gene (Ewing sarcoma breakpoint region 1, 22q12.2) are known to cause ESFT as well as other mesenchymal tumors.…”

Section: Discussionmentioning

confidence: 99%

Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

Stark¹,

Leuschner

Mehdorn³

et al. 2014

Case Reports in Medicine

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Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearranged EWSR-1 gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.

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“…Reported cases of extraskeletal Ewing's sarcoma of the spine in the pediatric [1, 2, 3] and adult [1, 4, 5] populations have been documented. There are even fewer case reports of Ewing's sarcoma occurring in the intracranial cavity [4, 5, 6, 7, 8], and even then those cases occurred in children. The rarity of this malignancy, in combination with the unusual presentation and location of our adult patient's tumor, contributed to the difficulty in making the histopathological diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Disseminated Intracranial Ewings Sarcoma in an Adult:A Rare and Difficult Diagnosis

et al. 2012

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The Ewing sarcoma family of tumors comprises a rare class of cancers of mesenchymal origin. Cases of Ewing’s sarcoma in the central nervous system – specifically, intracranial Ewing’s – are extremely rare. Almost all reported cases have occurred in children. However, this rare presentation can also occur in the adult population. It is important to distinguish these tumors from primitive neuroectodermal tumors at the time of diagnosis. Testing for EWSR1(22q12) gene rearrangement using fluorescence in situ hybridization is a useful tool for making the distinction between these 2 similar but distinct entities. We present here the case of a middle-aged male patient with intracranial Ewing’s sarcoma, and discuss diagnostic challenges and potential new treatment approaches for this rare disease.

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A Primary Extraosseous Ewing Sarcoma in the Cerebellopontine Angle of a Child: Review of Relevant Literature and Case Report

Cited by 16 publications

References 37 publications

A 36‐year‐old man with fourth ventricular mass

A 36‐year‐old man with fourth ventricular mass

Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

Disseminated Intracranial Ewings Sarcoma in an Adult:A Rare and Difficult Diagnosis

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A Primary Extraosseous Ewing Sarcoma in the Cerebellopontine Angle of a Child: Review of Relevant Literature and Case Report

Cited by 16 publications

References 37 publications

A 36‐year‐old man with fourth ventricular mass

A 36‐year‐old man with fourth ventricular mass

Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

Disseminated Intracranial Ewings Sarcoma in an Adult:A Rare and Difficult Diagnosis

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Disseminated Intracranial Ewings Sarcoma in an Adult:A Rare and Difficult Diagnosis