Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review

Mobley, Bret C.; Roulston, Diane; Shah, Gaurang; Bijwaard, Karen E.; McKeever, Paul E.

doi:10.1016/j.humpath.2006.02.011

Cited by 88 publications

(99 citation statements)

References 55 publications

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“…However, intracranial pPNET is rare, with only 15 cases of pPNET originating in the intracranial soft tissue. 1,3,[7][8][9][10][11][14][15][16][17][18]20) We describe a case of intracranial pPNET mimicking a cystic meningioma.…”

Section: Introductionmentioning

confidence: 99%

Intracranial Peripheral-Type Primitive Neuroectodermal Tumor -Case Report-

Furuno

Nishimura

Kamiyama

et al. 2008

Neurol. Med. Chir.(Tokyo)

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A 15-year-old man presented with headache. Magnetic resonance (MR) imaging revealed a large extraaxial tumor with cyst at the right frontotemporal region. The solid part of the tumor was homogeneously enhanced on T 1 -weighted MR imaging after injection of gadolinium. Digital subtraction angiography of the external carotid artery revealed sunburst appearance corresponding to the tumor, which was fed by the right middle meningeal artery. His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed. At surgery, the tumor strongly adhered to the dural membrane, and was obviously extraaxial. The tumor and cyst were gross totally removed. The attachment site at the dura mater was resected. Histological examination showed solid growth of small round cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical staining showed the cells were positive for MIC-2 (CD99). The MIB-1 labeling index was 53%. The histological diagnosis was peripheral-type primitive neuroectodermal tumor (pPNET). Following surgery, radiation therapy and chemotherapy were given. Ewing's sarcoma and pPNET form a family of small round cell tumors arising in the bone or soft tissue. MIC-2 is a useful marker in the differential diagnosis. Good prognosis may be attained if complete surgical excision of intracranial pPNET is achieved.

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Section: Introductionmentioning

confidence: 99%

Intracranial Peripheral-Type Primitive Neuroectodermal Tumor -Case Report-

Furuno

Nishimura

Kamiyama

et al. 2008

Neurol. Med. Chir.(Tokyo)

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“…It was revealed that Askin's tumor shares the characteristic chromosomal translocations and has a comparable morphology, immunohistochemistry and ultrastructure with classical peripheral PNET and Ewing's tumor (1,2). According to classification of tumors of the nervous system, Askin's tumor is a member of the Ewing's sarcoma family of tumors/peripheral PNETs.…”

Section: Discussionmentioning

confidence: 99%

“…Peripheral primitive neuroectodermal tumor (PNET) is a type of soft tissue sarcoma, described as arising intracranially (1,2). PNETs of the chest wall were originally reported by Askin et al in 1979 (3); since then, a PNET that occurs within the thoracopulmonary region is named an Askin's tumor.…”

Section: Introductionmentioning

confidence: 99%

Askin's tumor: 11 cases and a review of the literature

Zhang

Lu²,

Zhang³

et al. 2015

Oncology Letters

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Abstract. Askin's tumor is a peripheral primitive neruoectodermal tumor within the thoracopulmonary region, which primarily occurs in children and young adults. In addition, Askin's tumor is commonly misdiagnosed, as it is rare and easily mistaken for other small round-cell tumors. The present study aimed to investigate the clinical characteristics, prognostic factors and treatment outcomes of patients diagnosed with Askin's tumor. Computed tomography (CT) scans, histopathology and immunohistochemical analysis were used for diagnosis. Patients were treated with combined (surgery-chemotherapy-radiotherapy) or mono-therapy (chemotherapy or radiotherapy) methods. A total of 11 consecutive patients with Askin's tumor (aged 8-22 years) were treated at the First Affiliated Hospital of Zhengzhou University between April 2010 and June 2013; nine patients underwent combined therapy and two patients were treated using mono-therapy. Chest lumps, swelling and pain were the most common presenting symptoms. Patients were followed up for ≤24 months post surgery and the results revealed that the median survival time of the combined and mono-therapy treatment groups were 15 and 7 months, respectively. Primary tumor size, metastasis, lactate dehydrogenase indicators and tumor stages were found to be important prognostic factors affecting patient outcome. In conclusion, the results of the present study demonstrated that the combination of surgery, chemotherapy and radiotherapy resulted in the optimal outcome for Askin's tumor patients.

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“…The cauda equina is an extremely rare location and only about 5 cases of primary cauda equina Ewing's sarcoma/PNET have been reported to date (6,13). However, it is a tumor with highly malignant features, high MIB-1 proliferative index and with small round cell pattern which is quite different from the morphologic features of the PG.…”

Section: A B a Bmentioning

confidence: 99%

Cauda equina paraganglioma with ependymoma-like histology: a case presentation

Mıdı¹,

Yener²,

Sav³

et al. 2010

Turkish Neurosurgery

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Paraganglioma affecting the cauda equina region is very rare and can be misinterpreted as an ependymoma which is more common at this site. A 38-year-old woman with a paraganglioma in the cauda equina is presented. MRI revealed a well-circumscribed, intradural, extramedullary tumor nodule with the dimensions of 2.5x1x1 cm. The patient underwent L3 laminectomy and total excision of the tumor. The tumor was diagnosed as ependymoma and the patient was decided to undergo adjuvant radiotherapy. The patient applied to our medical center for a second opinion. Histopathologically, her tumor was found to be a paraganglioma with ependymal features. Therefore no adjuvant therapy was applied. There is no evidence of recurrence or metastases for 15 months after her operation. Paraganglioma in the cauda equina/ filum terminale is very rare and can be misdiagnosed as ependymoma especially when it exhibits ependymoma-like histology. This rare form of paraganglioma behaves like a WHO grade I tumor of CNS like classic paraganglioma. No recurrence or metastasis is expected when it is totally resected. Morphology can be misleading hence immunohistochemistry and/or ultrastructural study is necessary for correct diagnosis.KeywoRds: Paraganglioma, Ependymoma-like histology, Filum terminale, Immunohistochemistry, CD 99 ÖZKauda ekuina yerleşimli paraganglioma oldukça nadir olup, bu lokalizasyonda daha sık görülen ependimoma ile karışabilir. Bu çalışmada kauda ekuina yerleşimli, ependimoma benzeri histoloji gösteren paraganglioma olgusunu sunmayı amaçladık. 38 yaşındaki bayan hastaya, MR yöntemi ile saptanan 2,5x1x1 cm boyutlarda, intradural-ekstramedüller yerleşimli, iyi sınırlı kitle nedeniyle bir başka merkezde L3 laminektomi ve kitle eksizyonu uygulanmıştır. Burada tümor, ependimoma olarak rapor edilmiştir ve hastaya adjuvan radyoterapi verilmesi kararı alınmıştır. Hasta, tanı ve tedavisi konusunda ikinci bir görüş almak üzere merkezimize başvurmuştur. Merkezimizde yapılan patolojik inceleme ve immünohistokimyasal boyamalar sonucu, tümörün ependimoma benzeri histoloji gösteren paraganglioma olduğu rapor edilmiştir. Bunun üzerine hastanın ek tedavi almamasına karar verilmiştir. Hastanın, ameliyatından sonraki 15 aylık izlemi sonucunda nüks ya da metastaz bulgusu izlenmemektedir. Kauda ekuina/filum terminale yerleşimli paraganglioma oldukça nadir olup özellikle ependimoma-benzeri histoloji gösteren olan olgular, ependimoma ile karışabilmektedir. Bu tür olgularda klinik seyir, SSS tümörleri-WHO Sınıflamasında yeralan derece I tümörlerdeki gibi olup, tedavide kitlenin total eksizyonunun yapılması yeterlidir. Morfolojik bulgular yanıltıcı olabileceği için immünohistokimyasal ve/veya ultrastrüktürel yöntemler, kesin tanı için gereklidir.

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Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review

Cited by 88 publications

References 55 publications

Intracranial Peripheral-Type Primitive Neuroectodermal Tumor -Case Report-

Intracranial Peripheral-Type Primitive Neuroectodermal Tumor -Case Report-

Askin's tumor: 11 cases and a review of the literature

Cauda equina paraganglioma with ependymoma-like histology: a case presentation

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