• Journal of Korean Neurosurgical Society
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• 제49권6호
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• pp.359-362
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• 2011

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an unusual malignancy with aggressive behavior. ES/PNET in the cerebellopontine angle (CPA) is extremely uncommon, and we report on a rare case here. A 31-year-old man presented with one month history of left facial palsy, hearing loss, swallowing difficulty, and hoarseness. Magnetic resonance images showed a large mass in the left CPA and a small one in the right cerebellar hemisphere. The patient underwent a surgery for the CPA mass lesion, and the pathology was compatible with ES/PNET. Radiation therapy and chemotherapy were administered. In contrast to the initial radiologic findings resembling vestibular schwannoma or meningioma, ES/PNET had several distinct clinical features. A patient with a CPA mass and presenting unusual clinical features should be suspected of having a rare malignancy.

• 대한세포병리학회지
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• 제15권2호
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• pp.120-125
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• 2004

Ewing's sarcoma (ES)/PNET is common in both axial and appendicular skeletons, but is extremely rare in the talus. Here, we report a case of ES/PNET of the left talus in a 29-year-old male patient diagnosed by fine needle aspiration cytology, and review the literature on similar cases. The cytological smears were composed of individually dispersed small round cells and occasional clusters of loosely cohesive cells. The tumor cells were fragile, frequently exhibiting naked nuclei. Two distinct types of cells were observed. The light (chief) cells displayed round or slightly oval nuclei with frequent indentations, generally inconspicuous nucleoli, and a thin rim of cytoplasm, which sometimes harbored small vacuoles. The dark cells were smaller, displaying scanty cytoplasm with dense hyperchromatic nuclei, intermixed with chief cells, and often manifesting as small molded groups. However, no significant nuclear pleomorphisms or mitoses were noted. Tumor cells in the ceil block revealed positive cytoplasmic glycogen, as determined by a PAS stain with diastase control, and also exhibited positive immunoreactivity for CD99.

• Asian Pacific Journal of Cancer Prevention
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• 제15권12호
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• pp.4861-4864
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• 2014

Background: The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors. Materials and Methods: All patients with documented Ewing's sarcoma/primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated. Results: Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated, only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival. Conclusions: Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.

• Imaging Science in Dentistry
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• 제44권4호
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• pp.307-314
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• 2014

Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

• Radiation Oncology Journal
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• 제9권1호
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• pp.117-122
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• 1991

흉폐부 또는 흉벽에서 발생하는 악성소세포 종양군인 Ewings sarcoma, 횡문근육종, Askin tumor, 신경아세포종, PNET, 임파종 등은 현미경학적 소견만으로는 감별하기 어렵다. 그러나 최근에는 조직세포화학적검사, 면역세포화학적검사, 세포배양, 세포유전학적 검사등의 도움으로 상기한 악성소세포 종양군들이 모두가 같은 계통의 primitive pluripotent cells로 부터 분화되어 발병되는 것으로 확인되었다. 치료는 외과적 절제술, 방사선치료, 항암요법 등이 시도되고 있으나 예후는 재발 과원격전이로 인하여 불량한 것으로 보고되고 있다. 본 저자들은 본원에서 치료한 예를 보고하면서 흉폐부에서 발생하는 악성소세포 종양군의 조직발생과 감별진단에 대하여 논하고자 한다.