• Journal of Korean Neurosurgical Society
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• v.49 no.6
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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.

• The Korean Journal of Cytopathology
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• v.15 no.2
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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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• v.15 no.12
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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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• v.44 no.4
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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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• v.9 no.1
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• pp.117-122
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• 1991

Malignant small round cell tumor (SRCT) of the thoracopulmonary region appears to originate in the soft tissues of the chest wall or the peripheral lung. A differential diagnosis of poorly differentiated small round cell tumors which include Ewing's sarcoma of bone and soft tissue, embryonal rhabdomyosarcoma, Askin tumor, neuroblastoma, peripheral neuroectodermal tumor, small cell osteogenic sarcoma and Iymphoma are often difficult by light microscopy alone. In recent, by the extensive studies electron microscopic examination, histochemical study, immune-chemical study, cytogenetics and gene analysis, these tumors may be derived from the primitive and pluripotential cells, differentiating into mesenchymal, epithelial and neural features in variable proportions. Treatment for SRCT of thoracopulmonary region is not determined because of massive involvement of the lung, pleura or soft tissues of the chest wall resulted in a dismal outcome despite aggressive surgery, irradiation and chemotherapy.