• Journal of Korean Neurosurgical Society
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• v.45 no.1
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• pp.39-42
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• 2009

We report a supratentorial primitive neuroectodermal tumor (sPNET) in 17-year-old primipara in the second trimester her pregnancy. Magnetic resonance imaging revealed a left frontoparietal mass with solid and cystic component. Gross-total resection was achieved via a left frontoparietal craniotomy. It was decided to suspend the radiotherapy and chemotherapy until the 30 weeks of gestation. But, a sudden uncal herniation was developed due to the reccurrence of the tumor and bleeding into the tumor at the 25 weeks of gestation and the patient died after urgent decompressive surgery. sPNETs is an extremely rare brain tumor in pregnancy and only two cases were reported in the literature to date. There is no universally agreed treatment protocol for sPNETs during pregnancy and a multidisciplinary approach is required in treatment. In the present study, the clinical, histopathological features and therapeutical difficulties of sPNETs diagnosed during pregnancy was discussed with the literature review.

• Kosin Medical Journal
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• v.33 no.2
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• pp.263-270
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• 2018

Primitive neuroectodermal tumor (PNET) arising primarily in the lung is an extremely rare and aggressive malignancy with poor chances of patient survival. We present a case of long-term survival by a 29-year-old woman with PNET diagnosed after a hertological and immunohertochemical examination of a biopsy specimen obtained by performing video-assisted thoracic surgery. The patient underwent a left lower lung lobe lobectomy and 6 cycles of adjuvant chemotherapy. The patient has been free of any symptoms of the recurrence of the disease for 6 years after treatment completion.

• Annals of Clinical Neurophysiology
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• v.23 no.2
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• pp.126-129
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• 2021

We present a patient with a primitive neuroectodermal tumor arising from the right atrium who experienced multiple syncope episodes daily, which had first appeared 1 month after surgery. The symptoms continued to worsen over the course of chemotherapy, and the autonomic function test (AFT) was performed after the 14th chemotherapy cycle. The AFT revealed orthostatic hypotension and reduced baroreflex-dependent sympathetic reactivity. Physical counterpressure techniques were applied with a visual biofeedback intervention, and were found to be effective in reducing the syncope episodes.

• 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.

• Journal of Korean Neurosurgical Society
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• v.37 no.3
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• pp.193-196
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• 2005

Objective: Strategies for managing supratentorial primitive neuroectodermal tumors(PNETs) in children include surgical resection, craniospinal irradiation and chemotherapy. This study is performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. Methods: We have reviewed all medical records and pathology slides of six children(four males and two females) with supratentorial PNET from November, 1987 to May, 2003. The extent of resection was confirmed by computed tomography and magnetic resonance studies. Results: The patients were aged 1 to 13 years and treated postoperatively with/without adjuvant therapy. Tumor location included was four cortical, one gangliobasal, and one pineal region. The presenting symptoms and signs consisted of increased intracranial pressure and focal neurological deficits such as seizure and hemiparesis. The treatment consisted of surgical resection alone in one patient, postoperative radiotherapy in one patient, postoperative chemotherapy in one, and postoperative radiotherapy with chemotherapy in three. Five patients lived more than 12 months after diagnosis and one patient among them has been living more than 5 years after diagnosis. Conclusion: We can improve the survival and prognosis of supratentorial PNET patients by radical gross total resection of tumor followed by craniospinal irradiation and aggressive chemotherapy. First of all, gross total resection of tumor is the most important among many factors.

• 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.

• 대한두경부종양학회:학술대회논문집
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• 1999.12a
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• pp.271.1-271.1
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• 1999

• Nuclear Medicine and Molecular Imaging
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• v.43 no.4
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• pp.366-367
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• 2009

Ga-67 scintigraphy demonstrated increased uptake in the lungs and stomach in a 26-year-old man with hypercalcemia. A primitive neuroectodermal tumor was confirmed by bone marrow examination. Tc-99m MDP uptake in the same locations as Ga-67 revealed by bone scintigraphy was consistent with metastatic calcification. Although the mechanism of Ga-67 uptake in metastatic calcification is not understood, the presence of an inflammatory process is suggested.

• Proceedings of the KSAR Conference
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• 2001.03a
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• pp.47-47
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• 2001

The neuropeptide vasopressin (VP) is a nine- amino acid hormone synthesized as preprohormone in the cell bodies of hypothalamic magnocellular neurons. The tumor in magnocellular neurons of the hypothalamus is associated with disfunctions of the cell bodies, leading to the diabetes insipidus. In order to study with the diabetes insipidus caused by a defect in VP synthesis and its secretion, we have produced the transgenic mice regulated by vasopressin promoter inserted to SV40 T antigen coding sequence (pVPSV.IGR2.1). One transgenic line expressing high levels of SV40 T antigen was propagated. The founder and all transgene positive adult animals have appeared with shorten mortality or apparent phenotypic abnormalities, including immune complex disease, and eventually die between 4 and 8 months of age. The mRNA and protein of SV40T antigen transgene were detected in brain of fetus as well as in brain, spleen, lung and lymph node in moribund at the age of 20 weeks. Histological analysis of transgenic mice showed that tumor developed in brain similar to primitive neuroectodermal tumors (PNET) in man. We also detected lymphomas in spleen and lymph node, and consequent tumor formation in various tissues of the transgenic mice. In pVPSV.IGR2.1, 21% mice showed brain tumor (PNET) at 5 weeks and 100% mice showed brain tumor after 15 weeks. In addition, Expression of apoptosis related genes (Bcl-28 & Bax) was increased over their age in mice with PNET as compared to control mice. Apoptosis related gene expression might be deregulated in mice with brain tumor. However, transgenic mice were not developed with the diabetes insipidus. These mice represent the first disease model to exhibit primitive neuroectodermal tumor in brain, as well as a unique model system for exploring the cellular pathogenesis of lymphomas.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.214-218
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• 2008

Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient's clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET.