• 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.

• 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.

• 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.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1422-1427
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• 1992

Askin tumor is rare malignant small round cell tumor that orgins from interconstal nerve of chest in children It was not until 1979 that Askin first reported that tumor. Although few sporadic reports had been reported, its incidence were too low to analize its clinical featurs. That tumors prognosis is so grave that no therapy would success to cure, but early diagnosis and enbloc excision with following combind chemotherapy and radiotherapy will prolong their survival. Other small round cell tumors of chest wall that must differentiate are Ewing`s sarcoma, rhabdomyosarcoma, lymphoma, neuroblastoma and pulmonary bla-stoma. The most prominant histologic charactersistics of this tumor is neuron specific eno-lase which is detected with immunohistochemistry technique, and neurosecretary electron dense granules within cytoplasm. We expirienced a case of Askin tumor occuring 12-year-old female who has huge right lower chest mass with dull chest pain. She have been underwent excision and postoperative radiotherapy. We are following her up for months and there is no evidence of local recurrence.

• The Korean Journal of Cytopathology
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• v.9 no.1
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• pp.21-28
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• 1998

Cytologic evaluation of cerebrospinal fluid(CSF) is an effective tool in diagnosing many disorders involving the central nervous system(CNS). CSF examination has been found to be of particular value in the diagnosis of metastatic carcinoma, lymphomatous or leukemic involvement of CNS and certain primary CNS tumors. As a survey of metastatic tumors to CSF and an evaluation of the preparation techniques increasing cellular yield in our laboratory, 713 CSF specimens examined between July 1995 and April 1997(1 year 10 months), were reviewed. There were 75 positive and 5 suspicious cases, the latter have had no evidence of tumors clinically. Primary tumors of 75 positive cases were classified as follows; 4(5.3%) as primary brain tumors, 40(53.3%) as secondary carcinomas, 13(17.3%) as leukemias, and 18 (24.0%) as lymphomas. The most common primary site of metastatic carcinomas was the lung in 17 cases(42.5%) followed by the stomach in 13(32.5%), breast in 8 (20.0%), and unknown primary in 2(5.0%). Four primary brain tumors were 3 cerebellar medulloblastomas and a supratentorial primitive neuroectodermal tumor (PNET). All 40 metastatic carcinomas were adenocarcinoma presented as single cells or cell clusters. Although signet ring cells were frequent in the cases of gastric primary cancers, no significant cytologic differences according to the primary site were observed. The cytologic features of leukemia and lymphoma were characterized by hypercellular smears presenting as individual atypical cells with increased N/C ratio, presence of nucleoli, and nuclear protrusions. In medulloblastomas and PNET, the principal cytologic findings were small undifferentiated cells arranged singly or in loose clusters with occasional rosettoid features. This study suggests that the CSF cytology is useful in the diagnosis of malignancy, especially metastatic extracranial tumors and the diagnostic accuracy can be improved by increasing cellular yield using cytocentrifuge.

• Archives of Plastic Surgery
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• v.37 no.4
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• pp.477-480
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• 2010

Purpose: Ganglioneuromas are well-differentiated tumors derived from neuroectodermal neural crest cells. Although these tumors can occur anywhere along the sympathetic chain from the base of the skull to the pelvic cavity, they usually develop in the posterior mediastinum and retroperitoneum these tumors are rarely found in the cervical region. Method: We report the case of a 16-year-old male patient with neurofibromatosis type 1 who was admitted because of a palpable mass centrally located on the left side of the neck. A preoperative contrast-enhanced neck computed tomography image showed a low-density homogeneous mass on the parapharyngeal space along with marked displacement of the trachea and carotid vessels. Round and soft masses were also detected on both axillae. Results: The patient subsequently underwent complete excision of the neck mass via the transcervical approach. The mass was smooth and well encapsulated between the sternocleidomastoid muscle and the trachea. Further, the mass appeared to arise from the cervical sympathetic chain, which was preserved during surgery. Both the axillary masses were also excised. The histopathological findings were ganglioneuroma for the neck mass and neurofibroma for both the axillary masses. Conclusion: Cervical ganglioneuromas are rare tumors that present as enlarging parapharyngeal cervical masses in the oropharynx or neck. To our knowledge, a case of cervical ganglioneuroma associated with neurofibromatosis type 1 has never been reported. In patients with neurofibromatosis, multiple tumors may develop, and therefore periodic clinical and radiological follow-up is recommended. Further, repeated imaging analysis should be performed if the presence of another tumor is suspected.

• 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.

• Korean Journal of Head & Neck Oncology
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• v.37 no.1
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• pp.39-42
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• 2021

Schwannomas are benign tumors of neuroectodermal origin and can be found in any part of the body. Although schwannomas are common in the head and neck region, they are rare in the auricle. Auricular schwannomas are rarely symptomatic but the mass itself or a surgical procedure to remove it may disrupt the complicated structure of the auricle. We report a rare case of auricular schwannoma with a literature review and describe the surgical technique used in this case.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.7
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• pp.3065-3070
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• 2014

Background: Neuroblastoma (NB), is a neuroectodermal tumor derived from neural crest cells, and it is the second most common pediatric malignant tumor. The biological and clinical behavior of NB is very heterogeneous. This study was conducted to evaluate the expression of Ki-67, p53 and VEGF markers in tissues obtained from NB patients with different histologic types and stage. Materials and Methods: Tissue microarray (TMA) blocks were constructed from paraffin blocks of the NB tissues. Immunohistochemical staining was performed on TMA sections to detect the expression of Ki-67, p53 and VEGF markers. The association between the expression of these markers and clinicopathological parameters were then analyzed. Results: We had 18 patients with NB, one patient with ganglioneuroblastoma (GNB) and one with ganglioneuroma. Ki-67 was expressed in 13 (65%) tumors, and negatively correlated with age, prognosis, histologic type and stage of NB (all p<0.05). High and moderate expression of VEGF was found in 5% (1/20) and 65% (13/20) of the tumors, respectively; and it was positively correlated with age, prognosis and histologic types (all p<0.05) and negatively correlated with MKI (mitosis-karyorrhexis index). p53 expression was observed in 10% (2/20) of the tumors, which showed a relative correlation with MKI (p value=0.07). Conclusions: VEGF as a candidate for anti-angiogenic targeted therapy was correlated with the development and progression of NB; therefore, VEGF along with Ki-67 can serve as a valuable marker for the prognosis of this tumor type.