• Title/Summary/Keyword: Neuroepithelial tumors

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Spinal Cord Tumors : An Analysis of 654 Cases(1973-1999) (척수 종양 654예의 임상 분석(1973-1999))

  • Choe, Woo Jin;Chung, Chun-Kee;Cho, Byung-Kyu;Kim, Hyun Jib
    • Journal of Korean Neurosurgical Society
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    • v.30 no.8
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    • pp.1004-1012
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    • 2001
  • Objective : The spinal cord tumors(including vertebral tumors) are increasingly diagnosed and operated due to development of refined diagnostic and therapeutic tools. It is necessary to re-evaluate clinical features and surgical results of spinal cord tumors with increasing cases and developing treatment modalities. The authors reviewed the spinal cord tumor cases to evaluate their clinical characteristics. Material and Methods : The retrospective review of 654 cases of spinal cord tumors between 1973 and 1999 was done. The clinical features, pathological analysis and surgical results were analyzed and compared to the literature. The results of the study are analyzed with a more detailed consideration of each of major pathologies : neurogenic tumors, meningeal tumors, neuroepithelial tumors, and metastatic tumors. Results and Conclusion : The spinal cord tumor was most common in the 5th decade of age(145 cases, 22.1%) and 78 cases(11.9%) were found in children under 15 years of age. The ratio of male to female was 1.2 : 1. The pathologic diagnosis was neurogenic tumor in 266 cases(40.7%), neuroepithelial tumor in 131(20.0%), metastatic tumor in 118(18.0%), and meningeal tumor in 94(14.4%) in the order of frequency. The tumor was located most frequently in the thoracic area(36.5%) and in the intradural extramedullary space(38.1%). The most common initial presentation was pain(40.1%) and the mean duration for presentation to operation was 14.8 months. The total or gross total removal was possible in 404 cases(61.7%) and the surgical result on the postoperative one month was recovery or improvement in 424 cases(64.8%), stationary in 188(28.7%), progression in 42(6.4%). As a surgical complication, there was a spinal deformity(12 cases), wound infection(5 cases), aspiration pneumonia(5 cases) etc. Neurogenic tumors and menigiomas showed good surgical results, whereas neuroepithelial tumors(except ependymoma) and metastatic tumors showed relatively poor prognosis.

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Factors associated with seizure and cognitive outcomes after epilepsy surgery for low-grade epilepsy-associated neuroepithelial tumors in children

  • Ko, Ara;Lee, Joon Soo
    • Clinical and Experimental Pediatrics
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    • v.63 no.5
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    • pp.171-177
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    • 2020
  • Low-grade epilepsy-associated neuroepithelial tumors (LEATs) are responsible for drug-resistant chronic focal epilepsy, and are the second-most common reason for epilepsy surgery in children. LEATs are extremely responsive to surgical treatment, and therefore epilepsy surgery should be considered as a treatment option for LEATs. However, the optimal time for surgery remains controversial, and surgeries are often delayed. In this review, we reviewed published article on the factors associated with seizure and cognitive outcomes after epilepsy surgery for LEATs in children to help clinicians in their decision whether to pursue epilepsy surgery for LEATs. The achievement of gross total resection may be the most important prognostic factor for seizure freedom. A shorter duration of epilepsy, a younger age at surgery, and extended resection of temporal lobe tumors have also been suggested as favorable prognostic factors in terms of seizure control. Poor cognitive function in children with LEATs is associated with a longer duration of epilepsy and a younger age at seizure onset.

A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures

  • Han, Jee-Yeon;Yum, Mi-Sun;Kim, Eun-Hee;Hong, Seokho;Ko, Tae-Sung
    • Clinical and Experimental Pediatrics
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    • v.59 no.sup1
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    • pp.139-144
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    • 2016
  • Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.

Clinical Pearls and Advances in Molecular Researches of Epilepsy-Associated Tumors

  • Phi, Ji Hoon;Kim, Seung-Ki
    • Journal of Korean Neurosurgical Society
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    • v.62 no.3
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    • pp.313-320
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    • 2019
  • Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.

A Case of Angiocentric Glioma with Unusual Clinical and Radiological Features

  • Rho, Gyung-Jun;Kim, Hyo-Joon;Kim, Hyoung-Ihl;Ju, Myoung-Jin
    • Journal of Korean Neurosurgical Society
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    • v.49 no.6
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    • pp.367-369
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    • 2011
  • Angiocentric glioma was recently recognized as a distinct clinicopathological entity in the 2007 World Health Organization classification of tumors of the central nervous system. Typically, it presents with seizure in children and young adults. However, our patient did not have a history of seizure. Seizure did not occur up to 6 months after operation. Although it usually does not have calcification brain magnetic resonance imaging in our patient showed T1-hyperintense and T2-hypointense signals with calcification.

Intracranial Germ Cell Tumor in the Molecular Era

  • Phi, Ji Hoon;Wang, Kyu-Chang;Kim, Seung-Ki
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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    • pp.333-342
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    • 2018
  • Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.

Transition over 35 Years in the Incidence Rates of Primary Central Nervous System Tumors in Shanghai, China and Histological Subtyping Based on a Single Center Experience Spanning 60 Years

  • Shen, Fang;Wu, Chun-Xiao;Yao, Yu;Peng, Peng;Qin, Zhi-Yong;Wang, Yin;Zheng, Ying;Zhou, Liang-Fu
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.12
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    • pp.7385-7393
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    • 2013
  • Background: Only few epidemiological data on primary central nervous system (CNS) tumors in Shanghai have been reported. Methods: All cases of primary CNS tumors that were registered at Center for Disease Control and Prevention (CDC) were collected (1973-2007: urban Shanghai; 2003-2007: whole Shanghai city). Trends were analyzed using joinpoint analysis and rates were stratified by age, gender and region. Histological data were collected from both CDC and Huashan Hospital. Results: From 1973 to 2007, the five-year average incidence rate in urban Shanghai increased in both genders, especially in the elderly population. Joinpoint analysis showed the age-adjusted incidence rate for males increased first but then plateaued, whilst rates for females continued increasing over the 35 years. For the five-year status quo (2003-2007), rural had a higher age-adjusted incidence rate than urban populations, and females higher than males, especially those with advanced age. According to CDC (2003-2007) and Huashan Hospital (1951-2011), the two most common histological subtypes were neuroepithelial tumors (with male predominance) and meningiomas (with female predominance). Conclusions: In Shanghai, a steadily increased incidence rate of primary CNS tumors was observed in general, and in the elderly and female population in particular.

Transventricular Biopsy of Brain Tumor without Hydrocephalus Using Neuroendoscopy with Navigation

  • Song, Ji-Hye;Kong, Doo-Sik;Seol, Ho-Jun;Shin, Hyung-Jin
    • Journal of Korean Neurosurgical Society
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    • v.47 no.6
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    • pp.415-419
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    • 2010
  • Objective : It is usually difficult to perform the neuroendoscopic procedure in patients without hydrocephalus due to difficulties with ventricular cannulation. The purpose of this study was to find out the value of navigation guided neuroendoscopic biopsy in patients with peri- or intraventricular tumors without hydrocephalus. Methods : Six patients with brain tumors without hydrocephalus underwent navigation-guided neuroendoscopic biopsy. The procedure was indicated for verification of the histological diagnosis of the neoplasm, which was planned to be treated by chemotherapy and/or radiotherapy as the first line treatment, or establishment of the pathological diagnosis for further choice of the most appropriate treatment strategy. Results : Under the guidance of navigation, targeted lesion was successfully approached in all patients. Navigational tracking was especially helpful in entering small ventricles and in approaching the third ventricle through narrow foramen Monro. The histopathologic diagnosis was established in all of 6 patients : 2 germinomas, 2 astrocytomas, 1 dysembryoplastic neuroepithelial tumor and 1 pineocytoma. The tumor biopsy sites were pineal gland (n = 2), suprasellar area (n = 2), subcallosal area (n = 1) and thalamus (n = 1). There were no operative complications related to the endoscopic procedure. Conclusion : Endoscopic biopsy or resection of peri- or intraventricular tumors in patients without hydrocephalus is feasible. Image-guided neuroendoscopic procedure improved the accuracy of the endoscopic approach and minimized brain trauma. The absence of ventriculomegaly in patients with brain tumor may not be served as a contraindication to endoscopic tumor biopsy.

Neuroendocrine Carcinoma of the Lung - A Report of two Cases - (Neuroendocrine carcinoma of the lung 치험 2례)

  • 문준호
    • Journal of Chest Surgery
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    • v.25 no.8
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    • pp.806-811
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    • 1992
  • The primary neuroendocrine carcinomas[NEC] of the lung are relatively rare. There are classified histologically into the following groups. 1] bronchial carcinoid, 2] well differentiated neuroendocrine carcinoma[WDNC], 3] intermediate cell neuroendocrine carci-noma[ICNC], 4] small cell neuroendocrie carcinoma[SCNC]. The NE cells can be found throughout the mucosa of the bronchial tree by the electron microscopy. By the immunocytochemistry, Neuron-specific enolase[NSE], serotonin, born-csin, and calcitonin are found in normal solitary NE cells and neuroepithelial bodies. The immunohistochemistry has the advantage that it can be applied on routine pathology specimens. The NSE was localized in all of the NE granule bearing tumors but also present in 57 per cent of the non NE carcinomas. Together the combination of chromogranin A, bombesin and NSE should provide an exellent signature for tumor cells expressing NE features, such as carcinoids and other NE carcinomas. We have experienced two cases of primary pulmonary NEC in 59 and 29 years old men. The former was admitted due to exertional dyspnea and left chest pain, the latter, newly developed mass on chest X-ray. They were treated by Lt pneumonectomy with LN dissection and RLL resection with LN dissection The postoperative diagnoses were could be confirmed by NSE study as NEC which were SCNEC and INEC in subgroups. The postoperative courses were uneventful.

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Outcome of the Teratoma in Pediatric Surgical Patients (소아외과 환자에서 기형종의 치료 결과)

  • Lee, Ho-Kyun;Park, Kyung-Sub;Choi, Soo-Jin-Na;Kim, Shin-Kon;Chung, Sang-Young
    • Advances in pediatric surgery
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    • v.9 no.2
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    • pp.94-97
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    • 2003
  • The teratoma is a unique complex neoplasm and is one of the most frequent pediatric tumors originated from the extragonadal germ cells. Mature teratoma is composed of mature differentiated tissues, while immature teratoma always contains embryonic tissues of variable degrees of immaturity, especially in the neuroepithelial elements. Diagnosis of teratoma is relatively easy by conventional radiologic study, but the immaturity can be identified only by histopathological examination. Between January 1993 to December 2002, 63 cases of teratoma were operated and analysed retrospectively at the Chonnam University Hospital Female to male ratio was about 3:1 and age distribution was relatively even. Among 63 cases, gonadal teratoma was the most common (52.4%), followed by sacrococcygeal (25.4%), retroperitoneal (9.5%) and mediastinal teratoma (9.5%). Fifty-six cases were mature teratomas and seven were immature teratomas. Alpha-fetoprotein (AFP) was elevated in 4 of 6 immature cases, but in 2 of 51 mature ones Elevated AFP progressively returned to normal range by 1 month after operation in all. Complete excision of the mass was performed, and major complication was not noticed. In five immature cases, PEB chemotherapy (Cisplatin, Etoposide, Bleomycin) was performed. Two of 2 cases in histological grade II were well tolerated to the aggressive chemotherapy. One of three cases in grade III expired due to severe bone marrow depression, and two of them expired by tumor recurrences. In conclusion, immature teratoma in histological grade III showed high potentiality of recurrence. Therefore, postoperative chemotherapy has to be applied to the high graded immature tumors.

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