• 제목/요약/키워드: primitive neuroectodermal tumor

검색결과 21건 처리시간 0.029초

흉폐부에서 발생한 악성소세포 종양의 방사선치료 (Radiation Treatment for Malignant Small Cell Tumor of the Thoracopulmonary Region (Primitive Pluripotent Histogenesis and Differential Diagnosis - A Case Report and Review of Literatures -))

  • 오원용;양진영;황인순
    • 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.

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Receiver Operating Characteristic Curve Analysis of SEER Medulloblastoma and Primitive Neuroectodermal Tumor (PNET) Outcome Data: Identification and Optimization of Predictive Models

  • Cheung, Min Rex
    • Asian Pacific Journal of Cancer Prevention
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    • 제15권16호
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    • pp.6781-6785
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    • 2014
  • Purpose: This study used receiver operating characteristic curves to analyze Surveillance, Epidemiology and End Results (SEER) medulloblastoma (MB) and primitive neuroectodermal tumor (PNET) outcome data. The aim of this study was to identify and optimize predictive outcome models. Materials and Methods: Patients diagnosed from 1973 to 2009 were selected for analysis of socio-economic, staging and treatment factors available in the SEER database for MB and PNET. For the risk modeling, each factor was fitted by a generalized linear model to predict the outcome (brain cancer specific death, yes/no). The area under the receiver operating characteristic curve (ROC) was computed. Similar strata were combined to construct the most parsimonious models. A Monte Carlo algorithm was used to estimate the modeling errors. Results: There were 3,702 patients included in this study. The mean follow up time (S.D.) was 73.7 (86.2) months. Some 40% of the patients were female and the mean (S.D.) age was 16.5 (16.6) years. There were more adult MB/PNET patients listed from SEER data than pediatric and young adult patients. Only 12% of patients were staged. The SEER staging has the highest ROC (S.D.) area of 0.55 (0.05) among the factors tested. We simplified the 3-layered risk levels (local, regional, distant) to a simpler non-metastatic (I and II) versus metastatic (III) model. The ROC area (S.D.) of the 2-tiered model was 0.57 (0.04). Conclusions: ROC analysis optimized the most predictive SEER staging model. The high under staging rate may have prevented patients from selecting definitive radiotherapy after surgery.

대퇴부 지방육종 수술 후 발생한 반대측 근위 경골의 유잉육종/원시 신경 외배엽 세포종 (Double Primary Presentation of Liposarcoma and Ewing's Sarcoma: A Case Report)

  • 박형원;정성택;나선윤
    • 대한골관절종양학회지
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    • 제17권2호
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    • pp.95-99
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    • 2011
  • 악성 연부조직 종양 수술 후 이시성(metachronous)으로 발생하는 악성 골졸양은 매우 드물다. 이시성 종양은 두 경부 또는 복부에 주로 발생하며, 사지에 발생하는 경우는 더욱더 드물다. 저자들은 48세 남자환자에서 근위 대퇴부 악성 지방 육종의 광범위 변연 절제술 후 발생한 근위 경골 유잉육종/원시 신경 외배엽 세포종 1예를 경험하여 문헌 고찰과 함께 보고하는 바이다. 육종 환자들에게서는 이와 같이 이차적으로 악성 종양이 발생할 확률이 높아지기 때문에 추시 기간 동안 잠복 종양(occult tumor)에 대한 검사가 필요하겠다.

소아에서 피하매몰 중심정맥포트가 전복된 사례에 대한 경험 - 2예 보고 - (Inversion of Implantable Central Venous Port in Children - 2 Cases Report -)

  • 최준영;김현영;정성은;박귀원;김우기
    • Advances in pediatric surgery
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    • 제12권1호
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    • pp.17-23
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    • 2006
  • A 3-year-old girl with a primitive neuroectodermal tumor (PNET) and a 6-yearold girl with acute lymphoid leukemia were referred to us because of problems with their implantable central venous ports (Port-A-Cath$^{(R)}$). On physical examination, the ports were upside-down, so a needle could notbe inserted through the membrane of the port. Right lateral side view of the chest radiogram confirmed port inversion in both cases. At operation, the ports were inverted and the transfixing sutures were totally absorbed. The ports were rotated 180 degrees and anchoring sutures placed.

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천막상부 원시신경외배엽 종양의 방사선요법 (Radiotherapy of Supratentorial Primitive Neuroectodermal Tumor)

  • 김일한;유형준;조영갑;김대용
    • Radiation Oncology Journal
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    • 제15권1호
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    • pp.11-18
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    • 1997
  • 목적 : 원시신경외배엽 종양은 빈번한 국소 재발 및 광범위한 지주막하 전이를 나타내는 종양이기때문에 수술 및 항암 약물요법과 함께 전뇌척수조사를 시행하는 방사선요법으로 치료 성적을 높이고자 꾸준한 노력을 시도하여 왔으며, 이에 저자들은 복합요법을 시행한 환자들의 실패 경향, 장기 생존율과 치료 후유증 등을 분석하고자 한다. 대상 및 방법 : 총 18명의 환자들을 대상으로 분석하였으며, 1세-27세 (중앙값=5세)의 연령분포 로 12명 대 6명의 남녀비를 나타내었다. 수술적 절제범위에 따라 완전절제술이 9명, 부분절제술 이 8명, 단순 조직생검술이 1명이었다. 모든 환자에서 수술후 방사선요법을 시행하였으며 방사선 조사야는 16명에서 전뇌척수조사를, 2명에서는 각각 전뇌조사 및 원발병소조사만을 시행하였고, 조사선량은 원발병소에 3120-5800cGy(중앙값=5460cGy), 전뇌부위에 1500-4200cGy(중앙값=3600cGy), 그리고 전척수부위에 1320-3600cGy(중앙값=2400cy)이었다. 항암약물요법은 13명 의 환자들에 대해서만 실시하였다. 추적관찰기간은 1개월부터 89개월로 중앙값은 45개월이다. 결과 : 치료도중 사망한 1명을 포함 총 9명의 환자에서 0-40개월뒤 재발을 확인하였으며 재발경향은 원발병소 단독 재발이 1명, 두개내의 전이성 재발은 2명, 척수부위 재발은 4명, 광범위한 뇌척수 재발이 1명, 그리고 전신적 다발성 골전이를 나타낸 환자가 1명이었다. 전뇌척수부위 방사선요법을 시행하지않은 2명의 환자중 2명 모두 척수부위 재발을 나타내었으며, 전뇌척수부위 방사선요법을 시행한 IS명의 환자중에서는 3명이 척추부위를 포함한 재발 경향을 보였고, 재발한 9명의 환자는 모두 재발 후 1-13개월내 사망하였다. 전체 환자의 2년 및 5년 생존율은 각각 $61\%,\;49\%$이며, 예후인자 분석에서는 성별, 나이, 종양위치와는 무관하였으나 완전절제술 및 항암 약물요법을 시행한 환자군에서 높은 생존율을 나타내었다. 치료 후 후유증으로는 원발병소에 5580cGy를 조사한 환자 1명에서 40개월 뒤 자기공명영상 및 자기공명분광법으로 방사선괴사로 추정되고 있으며, 생존하는 9명의 환자에서 뇌하수체기능저하증이 2명, 그리고 인지능력저하 1명, 기억장애 2명, 성장저하$(\leq5\%)$는 1명에서 관찰되었다. 결 론 : 천막상부 원시신경외배엽 종양 환자의 치료성적을 높이기 위해서는 가능한한 종양의 완전절제가 필수적이며 수술후 항암 약물요법과 방사선요법의 보조요법이 결과를 향상시킬 수 있을 것으로 기대되며, 방사선치료시 조사부위는 전뇌척수부위를 모두 포함하여 조사하여야 한다.

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Surgical Treatment of Primary Spinal Tumors in the Conus Medullaris

  • Han, In-Ho;Kuh, Sung-Uk;Chin, Dong-Kyu;Kim, Keun-Su;Jin, Byung-Ho;Cho, Yong-Eun
    • Journal of Korean Neurosurgical Society
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    • 제44권2호
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    • pp.72-77
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    • 2008
  • Objective : The objective of this study was to evaluate the characteristics and surgical outcome of the conus medullaris tumors. Methods : We retrospectively reviewed 26 patients who underwent surgery for conus medullaris tumor from August 1986 to July 2007. We analyzed clinical manifestation, preoperative MRI findings, extent of surgical resection, histopathologic type, adjuvant therapy, and outcomes. Results : There were ependymoma (13), hemangioblastoma (3), lipoma (3), astrocytoma (3), primitive neuroectodermal tumor (PNET) (2), mature teratoma (1), and capillary hemangioma (1) on histopathologic type. Leg pain was the most common symptom and was seen in 80.8% of patients. Pain or sensory change in the saddle area was seen in 50% of patients and 2 patients had severe pain in the perineum and genitalia. Gross total or complete tumor resection was obtained in 80.8% of patients. On surgical outcome. modified JOA score worsened in 26.9% of patients, improved in 34.6%, and remained stable in 38.5%. The mean VAS score was improved from 5.4 to 1.8 among 21 patients who had lower back pain and leg pain. Conclusion : The surgical outcome of conus medullaris tumor mainly depends on preoperative neurological condition and pathological type. The surgical treatment of conus medullaris tumor needs understanding the anatomical and functional characteristics of conus meudllaris tumor for better outcome.

Characterization of Brain Tumor Cell using Vasopressin-SV40 T Ag Transgenic Mouse

  • Kim, Sung-Hyun;Lee, Eun-Ju;Kim, Myoung-Ok;Park, Jun-Hong;Kyoungin-Cho;Jung, Boo-Kyung;Kim, Hee-Chul;Hwang, Sol-Ha;Lee, Hoon-Taek
    • 한국동물번식학회:학술대회논문집
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    • 한국동물번식학회 2003년도 학술발표대회 발표논문초록집
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    • pp.44-44
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    • 2003
  • In previous reports, pVPSV.IGR2.1 transgenic mouse were described that brain tumor and lymphoma by reason of Vasopressin-SV40 T antigen. In this study, we produced pVPSV.IGR3.6 transgenic mouse that used pVPSV.IGR3.6 vector. Expression of transgene was vary different in transgenic mouse. We obtained 6 transgenic mouse line, moreover they had died at the age of 2~6 weeks without transmitting the transgene to their offspring, and had tumorigenesis on same location with pVPSV.IGR2.1 transgenic mouse. Only a founder mouse was investigated for expression of fusion gene. Here we extended this transgenic approach to the study of tumor progression. From the mouse, we confirmed brain tumor cell, after then cultured for investigate characterization. In this report, we demonstrate that reduction of survival rate in transgenic mouse fused vasopressin gene length, acquisition of brain tumor cell, composition with astrocyte cells and neuronal cells. Finally, cells had no change with increase of passage.

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원발성 뇌종양의 세포학적 소견 (Cytologic Features of Primary Tumors in Central Nervous System)

  • 옥순애;정재걸;공경엽;최기영;유은실;이인철
    • 대한세포병리학회지
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    • 제5권2호
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    • pp.90-98
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    • 1994
  • There has been a marked increase in the utility of aspiration cytology for pathologic diagnosis. It may be applied to any kinds of organs and substitutes surgical biopsy. Be cause of the high risk of complication and difficulties In localization, aspiration cytology in the central nervous system (CNS) has been used with less frequency compared to other sites. However, with the advent of sophisticated imaging instruments, aspiration cytology of lesions in the CNS is being used increasingly. We present cytologic features of brain tumors as well as corresponding histologic findings. Eight types of tumors were aspirated intraoperatively and stained with the Papanicolaou method; 1 anaplastic astrocytoma, 1 glioblastoma multiforme, 1 ependymoma, 1 malignant ependymoma, 1 central neurocytoma, 1 primitive neuroectodermal tumor, 1 benign neurogenic tumor and 1 germinoma. Cytologic features of the CNS neoplasms were quite similar to those of histology except one spindle cell tumor. Reviewing various CNS neoplasms, it appears that cytology may be a useful diagnostic method.

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뇌척수액에서 진단된 악성 종양세포의 세포학적 분석 (Cytologic Analysis of Malignant Tumor Cells in Cerebrospinal Fluid)

  • 서재희;공경엽;강신광;김온자
    • 대한세포병리학회지
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    • 제9권1호
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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.

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Survival of Patients with Ewing's Sarcoma in Yazd-Iran

  • Akhavan, Ali;Binesh, Fariba;Shamshiri, Hadi;Ghanadi, Fazllolah
    • 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.