• Title/Summary/Keyword: Germ-cell tumor

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Diffuse Leptomeningeal Glioneuronal Tumor with FGFR1 Mutation in a 29-Year-Old Male (29세 남성에서 발생한 FGFR1 돌연변이를 동반한 미만성 연수막성 신경교종)

  • Minsu Kim;Ki Rim Lee;Gheeyoung Choe;Kihwan Hwang;Jae Hyoung Kim
    • Journal of the Korean Society of Radiology
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    • v.84 no.4
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    • pp.970-976
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    • 2023
  • This study reports on diffuse leptomeningeal glioneuronal tumor (DL-GNT) in a 29- year-old male. DL-GNT is a rare central nervous system (CNS) tumor mostly seen in children and only few cases have been reported in adult patients. Our patient presented with a chronic headache that lasted for five months. MR imaging showed mild hydrocephalus, multiple rim-enhancing nodular lesions in the suprasellar cistern, diffuse leptomeningeal enhancement in the lumbosacral area, and multiple small non-enhancing cyst-appearing lesions not suppressed on fluid attenuated inversion recovery (FLAIR) images in the bilateral basal ganglia, thalami, and cerebral hemispheres. Under the impression of germ cell tumor with leptomeningeal seeding, the patient underwent trans-sphenoidal tumor removal. DL-GNT was pathologically confirmed and FGFR1 mutation was detected through a next-generation sequencing test. In conclusion, a combination of leptomeningeal enhancement and multiple parenchymal non-enhancing cyst-appearing lesions not suppressed on FLAIR images may be helpful for differential diagnosis despite overlapping imaging features with many other CNS diseases that have leptomeningeal enhancement.

A Case of Bleomycin Induced Bronchiolitis Obliterans Organizing Pneumonia (Bleomycin에 의해 유발된 Bronchiolitis Obliterans Organizing Pneumonia 1예)

  • Oh, Hye-Lim;Kang, Hong-Mo;Choi, Cheon-Woong;Lee, Ho-Jong;Cho, Yong-Seun;Yoo, Jee-Hong
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.4
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    • pp.504-509
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    • 2001
  • There are numerous agents with potential toxic effects on the lung. In particular, cytotoxic drugs constitute the largest and most important group of agents associated with lung toxicity. Bleomycin is commonly used, either alone or in combination with other chemotherapeutic agents, in the treatment of squamous cell carcinoma(head and neck, esophagus, and genitourinary tract), lymphoma, and germ cell tumor. One of the therapeutic advantages of bleomycin is its minimal bone marrow toxicity. However, pulmonary toxicity is one of the most serious adverse side effects. Classically, pulmonary toxicity manifests as a diffuse interstitial process or less commonly as a hypersensitivity reaction. This pulmonary toxicity is generally considered to be dose related and can progress to a fatal fibrosis. It is also possible that bronchiolitis obliterans organizing pneumonia(BOOP) is another manifestation of bleomycin induced toxicity. Bleomycin induced BOOP is less common and has a favorable response to steroid therapy. Here we present a case that demonstrates a BOOP, secondary to a relatively small cumulative dose of bleomycin($225mg/m^2$), may be reversible.

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Endoscopic Management of Pineal Region Tumors with Associated Hydrocephalus (수두증을 동반한 송과체 부위 종양에 대한 내시경적 치료)

  • Kim, Jeong Hoon;Ra, Young Shin;Kim, Joon Soo;Ahn, Jae Sung;Kim, Chang Jin;Kwun, Byung Duk
    • Journal of Korean Neurosurgical Society
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    • v.30 no.5
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    • pp.575-580
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    • 2001
  • Purpose : In general, pineal region tumors are managed by using microsurgical approach or stereoctactic biopsy. However, in selected cases endoscopic approach to pineal lesions might prove to be as effective as microsurgery and less invasive. We report an alternative surgical strategy for managing certain patients with pineal neoplasms that allows treatment of the symptomatic hydrocephalus as well as tumor biopsy under direct vision in the same sitting. Materials and Methods : Twenty-two patients with pineal region tumors with associated hydrocephalus were treated in one session by endoscopic third ventriculostomy and endoscopic tumor biopsy at our institution from October 1996 to January 2000. All patients were retrospectively evaluated. Results : There was no operative mortality. There was one cause of significant bleeding during biopsy, but was controlled endoscopically, and the patient recovered completely without neurologic deficit resulting from intra-operative bleeding. The symptoms related to increased intracranial pressure(ICP) have resolved in all patients, and the need for a shunt is completely eliminated. Histological diagnosis was achieved in 21 of the 22 patients by this procedure. A biopsy was not obtained in one patient. Although this pineal region tumor was seen endoscopically, this could not be biopsied because of technical difficulties in working around an enlarged massa intermedia. The lesions included fourteen germinomas, three mixed germ cell tumors, and one each of the followings: pineocytoma, pineoblastoma, pineocytoma/pineoblastoma(intermediate type), meningioma, and low grade glioma. Five of the 22 patients subsequently underwent formal microsurgical tumor removal. Additional chemotherapy or radiotherapy could then be initiated according to the histological diagnosis. Conclusion : We consider that endoscopy affords a minimally invasive way of reaching three objectives by one-step surgery in the management of pineal region tumors with associated hydrocephalus : 1) cerebrospinal fluid(CSF) sample for analysis of tumour markers and cytology, 2) treatment of hydrocephalus by third ventriculostomy, and 3) several biopsy specimens can be obtained identifying tumors which will require further open surgery or adjuvant radiation and/or chemotherapy. However, complications and morbidities should be emphasized so as to be avoided with further technical experience.

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Clinical, endocrinological and radiological courses in patients who was initially diagnosed as idiopathic central diabetes insipidus (초기에 특발성 중추성 요붕증으로 진단된 환자에서 임상, 내분비학 및 방사선학적 경과)

  • Chung, Seung Joon;Lee, Seong Yong;Shin, Choong Ho;Yang, Sei Won
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1110-1115
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    • 2007
  • Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was $7.8{\pm}3.6$ (2.1-14.7) years. Mean follow-up duration was $8.6{\pm}5.1$ (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

Clinical Review and PCNA Expressions of Teratomas in Pediatric Patients (소아 기형종의 임상적 고찰 및 PCNA 발현율에 관한 연구)

  • Joo, J.H.;Choi, S.J.N.;Chung, S.Y.;Kim, S.K.
    • Advances in pediatric surgery
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    • v.1 no.2
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    • pp.122-132
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    • 1995
  • Teratoma contains elements derived from all three embryonic germ layers and mature teratomas are entirely composed of mature differentiated tissues, while immature types always contain additional embryonic tissues of variable degree of immaturity, especially neuroepithelial elements. Twenty cases of teratoma in infancy and childhood were reviewed and the clinical features and pathologic findings including PCNA expression were studied. Sacrococcygeal teratomas were the most common type(45%), followed by retroperitoneal and ovarian teratomas. There was a predilection of females in a ratio of 4:1 and age distribution was most prevalent below the first year of age(45%). But in ovarian teratomas, the age at diagnosis was above 4 years of age in all cases. Serum alpha-fetoprotein levels were checked in 18 cases. In all mature teratomas and 1 of 5 immature teratomas, the levels were normal. But in 4 of 5 immature teratomas, the serum levels were elevated and progressively declined to normal range after mass excision. Radiologically, calcifications in tumor were found in 60.0% of teratomas and was higher in mature teratomas(69.2%) than immature teratomas(42.9%). Immunohistochemical staining for PCNA(proliferating cell nuclear antigen) was done in 16 cases and PCNA expression was higher in grade III immature teratomas than grade I and II. The operative modes were complete mass excisions. Five immature teratomas were treated with multiagent PEB(Bleomycin, Etoposide, Cisplatin) adjuvant chemotherapy, 3 tolerated well without significant complications, but in one case, severe bone marrow suppression was developed and died of sepsis. In conclusion, grade III immature teratoma showed higher PCNA expression than mature or lower grade immature teratoma, which suggests that chemotherapy after surgical excision may be effective modality for grade III immature teratoma. We think, however, multicenter study is necessary because of low incidence of immature teratoma.

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Production of Polyclonal Antibody against $\alpha$-Fetoprotein and Polyclonal Antibody-Based Competitive Enzyme-Linked Immunosorbent Assay for $\alpha$-Fetoprotein (인간 $\alpha$-fetoprotein (AFP)에 대한 폴리클로날 항체의 생산 및 $\alpha$-fetoprotein 측정용 효소면역분석법 (competitive ELISA)의 개발)

  • Michung Yoon
    • Biomedical Science Letters
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    • v.3 no.2
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    • pp.115-123
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    • 1997
  • $\alpha$-Fetoprotein (AFP) has been a useful marker in screening and/or monitoring patients with hepatocellular carcinoma, gonadal germ cell tumor, gastric carcinoma and neural tube defects. In the present study, it was attempted to produce anti-human AFP polyclonal antibodies and to develop a competitive enzyme-linked immunosorbent assay (ELISA) for the measurement of AFP in human plasma and amniotic fluid. AFP was isolated from amniotic fluid using an isolation procedure consisting of affinity chromatography and preparative polyacrylamide gel electrophoresis. The antibody directed against AFP was raised in rabbits. Double immunodiffusion and Western blotting methods showed that the antiserum was highly specific, reacting with only AFP-containing samples. Standard curve was obtained by using purified AFP and specific antiserum. The assay sensitivity was 5ng/ml and the working range was 5~l,000ng/ml. The within-assay and between-assay coefficient of variance (CV) was 4.5% and 8.5%, respectively. These results indicate that the assay is valuable for the measurement of AFP and found to be simple, reproducible, and accurate.

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Characteristics of Incident Testicular Cancer in Lebanon - 1990-2015 Single Institutional Experience

  • Assi, Tarek;Nasr, Fadi;El Rassy, Elie;Ibrahim, Toni;Jabbour, Hicham;Chahine, Georges
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.4
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    • pp.1899-1902
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    • 2016
  • Background: Despite the fact that testicular cancer is a major health issue with its increasing incidence, very few studies have described its characteristics in the Middle East, particularly in Lebanon. Materials and Methods: We report in this paper a retrospective pilot study of the characteristics of testicular cancer in Lebanon. The demographic, epidemiologic and survival characteristics of 178 patients diagnosed between 1990 and 2015 at an oncology clinic affiliated to Hotel Dieu de France Hospital were analyzed. Results: The mean age at diagnosis was $32{\pm}10years$. The most prevalent testicular tumor was the germ cell type (GCT) (95.2%) of which non-seminomatous tumors (NST) were the commonest (64.7%). Most of our patients were diagnosed at an early stage. Lymph node spread affected most commonly the retroperitoneal region and distant visceral metastases occurred in 14.6%. All patients underwent orchiectomy with 67% receiving adjuvant treatment, mainly chemotherapy. After a median follow up of 2,248 days (75.9 months) 16 patients were reported dead. Two, five and ten-year overall survival rates were 96%, 94% and 89% respectively. The median overall survival rate was not reached. Conclusions: Despite being part of the developing world, demographic, epidemiologic and survival analyses of testicular cancer reported in our study are in line with those reported from developed countries and would allow us to extrapolate management plans from these populations.

Evaluation of the Kit's Efficiency of Alpha Fetoprotein (AFP) Test (Alpha feto protein(AFP)검사 키트의 유효성 평가실험)

  • Cho, Hyun-su;Noh, Gyeong-woon;You, Tae-min
    • The Korean Journal of Nuclear Medicine Technology
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    • v.22 no.2
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    • pp.101-104
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    • 2018
  • Purpose Alpha fetoprotein(AFP) is a fetal serum protein that increases in germ cell tumors derived from liver cancer or egg yolk. AFP test has been used for screening of liver cancer, determination of tumor stage, determination of therapeutic effect, and fetal congenital malformations. The purpose of this study was to compare the results of the four kits, identify the advantages and disadvantages of each kit, and select the appropriate kits for our laboratory. Materials and Methods Blood samples were obtained from 89 patients attending the Seoul national university hospital. Experiments were carried out in accordance with manufacturer's instructions of four companies(A, B, C, D). The precision, recovery, linearity, and sensitivity test were performed for each kit. Results In case of the precision within the measurement, the CV value of the C kit was less than 5% at the low, middle, and high concentrations. The A, B and D kit's the CV value was less than 5% at the concentrations except the low concentration. The recovery rates of the A, B, C, and D kits were $100{\pm}15%$, $100{\pm}30%$, $100{\pm}16%$ and $100{\pm}14%$, respectively. All kits showed good linearity. Sensitivity was measured as 0.5 IU/mL for A, 0.4 IU/mL for B, 0.98 IU/mL for C, and 0.3 IU/mL for D. Conclusion The CV values of the four kits were within 10%, and the correlation coefficients were close to 1 for $R^2=0.978$, $R^2=0.992$ and $R^2=0.8957$. As a result, they are clinically available. Therefore, each laboratory should select the appropriate kit for their experiment's environment.

Teratoma Presenting as An Unilateral Mediastinal Mass with Contralateral Pleural Effusion (편측성 종격동 종괴와 반대측 흉수로 발현된 기형종 1예)

  • Ha, Eun Sil;Hur, Gyu Young;Jung, Ki Hwan;Lee, Sung Yong;Jo, Won Min;Lee, Sang Yeub;Kim, Je-Hyeong;Lee, Eung Seok;Shin, Chol;Shim, Jae Jeong;In, Kwang Ho;Kang, Kyung Ho;Yoo, Se Hwa
    • Tuberculosis and Respiratory Diseases
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    • v.60 no.3
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    • pp.347-352
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    • 2006
  • A teratoma is the most common benign germ cell tumor that develops in the mediastinum. Patients with a mediastinal teratoma are usually asymptomatic. However, a spontaneous rupture of a mediastinal teratoma into the pleural cavity or adjacent organs can cause severe chest pain, hemoptysis, acute dyspnea, etc. Complications such as recurrent pneumonia, pericardial effusion, pleural effusion and great vessel invasion can sometimes occur. We encountered a case of a patient with an abrupt onset of dyspnea after persistent shoulder pain for one month. The X-ray examinations revealed a unilateral mediastinal mass with contralateral pleural effusion. Subsequent evaluations confirmed a spontaneous rupture of the teratoma into the contralateral pleural cavity.

Diffusion Weighted Imaging Can Distinguish Benign from Malignant Mediastinal Tumors and Mass Lesions: Comparison with Positron Emission Tomography

  • Usuda, Katsuo;Maeda, Sumiko;Motono, Nozomu;Ueno, Masakatsu;Tanaka, Makoto;Machida, Yuichiro;Matoba, Munetaka;Watanabe, Naoto;Tonami, Hisao;Ueda, Yoshimichi;Sagawa, Motoyasu
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.15
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    • pp.6469-6475
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    • 2015
  • Background: Diffusion-weighted magnetic resonance imaging (DWI) makes it possible to detect malignant tumors based on the diffusion of water molecules. It is uncertain whether DWI is more useful than positron emission tomography-computed tomography (PET-CT) for distinguishing benign from malignant mediastinal tumors and mass lesions. Materials and Methods: Sixteen malignant mediastinal tumors (thymomas 7, thymic cancers 3, malignant lymphomas 3, malignant germ cell tumors 2, and thymic carcinoid 1) and 12 benign mediastinal tumors or mass lesions were assessed in this study. DWI and PET-CT were performed before biopsy or surgery. Results: The apparent diffusion coefficient (ADC) value ($1.51{\pm}0.46{\times}10^{-3}mm^2/sec$) of malignant mediastinal tumors was significantly lower than that ($2.96{\pm}0.86{\times}10^{-3}mm^2/sec$) of benign mediastinal tumors and mass lesions (P<0.0001). Maximum standardized uptake value (SUVmax) ($11.30{\pm}11.22$) of malignant mediastinal tumors was significantly higher than that ($2.53{\pm}3.92$) of benign mediastinal tumors and mass lesions (P=0.0159). Using the optimal cutoff value (OCV) $2.21{\times}10^{-3}mm^2/sec$ for ADC and 2.93 for SUVmax, the sensitivity (100%) by DWI was not significantly higher than that (93.8%) by PET-CT for malignant mediastinal tumors. The specificity (83.3%) by DWI was not significantly higher than that (66.7%) for benign mediastinal tumors and mass lesions. The accuracy (92.9%) by DWI was not significantly higher than that (82.1%) by PET-CT for mediastinal tumors and mass lesions. Conclusions: There was no significant difference between diagnostic capability of DWI and that of PET-CT for distinguishing mediastinal tumors and mass lesions. DWI is useful in distinguishing benign from malignant mediastinal tumors and mass lesions.