• 제목/요약/키워드: Hepatocellular adenoma

검색결과 12건 처리시간 0.023초

당원병 제1형의 장기 추적 관찰; 간선종 발생의 위험 요인에 대한 연구 (Long-term Outcome of Glycogen Storage Disease Type 1; Analysis of Risk Factors for Hepatic Adenoma)

  • 양혜란;서정기
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제6권2호
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    • pp.129-139
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    • 2003
  • 목 적: 당원병 제1형은 어린 소아연령에서 발현하는 가장 심한 형태의 당원병이다. 장기간 관찰시 일부 환자에서 간선종이 발생하고 드물게 간암이 합병되어 예후에 심각한 영향을 미칠 수 있다고 알려져 있지만, 아직까지 이에 대한 연구가 거의 이루어지지 못하였다. 본 연구에서는 한국의 당원병 제1형 환자들의 추적관찰을 통해 간선종과 간암 발생의 측면에서 당원병 제1형의 장기적인 예후를 살펴보고자 하였다. 방 법: 1982년 3월에서 2003년 2월까지 서울대병원 소아과에서 간 조직 검사 및 효소검사를 통해 당원병 제1형으로 진단된 43명의 환자(제1a형 40명, 제1b형 3명)를 대상으로 하였다. 복부 초음파, 전산화단층촬영 또는 자기공명영상을 시행하여 간선종의 발생여부를 관찰하였으며, 간의 종괴가 확인된 경우 가능하면 조직검사를 시행하여 간선종 및 간암을 확진하였다. 간선종 발생에 관여하는 위험요인을 알아보기 위하여 간선종이 발생한 환자를 연령에 대해 짝짓기를 시행하여 선종군과 비선종군에서 여러 임상 지표를 비교하였다. 결 과: 1) 전체 43명 중 16명(37.2%)에서 간선종이 발생하였는데, 간선종이 처음 발견된 나이는 평균 $14.2{\pm}4.1$세(범위: 7.9~25.7세)였다. 처음 발견시 다발성 선종인 경우는 14례(87.5%)였다. 2) 간선종이 발생하였던 선종군(n=16)과 비선종군(n=16)을 비교하였을 때, 신장질환이 선종군에서는 7명(43.8%)에서 동반되고, 비선종군에서는 1명(6.3%)에서 병발하여 두 군 간에 유의한 차이를 보였다(p=0.037; 교차비 11.667, 95% 신뢰구간=1.227~110.953). 저신장은 선종군 6명(37.5%)과 비선종군의 10명(62.5%)에서 관찰되어 두 군 간에 유의한 차이가 없었다(p=0.157). 당원병 제1형 환자의 대부분에서 생옥수수 전분 치료를 시행하였는데, 생옥수수 전분 치료를 시작한 연령은 선종군이 $9.1{\pm}5.2$세인 반면, 비선종군은 $3.0{\pm}1.8$세로서 통계적으로 유의하게 선종군의 치료 시작 연령이 늦었다(p=0.003). 문맥하공정맥 문합술 시행 당시의 연령 차이가 없는 상태에서(p=0.170), 선종군의 11명(68.8%)과 비선종군의 3명(18.8%)에서 단락수술을 시행 받아 두 군간에 유의한 차이를 보였다(p=0.004; 교차비 9.533, 95% 신뢰구간=1.847~49.204). 고콜레스테롤혈증, 저혈당, 고요산혈증 등의 여부는 두 군 간에 유의한 차이를 보이지 않았다(p=0.833, p=0.273, p=0.550). 3) 25.7세에 간선종이 발견되었던 1명은 26.5세에 간암으로 진단되어 간동맥 항암 화학 색전술을 시행 받았다. 결 론: 간선종은 당원병 제1형 환자들을 장기간 추적관찰 하였을 때 비교적 흔하게 발생하는 합병증으로서, 일부에서 악성화 될 가능성이 있어 중요성이 매우 크다. 당원병 제1형 환자들의 장기적 예후를 위해서는 생옥수수 전분 치료의 조기 도입과 규칙적인 추적관찰을 통한 간선종의 조기 발견이 필요하다.

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Atypical β-Catenin Activated Child Hepatocellular Tumor

  • Turan, Aynur;Unlu, Havva Akmaz;Karakus, Esra;Erdem, Arzu Yazal;Yakut, Zeynep Ilerisoy
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제18권2호
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    • pp.144-148
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    • 2015
  • Hepatocellular adenomas are a benign, focal, hepatic neoplasm that have been divided into four subtypes according to the genetic and pathological features. The ${\beta}$-catenin activated subtype accounts for 10-15% of all hepatocellular adenomas and specific magnetic resonance imaging features have been defined for different hepatocellular adenomas subtypes. The current study aimed to report the magnetic resonance imaging features of a well differentiated hepatocellular carcinoma that developed on the basis of ${\beta}$-catenin activated hepatocellular adenomas in a child. In this case, atypical diffuse steatosis was determined in the lesion. In the literature, diffuse steatosis, which is defined as a feature of the hepatocyte nuclear factor-$1{\alpha}$-inactivated hepatocellular adenomas subtype, has not been previously reported in any ${\beta}$-catenin activated hepatocellular adenomas case. Interlacing magnetic resonance imaging findings between subtypes show that there are still many mysteries about this topic and larger studies are warranted.

Hepatic Angiomyolipoma Presenting as a Hyperintense Lesion During the Hepatobiliary Phase of Gadoxetic Acid Enhanced-MRI: a Case Report

  • Jeong, Boryeong;Kim, So Yeon;Kang, Hyo Jeong;Shin, Jinho
    • Investigative Magnetic Resonance Imaging
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    • 제26권1호
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    • pp.60-65
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    • 2022
  • Gadoxetic acid-enhanced magnetic resonance imaging (MRI) has been widely used to detect and characterize focal hepatic lesions. Because gadoxetic acid is a hepatocyte-specific contrast agent, its patterns during hepatobiliary phase enhancement provide useful information for differential diagnoses of focal hepatic lesions. Hepatic angiomyolipoma (AML) is a rare mesenchymal hepatic neoplasm composed of blood vessels, epithelioid cells, and varying amounts of adipose tissue components. Hepatic AMLs usually show marked hypointensity during the hepatobiliary phase of gadoxetic acid-enhanced MRI as hepatic AMLs are devoid of hepatocytes and fibrotic components. The present study describes a patient with hepatic AML and an atypical imaging feature. This tumor showed hyperintensity during the hepatobiliary phase of gadoxetic acid-enhanced MRI, mimicking hepatocellular tumors such as hepatocellular adenoma. The hepatobiliary hyperintensity of this lesion was likely due to multifocal entrapped hepatocytes resulting from an intrasinusoidal growth pattern of tumor cells and insufficient hepatic parenchymal enhancement during the hepatobiliary phase of gadoxetic acid-enhanced MRI.

C3H/He Mice as an Incompatible Cholangiocarcinoma Model by Clonorchis sinensis, Dicyclanil and N-Nitrosodimethylamine

  • Uddin, Md. Hafiz;Li, Shunyu;Jin, Yan;Choi, Min-Ho;Jang, Ja June;Hong, Sung-Tae
    • Parasites, Hosts and Diseases
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    • 제54권3호
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    • pp.281-289
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    • 2016
  • Clonorchis sinensis is a Group-I bio-carcinogen, associated with cholangiocarcinoma (CCA). The hamster is the only experimental model of C. sinensis-mediated CCA, but we oblige another animal model. The present study intended to develop a C. sinensis (Cs) mediated CCA model using C3H/He mice, co-stimulated with N-nitrosodimethylamine (NDMA) and dicyclanil (DC). The mice were divided into 8 groups with different combinations of Cs, NDMA, and DC. Six months later the mice were sacrificed and subjected to gross and histopathological examination. The body weights were significantly reduced among the groups treated with 2 or more agents (eg. Cs+NDMA, Cs+DC, NDMA+DC, and Cs+NDMA+DC). In contrast, liver weight percentages to body weight were increased in above groups by 4.1% to 4.7%. A Change of the spleen weight was observed only in Cs+NDMA group. Though C. sinensis infection is evident from hyperplastic changes, only 1 worm was recovered. Two mice, 1 from Cs and the other from Cs+DC group, showed mass forming lesions; 1 ($281.2mm^3$) from the Cs group was a hepatocellular adenoma and the other ($280.6mm^3$) from the Cs+DC group was a cystic mass (peliosis). Higher prevalence of gray-white nodules was observed in Cs group (42.9%) followed by Cs+NDMA+DC group (21.4%). The mice of the Cs+NDMA+DC group showed hyper-proliferation of the bile duct with fibrotic changes. No characteristic change for CCA was recognized in any of the groups. In conclusion, C3H/He mice produce no CCA but extensive fibrosis when they are challenged by Cs, NDMA, and DC together.

성인기에 진단된 당원병 제 Ia형의 다양한 임상 양상 (Heterogenous Clinical Manifestations in Adult Patients with Late Diagnosis of Glycogen Storage Disease type Ia)

  • 김유미;전종근;김구환;유한욱
    • 대한유전성대사질환학회지
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    • 제15권1호
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    • pp.9-17
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    • 2015
  • 당원병 Ia형은 glucose-6-phosphatase 효소의 결핍으로 인해 발생되는 상염색체열성 질환으로 특징적인 임상양상으로 대부분 영아기에 진단되나 증상의 경중에 따라 진단 연령이 늦어지기도 한다. 또한 환자 진단 시 유전 양식을 고려한 올바른 유전 상담과 더불어 형제, 자매에 대한 스크리닝이 중요하겠다. 본 연구는 성인기에 진단된 GSD Ia 자매에서의 임상양상의 차이를 기술하고 성인기 합병증에 대한 문헌을 고찰하여, 특히 간질환, 신부전 또는 대사성 질환으로 진료 받는 성인 환자들에서 당원병에 대한 감별과 당원병 진단 시 합병증에 대한 검사와 관리에 대해 필요성을 보고하는 바이다. 저혈당, 고지혈증, 고요산증, 젖산혈증, 대사성 산증, 기관 내 당원 축척에 대한 적절한 검사 및 약물 요법을 통해 급성 및 만성 합병증 예방과 적절한 치료를 위해 의료진의 체계적인 접근 및 노력이 필요하겠다.

Enhancing Effects of Indole-3-carbinol on Hepatocarcinogenesis and Thyroid Tumorigenesis in a Rat Multi-Organ Carcinogenesis Model

  • Kim, Dae-Joong;Han, Beom-Seok;Ahn, Byeong-Woo;Kim, Chang-Ok;Choi, Kwang-Sik;Lee, Joon-Sup
    • 한국응용약물학회:학술대회논문집
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    • 한국응용약물학회 1994년도 춘계학술대회 and 제3회 신약개발 연구발표회
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    • pp.339-339
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    • 1994
  • It has been reported that Indole-3-carbinol (I3C), a naturally occurring compound In cruclferous vegetables, exerts anticarcinogenic activity In several organs In rodents. The modifying effects of I3C were therefore assessed uging a rat multi-organ carcinogenesis model. A total of 100 male Sprague-Dawley rats were divided Into 3 groups. Animals of groups 1 and 2 were sequentially treated with diethylnitrosamine (DEN; 100 mg/kg b.w., i.p.), N-methylnitrosourea (NNU; 20 mg/kg b.w., 4 times for 2 weeks, i.p), and dihydroxy-di-N-propylnitrosauine (DHPN; 0.1% In d.w. for 2 weeks) for 4 weeks (DMD treatment). Animals of groups 1 and 3 were given the diet of 0.25% I3C for 20 weeks after DMD initiation and then were given basal diet for 28 weeks. All animals were sacrificed at week 24 and 52, respectively. I3C has been clearly demonstrated promoting effects on the development of glutathione S-transferase placental form (GST-P) positive hepatic foci at 24 weeks of the experiment. And I3C also exerted promoting potential In the hepatocellular adenoma (4/14; 29%) and the adenoma (7/14; 50%) of the thyroid gland at 52 weeks of the experiment. Therefore, I3C may promote hepatocarcinogenesis and thyroid tumorigenesis in the rat multi-organ carcinogenesis model.

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간 혈관종의 Tc-99m 표지 적혈구 혈액풀 스캔 (Evaluation of Hepatic Hemangioma by Tc-99m Red Blood Cell Hepatic Blood Pool Scan)

  • 손명희
    • 대한핵의학회지
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    • 제39권3호
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    • pp.151-162
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    • 2005
  • Hemangioma is the most common benign tumor of the liver, with a prevalence estimated as high as 7%. Tc-99m red blood cell (RBC) hepatic blood pool scan with single photon omission computed tomography (SPECT) imaging is extremely useful for the confirmation or exclusion of hepatic hemangiomas. The classic finding of absent or decreased perfusion and increased blood pooling ("perfusion/blood pool mismatch") is the key diagnostic element in the diagnosis of hemangiomas. The combination of early arterial flow and delayed blood pooling ("perfusion/blood pool match") is shown uncommonly. In giant hemangioma, filling with radioactivity appears first in the periphery, with progressive central fill-in on sequential RBC blood pool scan. However, the reverse filling pattern, which begins first in the center with progressive peripheral filling, is also rarely seen. Studies with false-positive blood pooling have been reported infrequently in nonhemangiomas, including hemangiosarcoma, hepatocellular carcinoma, hepatic adenoma, and metastatic carcinomas (adenocarcinoma of the colon, small cell carcinoma of the lung, neruroendocrine carcinoma). False-negative results have been also reported rarely except for small hemagniomas that are below the limits of spatial resolution of gamma camera.

간세포암종의 세침흡인 세포학적 소견 - 247예에 대한 연구 - (Fine Needle Aspiration Cytology of Hepatocellular Carcinoma - A Study on 247 Cases -)

  • 이광길;이종태;최수임;박찬일
    • 대한세포병리학회지
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    • 제1권1호
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    • pp.1-17
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    • 1990
  • Hepatocellular carcinoma (HCC) is malignant tumor frequently occurring in Koreans. There have been few reports regarding the cytologic findings of fine needle aspiration (FNA) of HCC. Most have suggested a diagnostic problem in the cytology distinguishing HCC from some benign hepatic lesions-for example, a regeneration nodule in cirrhosis and liver cell adenoma. In spite of its high frequency in Korea, no cytologic study has been reported, concerning the FNA of HCC. In an attempt to achieve cytologic criteria for the diagnosis of HCC, the authors studied retrospectively cytopathologic findings of 247 cases of HCC. These cases were confirmed either by histoiogic examination including lobectomy, biopsy, or ceil block material, or, when tissue diagnosis was unavailable, by a high serum alpha-fetoprotein level (over 400 I. U.). All aspiration smears were stained by the Papanicolaou method. In each case, the smears were analyzed for cell patterns and various cytomorphology of the tumor cells. The smear background was assessed for the presence of tumor cell necrosis and inflammatory components and compared to that of metastatic carcinomas. The cell patterns were classified as trabecular, acinar, dispersed, and irregular. The cytologic parameters analyzed included the degree of nuclear atypia and the presence of mitoses, intranuclear cytoplasmic inclusions, nucleolar prominency, endothelial lining, multinucleated giant cells, eosinophiic globules, bile, and Mallory body. Most of the FNA of HCC showed markedly cellular smears. The tumor cells were most frequently arranged in a trabecular pattern (80.3%). The irregular (12.6%), the acinar (5.5%), and the dispersed patterns (1.7%) followed in decreasing frequency. Individual hepatoma cells were larger than normal liver cells. However, they had morphologic features characteristic of the hepatic cells the cells were round or polygonal, their cytoplasm was abundant and granular with eosinophilic or amphophilic stainability, and their nuclei were round to oval, located centrally, and tended to have prominent nucleoli. Anaplasia and pleomorphism of tumor cells were generally mild to moderate. These findings existed even in very well differentiated cases. Mitotic figures were present in about 85% of the cases. Prominent nucleoli were observed only in about half the cases. The frequency of other cytologic features was as follows intranuclear cytoplasmic inclusion in 86.8% : endothelial lining in 56.1% : bile in 19.8% : and giant cells in 60.1%. Clear cells were often present in 11.7%, Most aspiration smears of HCC displayed clean background without necrosis or inflammatory material in contrast to the dirty, necrotic background of metastatic cancers and cholangiocarcinomas. Based on the above mentioned features, it is suqqested that the cytologic critieria most important for the diagnosis of HCC include a markedly cellular smear, trabecular pattern, hepatocytoid appearance of tumor cells, endothelial lining, the presence of bile, giant cells, intranuclear cytoplasmic inclusions, and prominent nucleoli, Among these, trabecular pattern, endothelial lining, giant cells and clean smear background are points to be considered in differentiating HCC from metastatic and cholangiocellular carcinoma.

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신경내분비종양 진단에 있어서 I-123 MIBG 조기 평면 영상과 SPECT 영상의 임상적 유용성 (Clinical Usefulness of I-123 MIBG Scintigraphy with Early Planar and SPECT Image in The Diagnosis of Neuroendocrine Tumors)

  • 신중우;류진숙;원경숙;최윤영;김희중;양승오;이희경;서용섭
    • 대한핵의학회지
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    • 제30권4호
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    • pp.516-523
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    • 1996
  • 목 적 : 신경내분비종양 진단에 대한 I-123 MIBG 스캔의 4시간 조기 영상과 SPECT 영상의 임상적 유용성을 평가하고자 시행하였다. 대상과 방법 : CT 또는 MRI를 시행하여 신경내분비종양이 의심되었던 21예를 대상으로 I-123 MIBG 스캔을 시행하였으며 4시간에 전신 평면 영상과 SPECT 영상을 얻고, 17예에서는 12-24시간 사이에 지연 평면 영상도 얻었다. 결 과 : 21예중 12예에서 신경내분비종양으로 확진되었고, 4시간 I-123 MIBG 스캔의 진단적 예민도는 75%, 특이도는 89%였다. SPECT 영상은 CT나 MRI와의 비교 분석에 용이하였다. 지연 영상은 일부 진양성을 소견을 보인 예에서 종양 대 배후 방사능의 비가 증가하여 병변 확인에 도움이 되었으나 4시간 평면 영상과 진단율에 차이는 없었다. 결 론 : I-123 MIBG 스캔은 4시간 조기 영상으로도 신경내분비종양의 진단에 유용했으며, SPECT 영상은 다른 진단매체와의 비교 분석이 용이하였고 향후 종양의 종류에 따라 더 많은 예의 연구가 필요할 것으로 생각하였다.

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