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증 례 : 분지형 점액분비성낭종 환자에서 발생한 침윤성 췌장암의 심각한 임상 경과: 증례 3예
배대환 ( Dae Hwan Bae ),양지열 ( Ji Youl Yang ),김근모 ( Keun Mo Kim ),김형우 ( Hyung Woo Kim ),윤웅수 ( Woong Su Yoon ),조병하 ( Byung Ha Cho ),김진영 ( Jin Young Kim ),한정호 ( Joung Ho Han ),박선미 ( Seon Mee Park ) 대한췌담도학회 2015 대한췌담도학회지 Vol.20 No.4
Branch duct intraductal papillary mucinous neoplasms of the pancreas (BD-IPMN) without malignant features rarely developed into invasive cancer. However, invasive cancer is aggressive once an invasive change occurs. We report three cases of invasive cancers which developed in patients with BD-IPMN and they showed grave clinical courses. All patients were diagnosed with BD-IPMN < 3 cm without malignant features on imaging. Invasive cancer was detected at 2.5 years, 3.0 years, and 4.0 years after BD-IPMN detection in each patient. The intervals of invasive cancer and the last follow-up were 9 months, 3 years, and 1.5 years in the three patients, respectively. All patients were diagnosed with locally advanced pancreas invasive cancers and were treated with palliative chemotherapy or conservative management. The patients died at 3 months, 9 months, and 10 months after the diagnosis of invasive cancers, respectively. We report three cases of invasive cancer developed in BD-IPMN patients and followed fatal courses.
재발성 급성 담관염을 보인 담도 과오종과 선천성 간섬유증을 동반한 1예
김기배 ( Ki Bae Kim ),지명진 ( Myoung Jin Ji ),양지열 ( Ji Youl Yang ),한정호 ( Joung Ho Han ),채희복 ( Hee Bok Chae ),박선미 ( Seon Mee Park ),강민호 ( Min Ho Kang ),성노현 ( Ro Hyun Sung ),윤세진 ( Sei Jin Youn ) 대한췌담도학회 2015 대한췌담도학회지 Vol.20 No.2
담도 과오종과 선천성 간섬유증은 태생기에 간판 기형에 의해 생기는 섬유 낭성 질환에 속한다. 담도 과오종은 증상을 유발하지 않고 우연히 발견되는 질환이지만, 선천성 간섬유증은 25%에서 담관염이 발생하고 증상이 발생하면 항생제 투여부터 수술까지 적극적 치료가 필요하다. 저자들은 복부 영상 소견으로 담도 과오종으로 진단하고 경과 관찰하던 64세 남자가 2년간 반복적인 담관염이 발생한 원인을 찾기 위해 시행한 간 생검에서 담도 과오종과 선천성 간섬유증이 병합된 것으로 진단한 증례를 경험하여 문헌고찰과 함께 보고한다. Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review. Korean J Pancreatobiliary 2015;20(2):105-110