CAJ | 학술논문

目的：探讨肝脏血管平滑肌脂肪瘤（HAML）的临床病理学特点、诊断及鉴别诊断。方法对15例肝脏血管平滑肌脂肪瘤的临床及病理学资料进行回顾性临床病理分析。结果15例HAML中女性13例，男性2例，男女比例1∶6. 5，年龄19～59岁（平均42. 2岁，中位年龄45岁）。6例患者有间断性腹部胀痛、恶心、反酸等不同的临床症状；1例合并慢性乙型病毒性肝炎，9例无明显症状。影像学检查均表现为肝脏内孤立性占位，其中6例考虑为血管瘤，6例为错构瘤，3例为肝癌。15例均行肿块切除术，12例行术中冰冻切片诊断，与石蜡切片诊断符合率97. 5％。肿瘤位于肝右叶11例（其中1例合并肾AML并肝脏复发，2例合并肝海绵状血管瘤，1例合并胃低分化腺癌），左叶2例，肝左右叶多发2例（其中1例合并肾AML，1例首发于右叶，2年后左叶发生并合并肝海绵状血管瘤）。肿瘤组织内可见不同比例的平滑肌、脂肪组织和血管成分，并可见多灶性髓外造血，瘤周肝组织无肝硬化改变。15例中经典型1例，血管瘤样型1例，脂肪瘤样型3例，平滑肌瘤样型10例（上皮样细胞型9例、梭形细胞型1例），其中上皮样平滑肌细胞可表现为多种形态：透明细胞型4例，嗜酸细胞型4例，多形细胞型1例。均有核异型性（15／15）：中度13例，重度2例；细胞密度：低密度3例，中密度7例，高密度5例。恶性HAML3例，其肿瘤组织与周围肝组织部分分界不清，呈浸润性生长。免疫组化标记HMB45、Melan‐A和SMA阳性，S‐100阴性。结论肝脏血管平滑肌脂肪瘤诊断困难，确诊主要依靠病理组织学改变及免疫组化染色；患者预后大多良好，术后需长期随访。
목적：탐토간장혈관평활기지방류（HAML）적림상병이학특점、진단급감별진단。방법대15례간장혈관평활기지방류적림상급병이학자료진행회고성림상병리분석。결과15례HAML중녀성13례，남성2례，남녀비례1∶6. 5，년령19～59세（평균42. 2세，중위년령45세）。6례환자유간단성복부창통、악심、반산등불동적림상증상；1례합병만성을형병독성간염，9례무명현증상。영상학검사균표현위간장내고립성점위，기중6례고필위혈관류，6례위착구류，3례위간암。15례균행종괴절제술，12례행술중빙동절편진단，여석사절편진단부합솔97. 5％。종류위우간우협11례（기중1례합병신AML병간장복발，2례합병간해면상혈관류，1례합병위저분화선암），좌협2례，간좌우협다발2례（기중1례합병신AML，1례수발우우협，2년후좌협발생병합병간해면상혈관류）。종류조직내가견불동비례적평활기、지방조직화혈관성분，병가견다조성수외조혈，류주간조직무간경화개변。15례중경전형1례，혈관류양형1례，지방류양형3례，평활기류양형10례（상피양세포형9례、사형세포형1례），기중상피양평활기세포가표현위다충형태：투명세포형4례，기산세포형4례，다형세포형1례。균유핵이형성（15／15）：중도13례，중도2례；세포밀도：저밀도3례，중밀도7례，고밀도5례。악성HAML3례，기종류조직여주위간조직부분분계불청，정침윤성생장。면역조화표기HMB45、Melan‐A화SMA양성，S‐100음성。결론간장혈관평활기지방류진단곤난，학진주요의고병리조직학개변급면역조화염색；환자예후대다량호，술후수장기수방。
Objective To investigate clinicopathological features ,diagnosis and differential diagnosis of hepatic agiomyoli‐poma(HAML).Methods The clinicopathological features were retrospectively analyzed in 15 cases of HAML.Results Of the 15 HALM patients ,there were 13 females and 2 males ,with the ratio of male to female being 1∶6.5 and the age ranging from 19 to 59 years(mean age:42.2 years ,median age:45 years).Six patients had intermittent abdominal pain ,nausea and sour regur‐gitation ,one case was combined with chronic viral hepatitis B ,and 9 cases had no obvious symptoms.Isolating mass was detec‐ted by liver imaging examination(hemangioma in 6 cases ,hamartoma in 6 cases ,hepatocellular carcinoma in 3 cases).Fifteen pa‐tients underwent oncotomy ,pathological examinations of frozen section were performed in 12 cases(diagnose accordance rate of frozen biopsy to patho‐wax scale was 97.5% ).In 11 cases ,the masses were in right lobe of liver (1 case combined with renal agiomyolipoma and recurrence in liver ,2 cases with hepatic cavernous hemangioma ,1 case with poorly differentiated adenocarci‐noma of stomach).In 2 cases ,the masses developed in the left lobe of liver ;2 cases had masses in the right and left lobe of liver meanwhile(1 case combined with renal agiomyolipoma ;in the other case ,the mass was originally found in right lobe of liver and 2 years later in left lobe of liver and associated with cavernous hemangioma of liver ).All tumors were composed of smooth mus‐cle ,adipose tissue and blood vessel ,and mulifocal medullary hematopoiesis could be observed.No cirrhosis was found around tumor tissue.The 15 HALM cases were classified into following subtypes :classical type(n=1) ,angiomatous type(n=1) ,lipo‐matous type(n=3) ,myomatous type(n=10;9 cases being epithelioid cell type ,1 case being spindle cell type).The epithelioid cell subtypes had a variety of forms :clear cell type(n=4) ,acidophilic cell type(n=4) ,polymorphic cell type(n=1).Moderate nuclear atypia was detected in 13 HAML cases and severe nuclear atypia in 2 HAML cases.Three HAML cases had low cell density ,7 medium density ,and 5 high density.HAML tissues of 3 cases were malignant ,in which the boundary between tumor tissues and liver tissues was indistinct ,and invasive growth was observed.Tumor cells were positive for HMB‐45 ,Melan‐A and SM A ,but negative for S‐100.Conclusion Hepatic angiomyolipoma diagnosis is difficult ,mainly relies on histomorphology and immunohistochemical staining ;prognosis is good ,and patients need long‐term follow‐up after surgery.