CAJ | 학술논문

目的通过分析儿童胰母细胞瘤（PB ）的临床表现、病理特点及预后等因素，提高对PB的认识。方法回顾性分析近5年我院PB患儿的临床资料。结果 PB患儿共9例，男5例，女4例，平均年龄5岁（2个月～12岁）；8例因偶然发现腹部包块就诊，1例产前（32周）超声检查提示胰腺占位就诊；腹部超声（n＝6）及CT（n＝7）检查均提示胰腺占位；2例外周血白细胞升高，3例血清甲胎蛋白（AFP）升高；8例行肿瘤切除，1例取瘤组织活检；1例术后第2年和第3年分别发生肝转移；肿瘤累及胰头部6例，体尾部2例，整个胰腺1例；肿瘤最大直径3～18 cm，平均10 cm，结节状，切面灰白色，实性，细腻，部分区域呈囊性，可见明显的出血及坏死；镜下：肿瘤富含细胞，由形态一致的上皮细胞构成，瘤细胞呈实性片巢状排列，混有分化好的腺泡样结构，可见特征性“鳞状上皮小体”；免疫组织化学染色显示有腺泡、内分泌和导管分化的证据，表现为胰酶、内分泌标记和癌胚抗原（CEA）的阳性表达。结论胰母细胞瘤是儿童胰腺最常见的恶性肿瘤，镜下显示有腺泡、内分泌和导管的分化及特征性鳞状小体结构，确诊主要依据病理检查结果，应注意与胰腺其它肿瘤鉴别。首选的治疗方案是肿瘤的完整切除。预后较成人好。
목적통과분석인동이모세포류（PB ）적림상표현、병리특점급예후등인소，제고대PB적인식。방법회고성분석근5년아원PB환인적림상자료。결과 PB환인공9례，남5례，녀4례，평균년령5세（2개월～12세）；8례인우연발현복부포괴취진，1례산전（32주）초성검사제시이선점위취진；복부초성（n＝6）급CT（n＝7）검사균제시이선점위；2예외주혈백세포승고，3례혈청갑태단백（AFP）승고；8례행종류절제，1례취류조직활검；1례술후제2년화제3년분별발생간전이；종류루급이두부6례，체미부2례，정개이선1례；종류최대직경3～18 cm，평균10 cm，결절상，절면회백색，실성，세니，부분구역정낭성，가견명현적출혈급배사；경하：종류부함세포，유형태일치적상피세포구성，류세포정실성편소상배렬，혼유분화호적선포양결구，가견특정성“린상상피소체”；면역조직화학염색현시유선포、내분비화도관분화적증거，표현위이매、내분비표기화암배항원（CEA）적양성표체。결론이모세포류시인동이선최상견적악성종류，경하현시유선포、내분비화도관적분화급특정성린상소체결구，학진주요의거병리검사결과，응주의여이선기타종류감별。수선적치료방안시종류적완정절제。예후교성인호。
Objective To further learn about pancreatoblastoma (PB )by investigating its clinical situation,pathologic features,prognosis,etc.Methods A retrospective review of pediatric PB cases was undertaken within 5 years in our hospital.Results PB 9 cases,5 male ,4 female,age range was 2 months to 1 2 years,mean age 5.03 years old;Abdominal huge mass after a casual physical examination in 8 case, one case was found interior gestation 32 weeks .Imaging findings [CT(n=7)and US(n=6)]indicated a well-defined heterogeneous large mass in the pancreas;Elevated serum α-fetoprotein levels were noted in three cases and white blood cell of blood were noted in two cases.Complete surgical excision the masses was performed in 8 cases and biopsy was performed in 1 case;One case was developed metastases to liver after surgical operation second year and third year;The tumors arising from the head of pancreas in 6 cases and the body and tail of pancreas in 2 cases and entire pancreas in 1 case;The size was 3~1 8 cm in diameter and the mean was 1 0 cm;Most tumors were solitary,circumscribed and soft,the cut section was revealed grey,with cystic change,hemorrhage and necrosis prominent;Histologically,the tumors were composed of dense epithelial elements separated by fibro-stromal tissue,resulting or organoid pattern.Immunohistochemical,the tumors exhibited markers of the acinar,endocrine and ductal differentiation,such as enzyme of pancreas, endocrinic markers and CEA.Conclusions PB was the common malignant pancreatic neoplasm of childhood. The diagnosis of PB depends mainly on the pathologic findings.The PB should be included in the differential diagnosis of a mass in the pancreas.Complete resection is the treatment of choice.The prognosis is better than pancreatic ones in adult.