CAJ | 학술논문

目的：探讨胰腺实性-假乳头状肿瘤( solid-pseudopapillary tumor, SPPT)的临床病理特点、诊断及组织起源。方法复习相关文献,并回顾性分析11例SPPT的临床资料、病理组织学及免疫表型特征。结果11例SPPT中女性10例,男性1例,年龄17~60岁,平均33岁。肿瘤切面均为囊实性,有包膜,直径3.2~10.0 cm。镜下肿瘤由乳头区和囊实区混合组成,瘤细胞围绕纤维血管轴心形成特征性假乳头结构。免疫组化标记表达EMA (1/11)、vimentin (10/11)、NSE (11/11)、Syn (7/11)、CgA(1/11)、CD56(11/11)、CD10(11/11)、PR(9/11)、CD99(9/11)、α-AT(11/11)、β-catenin(11/11)、E-cadherin(11/11)、Cyc-lin D1(11/11)、c-Myc(11/11)。6例获得随访,随访时间20~112个月,均无肿瘤复发及转移,并存活至今。结论 SPPT是一种好发于年轻女性,具有低度恶性潜能的少见胰腺肿瘤,可能来源于多能干细胞,其发生与β-catenin通路关系密切；病理形态特征和免疫组化标记对SPPT的诊断和鉴别诊断具有重要价值。
목적：탐토이선실성-가유두상종류( solid-pseudopapillary tumor, SPPT)적림상병리특점、진단급조직기원。방법복습상관문헌,병회고성분석11례SPPT적림상자료、병리조직학급면역표형특정。결과11례SPPT중녀성10례,남성1례,년령17~60세,평균33세。종류절면균위낭실성,유포막,직경3.2~10.0 cm。경하종류유유두구화낭실구혼합조성,류세포위요섬유혈관축심형성특정성가유두결구。면역조화표기표체EMA (1/11)、vimentin (10/11)、NSE (11/11)、Syn (7/11)、CgA(1/11)、CD56(11/11)、CD10(11/11)、PR(9/11)、CD99(9/11)、α-AT(11/11)、β-catenin(11/11)、E-cadherin(11/11)、Cyc-lin D1(11/11)、c-Myc(11/11)。6례획득수방,수방시간20~112개월,균무종류복발급전이,병존활지금。결론 SPPT시일충호발우년경녀성,구유저도악성잠능적소견이선종류,가능래원우다능간세포,기발생여β-catenin통로관계밀절；병리형태특정화면역조화표기대SPPT적진단화감별진단구유중요개치。
Purpose To investigate the clinicopathlogic characteristic, diagnosis and histogenesis of pancreatic solid-pseudopapillary tumors ( SPPT) . Methods Combined with relevant literature, the clinical history, histopathological features and immunohistochemi-cal characteristics were analyzed in 11 cases of SPPT. Results There were 10 female patients and only 1 male in total 11 cases, aged from1 7 to 60 years (mean 33). The sizes of tumors were from 3. 2 to 10. 0 cm. Histologically, they were composed of papillary and microcysticsolid structures. Pseudopapillary with a fibrovascular core was remarkable. Immunohistochemically, the tumors expressed EMA (1/11), vimentin (10/11), NSE (11/11), Syn (7/11), CgA (1/11), CD56 (11/11), CD10 (11/11), PR (9/11), CD99 (9/11),α-AT (11/11),β-catenin (11/11), E-cadherin (11/11), Cyclin D1 (11/11), c-Myc (11/11). 6 patients were followed up for a period of 20 to 112 months, and they were all alive and had no recurrence and metastasis. Conclusions SPPT is a tumor with low malignancy of the pancreas that most frequently affect young females. SPPT may be derived from multipotent stem cells and closely related withβ-catenin signaling pathway. Pathological morphology and immunohistochemistry are very important to the diag-nosis and differential diagnosis of SPPT.