Bhatnagar Ramneesh, Olson Matthew T, Fishman Elliot K, Hruban Ralph H, Lennon Anne M, Ali Syed Z
Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, Baltimore, Md., USA.
Acta Cytol. 2014;58(4):347-55. doi: 10.1159/000363546. Epub 2014 Jun 21.
Solid-pseudopapillary neoplasm (SPN) is a rare pancreatic malignancy with an excellent prognosis. It is most commonly diagnosed in young women. This article comprehensively reviews the clinical, pathological and radiological features of this neoplasm, as well as its clinical management.
A literature review of SPN was performed of all articles published in the English language in PubMed prior to November 1, 2013. Cytomorphological features, histopathology, immunohistochemistry, patient general demographics, molecular studies, radiologic imaging and clinical management were reviewed.
SPN displays distinct cytomorphological features on fine-needle aspiration - thin, delicate, branching vessels in a 'Chinese character' pattern lined by one to several layers of loosely cohesive neoplastic cells. Nuclear features include indented or grooved nuclei with an evenly distributed chromatin pattern and small inconspicuous nucleoli. SPN is characteristically immunoreactive for CD10, β-catenin (in an abnormal nuclear pattern), CD99 in a perinuclear dot-like pattern, α1-antitrypsin, and progesterone receptor. Almost all SPNs harbor an activating point mutation in exon 3 of the β-catenin gene (CTNNB1). Clinicopathological features generally do not correlate with prognosis, and most patients experience excellent long-term survival.
SPN can mimic other neoplasms of the pancreas, which can lead to diagnostic challenges in a limited cytologic specimen. Distinct cytomorphological features can help distinguish SPNs from other pancreatic neoplasms. Complete surgical resection as well as resection of metastatic disease is preferred given a low rate of tumor recurrence and long periods of disease-free intervals.
实性假乳头状肿瘤（SPN）是一种罕见的胰腺恶性肿瘤，预后良好。它最常见于年轻女性。本文全面综述了该肿瘤的临床、病理和放射学特征及其临床处理。
对2013年11月1日前在PubMed上发表的所有英文文章进行了关于SPN的文献综述。对细胞形态学特征、组织病理学、免疫组织化学、患者一般人口统计学、分子研究、放射影像学和临床处理进行了综述。
SPN在细针穿刺时表现出独特的细胞形态学特征——呈“汉字”样排列的细薄、精致、分支状血管，内衬一层至几层松散黏附的肿瘤细胞。核特征包括核呈凹陷或沟状，染色质分布均匀，核仁小且不明显。SPN的特征性免疫反应为CD10、β-连环蛋白（呈异常核模式）、呈核周点状模式的CD99、α1-抗胰蛋白酶和孕激素受体。几乎所有SPN在β-连环蛋白基因（CTNNB1）的外显子3中都存在激活点突变。临床病理特征一般与预后无关，大多数患者长期生存良好。
SPN可模仿胰腺的其他肿瘤，这可能在有限的细胞学标本中带来诊断挑战。独特的细胞形态学特征有助于将SPN与其他胰腺肿瘤区分开来。鉴于肿瘤复发率低且无病间期长，首选完整手术切除以及转移性疾病切除。
