Wang Brant G, Mani Haresh, Wang Zoe Q, Li Wenping
Department of Pathology, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA.
Diagnostics (Basel). 2022 Feb 9;12(2):449. doi: 10.3390/diagnostics12020449.
Pancreatic solid-pseudopapillary neoplasm (SPN) is a rare tumor that typically occurs in young females. Although a cytological diagnosis may be easily made in this age group when there are typical features, atypical clinical presentations and unusual cytological features may make this a challenging diagnosis. We present our single-institution experience in a cohort of these tumors, outlining both typical and atypical features. Awareness of unusual clinical and cytological features can help to avoid pitfalls during diagnosis.
We performed a review of all cases of pancreatic SPNs diagnosed over a 15-year period (January 2007 to December 2021). Detailed cytological, clinical, and follow-up histological features were presented and analyzed.
Twenty-two cases of SPN were diagnosed at our institution during this 15-year period. Patients ranged from 12 to 73 years of age (mean 33 y, median 26 y) and included 19 females and 3 males. Seventeen patients had cytological material, and fourteen were diagnosed by EUS-FNA. Typical cytological features included papillary clusters with central capillaries, myxoid stroma, monomorphism, cercariform cells, and hyaline globules. Atypical or unusual cytological features that were seen in a few cases were multinucleated giant cells, clear cells, and/or foamy macrophages. A few cases showed features that were similar to pancreatic neuroendocrine tumors (PanNETs). Tumor cells were always positive for β-catenin, CD10, CD56, cyclin-D1, progesterone receptor (PR), and vimentin by immunohistochemistry. They were always negative for chromogranin. Pancytokeratin and synaptophysin stains were positive in 9% and 46% of cases evaluated, respectively. All cases had histological confirmation on resection. The median follow-up duration was 69 months (a range of 2-177 months), with only three cases lost to follow-up. No recurrence or metastasis was identified.
We present our experience with cytological diagnoses of SPN in a well-characterized cohort of 22 patients with histological correlation and follow-up data. These tumors occur over a wide range and show varied cytological features. SPNs can be confidently diagnosed on limited cytological material, with limited panel immunohistochemistry aiding diagnosis in atypical cases. Recognizing the associated degenerative changes is crucial in avoiding a misdiagnosis.
胰腺实性假乳头状肿瘤（SPN）是一种罕见肿瘤，通常发生于年轻女性。尽管在这个年龄组中，当出现典型特征时细胞学诊断可能很容易做出，但非典型的临床表现和不寻常的细胞学特征可能使诊断具有挑战性。我们介绍了我们在一组此类肿瘤中的单机构经验，概述了典型和非典型特征。认识到不寻常的临床和细胞学特征有助于避免诊断过程中的陷阱。
我们回顾了15年期间（2007年1月至2021年12月）诊断的所有胰腺SPN病例。呈现并分析了详细的细胞学、临床和随访组织学特征。
在这15年期间，我们机构共诊断出22例SPN。患者年龄在12至73岁之间（平均33岁，中位数26岁），其中包括19名女性和3名男性。17例患者有细胞学材料，14例通过超声内镜引导下细针穿刺活检（EUS-FNA）确诊。典型的细胞学特征包括带有中央毛细血管的乳头簇、黏液样间质、单形性、尾蚴样细胞和透明小球。少数病例中出现的非典型或不寻常的细胞学特征有多核巨细胞、透明细胞和/或泡沫巨噬细胞。少数病例表现出与胰腺神经内分泌肿瘤（PanNETs）相似的特征。通过免疫组织化学检测，肿瘤细胞β-连环蛋白、CD10、CD56、细胞周期蛋白D1、孕激素受体（PR）和波形蛋白始终呈阳性。嗜铬粒蛋白始终呈阴性。在评估的病例中，全细胞角蛋白和突触素染色阳性率分别为9%和46%。所有病例均经手术切除组织学证实。中位随访时间为69个月（范围为2至177个月），仅有3例失访。未发现复发或转移。
我们介绍了在22例具有组织学相关性和随访数据的特征明确的队列中对SPN进行细胞学诊断的经验。这些肿瘤发病年龄范围广，细胞学特征多样。基于有限的细胞学材料可以可靠地诊断SPN，有限的免疫组化检测有助于非典型病例的诊断。认识到相关的退行性改变对于避免误诊至关重要。
