El Imad Talal, Haddad Fady G, Kesavan Mayurathan, Deeb Liliane, Andrawes Sherif
Department of Internal Medicine, Staten Island University Hospital.
Department of Gastroenterology, Staten Island University Hospital.
Cureus. 2017 May 16;9(5):e1252. doi: 10.7759/cureus.1252.
Solid-pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor that accounts for less than one percent of pancreatic tumors. The diagnosis could be challenging as SPN tend to manifest with nonspecific abdominal symptoms, variable radiological features, and inconsistent morphology. The cellular origin of SPN is unclear and might involve ductal, acinar and endocrine stem cells. We report a rare case of a 27-year-old female who presented with intermittent abdominal pain for two years, associated with a decrease in appetite. Her medical history was significant for abdominoplasty five years ago. Vital signs were stable. Physical examination revealed mild epigastric tenderness. Laboratory tests were unremarkable. Contrast computed tomography (CT) scan of the abdomen showed a 2 x 2 cm indeterminate pancreatic tail lesion. An endoscopic ultrasound (EUS) disclosed a 2.1 x 1.8 cm hypoechoic mass in the tail of the pancreas.Trans-gastric fine needle aspiration was obtained to show clusters of uniform neoplastic cells with abundant cytoplasm and oval bean-shaped nuclei. Immunohistochemical stains were positive for beta-catenin, Vimentin, CD10, CD56, cytokeratin-7 (Ck7), Cyclin D1, and negative for chromogranin, epithelial-cadherin (E cadherin) which was consistent with a pseudopapillary tumor. The patient underwent a robotic assisted en-bloc distal pancreatectomy and splenectomy. There were no intra-abdominal metastases. SPN is a rare tumor characterized by a specific immunohistological pattern which makes it highly distinct from other pancreatic neoplasms particularly neuroendocrine tumors, acinar carcinomas, and carcinoids. It is important to differentiate SPN from other pancreatic neoplasms because it is characterized as low potential for malignancy and a favorable prognosis after resection, with a five-year survival rate approaching 85%-95%.
胰腺实性假乳头状瘤(SPN)是一种罕见肿瘤,占胰腺肿瘤的比例不到1%。由于SPN往往表现为非特异性腹部症状、多样的影像学特征和不一致的形态,其诊断可能具有挑战性。SPN的细胞起源尚不清楚,可能涉及导管、腺泡和内分泌干细胞。我们报告一例罕见病例,一名27岁女性,间歇性腹痛两年,伴有食欲减退。她的病史中有五年前的腹壁成形术。生命体征稳定。体格检查发现上腹部轻度压痛。实验室检查无异常。腹部增强计算机断层扫描(CT)显示胰腺尾部有一个2×2cm的不确定病变。内镜超声(EUS)显示胰腺尾部有一个2.1×1.8cm的低回声肿块。经胃细针穿刺显示有成群的形态一致的肿瘤细胞,胞质丰富,核呈椭圆形豆状。免疫组化染色β-连环蛋白、波形蛋白、CD10、CD56、细胞角蛋白-7(Ck7)、细胞周期蛋白D1呈阳性,嗜铬粒蛋白、上皮钙黏蛋白(E钙黏蛋白)呈阴性,符合假乳头状瘤。患者接受了机器人辅助整块远端胰腺切除术和脾切除术。无腹腔内转移。SPN是一种罕见肿瘤,具有特定的免疫组织学模式,使其与其他胰腺肿瘤,特别是神经内分泌肿瘤、腺泡癌和类癌高度不同。将SPN与其他胰腺肿瘤区分开来很重要,因为它的特点是恶性潜能低,切除后预后良好,五年生存率接近85%-95%。