Ozcan Alper, Arslanoglu Ceyda, Unal Ekrem, Patiroglu Turkan, Ozdemir Mehmet Akif, Deniz Kemal, Ozcan Serdal Sadet, Karakukcu Musa
Department of Pediatrics, Division of Pediatric Hematology and Oncology, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
North Clin Istanb. 2018 Sep;5(3):207-210. doi: 10.14744/nci.2017.27443.
Solid pseudopapillary tumor (SPT) of the pancreas is an extremely rare primary tumor in the pediatric age group. It has a low malignant potential and the prognosis is good if radical resection of the tumor is performed. Local recurrence and distant metastasis has only rarely been reported following incomplete resection.
A retrospective review of the medical records of 6 patients diagnosed as SPT according to a histopathological examination at the Children's Hospital of Erciyes University School of Medicine between 2010 and 2017 was performed. Demographic characteristics, tumor localization and size, diagnostic method, immunohistochemical staining features, and medical and surgical treatments employed were recorded.
There were 4 girls and 2 boys with the diagnosis of SPT included in this study. The mean age of the patients was 14 years (min-max: 13-16 years). The most common presenting complaint was abdominal pain. The mass lesion was at the head of the pancreas in 3 cases (50%) and the tail of the pancreas in the remaining 3 patients (50%). A Whipple procedure was performed in 3 cases, a distal pancreatectomy in 1, a distal pancreatectomy plus splenectomy in 1, and a subtotal pancreatectomy in 1patient. Immunohistochemistry revealed positive staining for beta-catenin, keratin, CD56, vimentin, and CD10 in all cases.
SPT is a rarely seen pancreatic mass with low rate of malignancy. Diagnosis may be delayed due to its asymptomatic nature in most cases and a lack of descriptive symptoms. The survival rate is quite high after radical resection.
胰腺实性假乳头状瘤（SPT）是儿童年龄组中极为罕见的原发性肿瘤。其恶性潜能低，若对肿瘤进行根治性切除，预后良好。不完全切除后局部复发和远处转移仅鲜有报道。
对2010年至2017年间在埃尔西耶斯大学医学院儿童医院经组织病理学检查确诊为SPT的6例患者的病历进行回顾性分析。记录人口统计学特征、肿瘤定位和大小、诊断方法、免疫组化染色特征以及所采用的内科和外科治疗方法。
本研究纳入了4例诊断为SPT的女孩和2例男孩。患者的平均年龄为14岁（最小-最大：13 - 16岁）。最常见的主诉是腹痛。肿块病变位于胰头3例（50%），其余3例（50%）位于胰尾。3例行惠普尔手术，1例行远端胰腺切除术，1例行远端胰腺切除术加脾切除术，1例患者行次全胰腺切除术。免疫组化显示所有病例中β-连环蛋白、角蛋白、CD56、波形蛋白和CD10均呈阳性染色。
SPT是一种罕见的胰腺肿块，恶性率低。由于大多数情况下无症状且缺乏特征性症状，诊断可能会延迟。根治性切除后生存率相当高。
