Bülchmann G, Schuster T, Haas R J, Joppich I
Clinic for Pediatric Surgery, Universität München.
Klin Padiatr. 2000 Jul-Aug;212(4):185-8. doi: 10.1055/s-2000-9675.
Peripheral primitive neuroectodermal tumor (PPNET) is a malignant neoplasm of the peripheral nervous system and soft tissues. Representing the fourth case published we herein report a PPNET arising in the pancreas of a six year old girl. She presented with severe anemia due to ulcerative tumor growth and hemorrhage into the duodenum. From the first biopsy pancreatoblastoma was considered as histological diagnosis. Therefore pancreato-duodenectomy was successfully performed. Immunohistochemically, the tumor cells were positive for cytokeratines and several neuronal markers. Due to focal membranous staining for MIC-2 gene product and rosettes in one lymph node metastasis the diagnosis had to be altered into PPNET. This was confirmed by cytogenetic analysis. We conclude that the interpretation of histologic sample excisions from pediatric pancreatic neoplasms may be difficult and that PPNET should be included in the differential diagnosis.
外周原始神经外胚层肿瘤（PPNET）是一种发生于外周神经系统和软组织的恶性肿瘤。作为已发表的第四例病例，我们在此报告一例发生于一名6岁女童胰腺的PPNET。她因溃疡性肿瘤生长并向十二指肠内出血而出现严重贫血。首次活检时，组织学诊断考虑为胰腺母细胞瘤。因此成功实施了胰十二指肠切除术。免疫组化显示，肿瘤细胞细胞角蛋白及多种神经元标志物呈阳性。由于一个淋巴结转移灶中MIC-2基因产物呈局灶性膜染色且存在菊形团，诊断不得不改为PPNET。细胞遗传学分析证实了这一诊断。我们得出结论，小儿胰腺肿瘤组织样本切除的解读可能存在困难，PPNET应列入鉴别诊断。
