c Oliveira Bitar Silva
Santa Casa de São Paulo, SP, Brazil
Title: Pancreatic Peripheral Primitive Neuroectodermal Tumor diagnosed by Endoscopic Ultrasound
Biography
Biography: c Oliveira Bitar Silva
Abstract
A young girl presented with abdominal pain and jaundice of 1 month’s duration. She had conjugated hyperbilirubinemia and negative hepatitis serology. Computed tomography showed a mass in the head of the pancreas, with foci of calcification and cystic/necrotic areas. Pancreatoblastoma and Frantz Tumor were suspected. The patient underwent a cholecystojejunal anastomosis, and intraoperative biopsy of the pancreatic mass yielded inconclusive results. She was referred for endoscopic ultrasound (EUS) to re-evaluate the pancreatic mass. EUS showed a solid–cystic lesion in the head of the pancreas. EUS-guided fine-needle aspiration of the pancreatic mass was performed. Cytopathologic evaluation and immunohistochemical analysis confirmed the diagnosis of peripheral primitive neuroectodermal tumor (PNET).
PNET belongs to a rare group of tumors called the Ewing sarcoma family of tumors. Pancreatic PNETs are extremely rare and highly aggressive. Metastasis and recurrence are common. With modern multidisciplinary treatment, long-term survival can be achieved in 70% to 80 % of patients with disease that has not metastasized.
The correlation of clinical symptoms with imaging, cytopathologic, and immunohistochemical analysis is important to establish the diagnosis. An atypical rosette array of the cells, cytoplasmic neuronal secretory granules and neurofilaments, and pyknotic nuclear granules are important diagnostic criteria. Most tumors of the Ewing sarcoma family express high levels of a cell surface glycoprotein, CD99 [13, 14].
According to a 2014 review article, 14 cases of pancreatic PNET have been reported. This is the first case of a pancreatic PNET diagnosed by EUS.