Related Experiment Video
Updated: Jun 25, 2026
05:42
Robotic Enucleation of an Intra-Pancreatic Insulinoma in the Pancreatic Head
Published on: January 3, 2020
Primitive neuroectodermal tumor of the pancreas.
Hirokazu Doi1, Soichi Ichikawa, Atsushi Hiraoka
1Department of Gastroenterology, Ehime Prefectural Central Hospital, Matsuyama.
Internal Medicine (Tokyo, Japan)
|March 3, 2009
Summary
Primitive neuroectodermal tumor (PNET) of the pancreas is rare. This case highlights surgical resection and chemotherapy for a pancreatic PNET with EWSR1 rearrangement, a type of Ewing sarcoma.
Area of Science:
- Oncology
- Pathology
Background:
- Primitive neuroectodermal tumors (PNETs) are rare, belonging to the Ewing sarcoma family of tumors.
- Pancreatic PNETs are exceptionally uncommon, posing diagnostic and therapeutic challenges.
Observation:
- A 37-year-old male presented with a solitary 40 mm pancreatic tumor and multiple hepatic lesions.
- Immunohistochemical staining confirmed CD99 positivity, and FISH analysis revealed an EWSR1 22q12 rearrangement.
Findings:
- The patient underwent surgical resection followed by chemotherapy according to the Japan-Ewing protocol.
- This case adds to the limited reported instances of pancreatic PNET, with a mean patient age of 19.3 years.
Implications:
- This case underscores the importance of molecular diagnostics, such as EWSR1 rearrangement detection, in diagnosing rare pancreatic tumors.
- Optimal management likely involves a multimodal approach including surgery and chemotherapy for pancreatic PNET.