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Primitive neuroectodermal tumor of the pancreas
Ted George Achufusi1, Raman Sohal1, Ernesto Zamora1
1Department of Internal Medicine, State University of New York Upstate Medical University, Syracuse, New York.
Proceedings (Baylor University. Medical Center)
|January 18, 2021
Summary
Primitive neuroectodermal tumors (PNETs) are rare cancers. This case highlights an extremely rare instance of pancreatic PNET in an adult, emphasizing diagnostic challenges and poor prognosis.
Area of Science:
- Oncology
- Pathology
Background:
- Primitive neuroectodermal tumors (PNETs) are rare small round cell malignancies, often associated with Ewing sarcoma.
- PNETs predominantly affect pediatric and young adult populations.
Observation:
- Pancreatic involvement by PNETs is exceptionally rare.
- This report details a case of pancreatic PNET in a 61-year-old male presenting with abdominal pain and weight loss.
Findings:
- Clinical presentation of PNETs is often vague with nonspecific imaging findings.
- Diagnosis requires confirmation through histology, immunohistochemistry (CD99), and genetic testing (t(11;22) translocation).
Implications:
- Pancreatic PNETs in adults are associated with a poor prognosis and lack of standard treatment.
- This case underscores the importance of considering rare diagnoses in adult oncology.
