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Extraosseus Ewing's Sarcoma in Pancreas: A Review
Dharti Patel1, Nitish Singh Nandu2, Aravind Reddy2
1Internal Medicine, Oak Hill Hospital, Brooksville, USA.
Cureus
|May 7, 2020
Summary
Primitive neuroectodermal tumors (PNET) are rare, aggressive cancers. PNET of the pancreas, a type of Ewing
Area of Science:
- Oncology
- Pathology
Background:
- Primitive neuroectodermal tumors (PNET) are rare malignant neoplasms.
- PNETs are part of the Ewing's sarcoma family, characterized as round cell tumors.
- While typically arising in soft tissues, PNETs can occur in organs with neuroendocrine cells, including the pancreas.
Purpose of the Study:
- To review and consolidate reported cases of extraosseous Ewing's sarcoma (ES) of the pancreas.
- To highlight the aggressive nature and poor prognosis of pancreatic PNET.
- To emphasize the importance of considering PNET in the differential diagnosis of pancreatic tumors in young adults.
Main Methods:
- Literature review of 25 cases of extraosseous Ewing's sarcoma of the pancreas.
- Compilation of reported clinical data and outcomes.
Main Results:
- Pancreatic PNET is an aggressive tumor with a high recurrence rate and poor prognosis.
- Most cases occur in the second decade of life, with a male predominance.
- Surgery followed by chemoradiation are standard treatment modalities.
Conclusions:
- Primitive neuroectodermal tumors of the pancreas are rare but aggressive malignancies.
- Early consideration in differential diagnosis for young patients with pancreatic tumors is crucial.
- Despite aggressive treatment, prognosis remains poor, necessitating further research.
