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SURVIVAL IN PANCREATIC EWING'S SARCOMA FAMILY OF TUMORS IMPROVED WITH IFOSFAMIDE AND ETOPOSIDE REGIMENS: A SYSTEMATIC REVIEW AND SURVIVAL ANALYSIS
Kaiser O. Sadiq
*1, RIshab Belavadi
2, Swetha Lakshminarayanan
1, Patricia Ruiz Cota
11UC San Diego Health, San Diego, CA; 2Mayo Clinic Arizona, Scottsdale, AZ
BACKGROUND
Formerly classified as Ewing's sarcomas and primitive neuroectodermal tumors, the Ewing's Sarcoma Family of Tumors (ESFT) includes undifferentiated small round cell sarcomas of bone and soft tissue that share common cytogenetic and histological characteristics. These malignancies are rare in visceral organs like the pancreas, leading to limited data on their management and prognosis. This review examines the clinicopathological features, treatments, and survival outcomes in pancreatic ESFT.
METHODS
This systematic review and meta-analysis was registered on PROSPERO (CRD42024585711) and followed PRISMA guidelines. The Cochrane Library, Embase, PubMed, and Pubmed Central were last searched in Aug 2024 without time or language restrictions and included all studies with data that could be pooled for analysis. R 4.4.1 was used to calculate inferential statistics.
RESULTS
Out of 812 studies retrieved, 71 were included, totaling 89 unique patients. Ages ranged from 2 to 78 (median, 23) with 55.1% male and 43.8% female (Fig 1A). Most common clinical features (Fig 1B) were abdominal pain (68.8%) and jaundice (26.0%). Affected sites were the head (57.1%), uncinate process (10.4%), body (39.0%), and tail (31.2%) (Fig 2). Maximum diameter ranged from 1.8 to 33.0 cm (median, 7.75 cm). 11 were secondary tumors, 25 were necrotic, calcification was seen in 1, local invasion in 26, and 19 were metastatic. 80.1% were treated with chemotherapy; 41.4% adjuvant and 27.6% neoadjuvant. Regimens included VDC (Vincristine, doxorubicin, and cyclophosphamide) in 54.3%, IE (Ifosfamide and Etoposide) in 39.1%, and other agents in 21.7%. For local control, surgery (76.4%), radiation (1.4%), or a combination (8.3%) were used. On follow-up, disease progressed in two cases, recurred in two, and metastasized in six. Survival outcomes were unavailable for 18 cases, mortality was 38.0% for the remaining and 46.5% had no evidence of disease. Maximum follow up duration was 120 months (median, 12 months). Univariate analysis of Cox regression models (Fig 1C and D) of overall survival (OS) revealed a hazard ratio (HR) of 0.24 (
p<0.01) for primary tumors and 4.75 (
p<0.01) for metastases. HR for disease-free survival (DFS) was 0.28 (
p<0.01) for primary tumors, 4.40 (
p<0.01) for metastases, 0.40 (
p<0.05) for those treated with chemotherapy, and 0.21 (
p<0.05) for chemotherapy regimens including IE. Age, tumor size, necrosis, local invasion, surgery, and radiotherapy did not affect OS or DFS significantly.
CONCLUSION
Pancreatic ESFT is rare, mainly affecting those under 40, with slight male predominance. The head of the pancreas is most frequently involved, and abdominal pain is the most common symptom. DFS and OS are worse with metastases and secondary pancreatic involvement. Surgery and chemotherapy are common treatments, with chemotherapy and IE regimens showing improved DFS.
Fig 1. Graphical representation of (A) demographics, (B) clinical features, (C) overall survival, and (D) disease-free survival of Ewing's Sarcoma Family of Tumors involving the pancreas.
Fig 2. Distribution of anatomic involvement of pancreatic Ewing's sarcoma family of tumors.
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