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Sporadic pancreatic desmoid-type fibromatosis with curative resection: A case report
Jiayao Zhang1,2, Chenming Liu2, Fangzheng Zhou1,3
1Department of Hepatobiliary and Pancreatic Surgery, Shaoxing People's Hospital, Shaoxing, China.
Rationale:
Desmoid-type fibromatosis (DF) is an uncommon, locally invasive, non-metastatic soft-tissue neoplasm with variable and unpredictable manifestations. The therapeutic arsenal of DF therapy is consistently expanding; however, there remains no standard treatment modality. Sporadic pancreatic DF is rarely described in current literature, reflecting a significant deficiency in clinical treatment experience, this case aims to share some clinical experiences that can serve as a reference for managing this rare disease.
Patient Concerns:
A 36-year-old male presented with occasional abdominal discomfort and weight loss over a year. Ultrasound revealed a large mass in the pancreatic tail, which was not observed a year ago.
Diagnoses:
The diagnosis of DF was confirmed by immunohistochemistry nuclear staining of β-catenin.
Interventions:
Distal pancreatectomy with splenectomy was performed and the patient received no further therapy.
Outcomes:
After 13 months of follow-up, no recurrence or distant metastasis was observed.
Lessons:
DF is a distinct rare tumor entity, sporadic pancreatic DF is even rarer. It is imperative to select the individualized treatment strategy for each patient to optimize tumor control and enhance quality of life.
Insights
This case study highlights a rare instance of desmoid-type fibromatosis (DF) in the pancreas. Surgical resection led to a positive outcome with no recurrence after 13 months, offering insights for managing this uncommon tumor.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Desmoid-type fibromatosis (DF) is a rare, locally invasive soft-tissue neoplasm lacking standardized treatment.
- Pancreatic DF is exceptionally uncommon, with limited clinical data and treatment experience.
- This report details a case of sporadic pancreatic DF to inform clinical management.
Purpose of the Study:
- To present a clinical case of sporadic pancreatic desmoid-type fibromatosis.
- To share management experiences for this rare pancreatic tumor.
- To contribute to the limited literature on pancreatic DF.
Main Methods:
- A 36-year-old male presented with abdominal discomfort and weight loss.
- Imaging revealed a pancreatic tail mass; diagnosis confirmed via β-catenin immunohistochemistry.
- Treatment involved distal pancreatectomy with splenectomy, followed by no adjuvant therapy.
Main Results:
- The patient underwent successful surgical resection of the pancreatic mass.
- Post-operative follow-up at 13 months showed no evidence of tumor recurrence or metastasis.
- The intervention was effective in controlling the disease in this case.
Conclusions:
- Desmoid-type fibromatosis is a rare tumor, with pancreatic involvement being exceedingly rare.
- Individualized treatment strategies are crucial for optimizing outcomes in DF patients.
- This case provides valuable reference for managing sporadic pancreatic DF.
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