Sporadic pancreatic desmoid-type fibromatosis with curative resection: A case report.

Abstract

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.