Retroperitoneal desmoid-type fibromatosis: a case report

Area of Science:

• Oncology
• Surgical Pathology
• Radiology

Background:

• Desmoid-type fibromatosis (DF) is a rare soft tissue sarcoma, typically found in the anterior abdominal wall.
• Retroperitoneal DF is exceptionally rare, often associated with familial syndromes, making sporadic cases noteworthy.
• Reporting experiences with retroperitoneal DF is crucial for understanding oncological outcomes and management strategies.

Observation:

• Two patients presented with sporadic retroperitoneal DF, a rare clinical presentation.
• One patient experienced urinary obstruction due to tumor extension into the kidney, requiring surgical resection.
• The second patient, with a history of recurrent DF, was incidentally diagnosed with retroperitoneal DF, which recurred after initial treatment.

Findings:

• Both cases necessitated surgical tumor resection due to symptomatic presentation and as a curative measure.
• Recurrence of DF significantly impacts patient quality of life, as observed in one case.
• Histopathological and radiological features of these rare retroperitoneal DF cases are detailed.

Implications:

• These cases contribute to the limited literature on retroperitoneal DF, particularly sporadic forms.
• Findings may inform the development of practice-changing recommendations and guidelines for this rare condition.
• Further research is warranted to optimize management and improve outcomes for patients with retroperitoneal DF.