Huge Primary Intermediate-grade Myofibroblastic Sarcoma in the Retroperitoneum

Area of Science:

• Oncology
• Surgical Pathology
• Soft Tissue Tumors

Background:

• Myofibroblastic sarcoma is a rare malignancy, predominantly low-grade, with infrequent retroperitoneal involvement.
• The 2020 World Health Organization classification lacks consensus on high- and intermediate-grade myofibroblastic sarcoma definitions, unlike the 2013 version.
• Intermediate- and high-grade myofibroblastic sarcomas are associated with poor survival outcomes.

Observation:

• A case of intermediate-grade myofibroblastic sarcoma located in the retroperitoneum is presented.
• The patient underwent an en bloc surgical resection and ureteroplasty.
• No adjuvant therapy was administered post-operatively.

Findings:

• The patient remained asymptomatic and pain-free for 19 months following the surgical intervention.
• This case demonstrates a successful outcome following surgical management of intermediate-grade retroperitoneal myofibroblastic sarcoma.
• The findings suggest that surgical resection can be an effective treatment modality.

Implications:

• This case highlights the importance of surgical resection as a primary treatment for retroperitoneal myofibroblastic sarcoma.
• Further research is needed to clarify the classification and grading of myofibroblastic sarcomas.
• Successful surgical management without adjuvant therapy offers a potential treatment pathway for similar rare cases.