[Portal leiomyosarcoma: An extremely rare location!]

Area of Science:

• Gastroenterology
• Oncology
• Vascular Surgery

Background:

• Leiomyosarcomas are rare malignant tumors.
• Most large vessel leiomyosarcomas originate from the inferior vena cava.
• Portal vein leiomyosarcomas are exceptionally rare.

Observation:

• A 56-year-old female presented with chronic abdominal pain and a palpable mass.
• Imaging revealed a large mass in the duodenopancreatic region, extensively involving the portal vein.
• The mass caused portal cavernoma and secondary hepatic lesions, with pancreatic and duodenal origins excluded.

Findings:

• The pathological diagnosis of secondary hepatic lesions confirmed metastatic leiomyosarcoma.
• Imaging characteristics suggested a primary tumor of the portal vein.

Implications:

• This case highlights the importance of considering rare tumor locations in differential diagnoses.
• Early and accurate diagnosis of portal vein leiomyosarcoma is crucial for patient management.
• Further research into the etiology and treatment of vascular leiomyosarcomas is warranted.