A giant sporadic intra-abdominal desmoid tumor resection: a rare case report

Summary

Introduction: Desmoid tumors (DT) are rare fibroblastic proliferations with unpredictable clinical behavior, occurring at a rate of 2 to 4 cases per million annually, predominantly in females. Prognosis is variable, with spontaneous regressions in 20 % to 30 % of cases, and shorter progression-free survival is associated with younger age, larger tumor size, and extra-abdominal location. Case presentation: A 30-year-old female with chronic constipation and abdominal pain was diagnosed with a giant desmoid tumor originating from the mesentery, requiring surgical resection of the tumor, the right colon, and part of the ileum. Postoperatively, she recovered well and was referred to an oncologist for ongoing monitoring due to the tumor's potential for recurrence. Discussion: Desmoid tumors, or aggressive fibromatosis, are rare benign mesenchymal tumors characterized by local invasiveness, primarily affecting individuals aged 15 to 60, with a slight female predominance. Diagnosis is confirmed through histological evaluation and immunohistochemistry. Treatment typically involves local control through surgery and radiation, but there is a significant risk of recurrence, influenced by tumor size, location, and patient age. Conclusion: This case highlights the diagnostic challenges of desmoid tumors in the mesentery, demonstrated by a 30-year-old female patient with chronic abdominal pain. The tumor, one of the largest documented, required multidisciplinary management and careful surgical planning due to its location near major intestinal vessels, emphasizing the need for prompt action and specific clinical considerations.