LOCALLY INVASIVE DESMOID TUMOR: CASE REPORT

Case Presentation DCP, male, 36 years old. The patient reported progressive right hypochondrial pain over the past 30 days. A computed tomography (CT) scan revealed an oval, voluminous mesenteric mass measuring approximately 10 cm, occupying the right hypochondrium and flank. The mass exhibited mixed density, was contiguous with the distal ileum, displaced the ascending colon at the hepatic flexure, and showed intimate relations with the second and third portions of the duodenum, the superior mesenteric artery, and the inferior vena cava, causing compression. Endoscopy and colonoscopy results were unremarkable. The patient underwent exploratory laparotomy, confirming findings consistent with the CT. Tumoral dissection involved the duodenal portions, pancreatic head, inferior mesenteric artery, and inferior mesenteric vein. A right colectomy was performed with the creation of a double-barrel stoma. Histopathological and immunohistochemical analysis confirmed a diagnosis of desmoid fibromatosis, with nuclear beta-catenin expression. The patient developed a cutaneous mucosal fistula, which resolved favorably. A subsequent intestinal transit reconstruction was performed without complications.

Discussion Desmoid tumors, also known as deep fibromatosis, are locally aggressive tumors originating from fibroblasts in musculoaponeurotic tissues. They are non-malignant, incapable of metastasis, but highly invasive locally, potentially compressing adjacent structures and causing organ dysfunction. The etiology is uncertain, and these tumors are rare, with an incidence of 2–4 cases per million people annually, accounting for about 0.03% of all neoplasms. They are more prevalent in adult women. Histologically, they consist of spindle cells forming spiral bundles, and 67–80% show immunoreactivity to beta-catenin. Clinical manifestations vary depending on the site of involvement. Due to their high recurrence rate and potential invasion of vital structures, management can be challenging. Imaging is critical for diagnosis, determining lesion size, and assessing anatomical relationships. Surgical intervention is the preferred therapeutic approach when feasible. Complications may include intestinal obstructions, fistula formation, hemorrhages, and intestinal perforations.

Conclusion This case is remarkable not only for the tumor's rarity but also for the patient's demographic profile and the extent of the lesion. Regular follow-up is essential given the high likelihood of recurrence. The management of such cases requires specialized diagnostic tools and a well-trained surgical team to ensure optimal treatment and care.

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Artikel online veröffentlicht:
25. April 2025