A giant, rapidly growing intra‑abdominal desmoid tumor of mesenteric origin in an adolescent male: A case report and literature review

Abstract

A desmoid tumor is a fibroblastic proliferation of mesenchymal origin, which has no metastasizing potential but is locally aggressive. Although treatment has shifted to observation and active surveillance for newly diagnosed patients with desmoid tumors, intra-abdominal mesenteric tumors or tumors that persistently grow and provoke symptoms may need prompt surgical treatment. There have only been a small number of case reports that illustrate large sporadic intra-abdominal mesentery-deriving desmoid tumors in which the longest diameter was ≥19 cm. In the present study, an adolescent male patient with a rapidly growing 38-cm long sporadic intra-abdominal desmoid tumor of mesenchymal origin is reported. The patient was treated with chemotherapy followed by surgical resection due to non-responsiveness and progression of symptoms, then with maintenance adjuvant chemotherapy to prevent recurrence due to the large size of the tumor. Despite the rapid growth of the tumor and its high occupancy in the intra-abdominal cavity, an R0 resection was successful with organ preservation. The patient has been recurrence-free for 2 years, and further follow-up is expected in the future.

Unraveling the Mystery of Desmoid Tumors: Insights From a Moroccan Tertiary Center

“…Its efficacy has been demonstrated in prospective studies and retrospective ones with similar results in pediatric patients [ 6 ]. However, TKIs such as sunitinib, imatinib, sorafenib, and pazopanib are thought to have the ability to act quickly, which is why they are employed, particularly in urgent cases with aggressive and rapid clinical presentations [ 20 , 21 ]. Sorafenib (400 mg daily) was assessed in a placebo-controlled randomized phase III trial, which showed a two-year PFS of 71% compared to only 36% for the placebo cohort [ 21 ].…”

“…However, TKIs such as sunitinib, imatinib, sorafenib, and pazopanib are thought to have the ability to act quickly, which is why they are employed, particularly in urgent cases with aggressive and rapid clinical presentations [ 20 , 21 ]. Sorafenib (400 mg daily) was assessed in a placebo-controlled randomized phase III trial, which showed a two-year PFS of 71% compared to only 36% for the placebo cohort [ 21 ]. In a phase II trial led by the French Sarcoma Group, Pazopanib was even found to be superior to the MTX-VBL combination in terms of objective response and one-year PFS [ 21 ].…”

“…Sorafenib (400 mg daily) was assessed in a placebo-controlled randomized phase III trial, which showed a two-year PFS of 71% compared to only 36% for the placebo cohort [ 21 ]. In a phase II trial led by the French Sarcoma Group, Pazopanib was even found to be superior to the MTX-VBL combination in terms of objective response and one-year PFS [ 21 ]. Nevertheless, Nirogacestat is the only treatment for progressive DT that the FDA has approved.…”

Contrast-enhanced ultrasonography combined with superb microvascular imaging for preoperative diagnosis of sporadic intra-abdominal desmoid-type fibromatosis: A case report

“…Intra-abdominal desmoid-type fibromatosis is usually diagnosed using abdominal computed tomography (CT) and/or abdominal magnetic resonance imaging (MRI) [ 6 , 7 ]. Transabdominal ultrasonography (US) and contrast-enhanced US are also useful for diagnosing intra-abdominal lesions [ [8] , [9] , [10] , [11] ].…”