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

Experimental and therapeutic medicine Pub Date : 2023-09-05 eCollection Date: 2023-10-01 DOI:10.3892/etm.2023.12189
Sun Jung Kim, Jung Woo Han, Taehan Yoon, Hyungwook Choi, Yoon Dae Han
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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.

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一例青少年男性肠系膜来源的巨大、快速生长的腹部硬纤维瘤:病例报告和文献综述。
结缔组织样肿瘤是间充质来源的成纤维细胞增殖,没有转移潜力,但具有局部侵袭性。尽管治疗已转向观察和积极监测新诊断的硬纤维瘤患者,但腹腔内肠系膜肿瘤或持续生长并引发症状的肿瘤可能需要及时手术治疗。只有少数病例报告显示,最长直径≥19厘米的大型散发性腹腔间充质系膜内硬纤维瘤。在本研究中,报告了一名患有快速生长的38厘米长的散发性腹腔内间充质硬纤维瘤的青少年男性患者。由于无反应性和症状进展,患者接受了化疗,然后进行了手术切除,然后进行维持辅助化疗,以防止因肿瘤体积大而复发。尽管肿瘤生长迅速,在腹腔内占有率很高,但R0切除术在器官保存方面取得了成功。该患者已2年无复发,预计未来将进行进一步随访。
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