Department of Radiology, Shougang Hospital, Peking University, Beijing, China.
Pathology Department, Shougang Hospital, Peking University, Beijing, China.
Medicine (Baltimore). 2022 Sep 9;101(36):e30326. doi: 10.1097/MD.0000000000030326.
Desmoid-type fibromatosis is a rare benign mesenchymal neoplasm. Only 8% of desmoid-type fibromatosis develops in the abdominal cavity. The mesentery is seldom affected and gastrointestinal stromal tumors need to be considered in the differential diagnosis, particularly when imaging examination shows a tumor containing gases in the abdominal cavity. Only a few cases of gas-containing mesenteric desmoid-type fibromatosis have been reported in the literature.
A 69-year-old male patient presented with hematochezia and intermittent upper abdominal pain.
Contrast-enhanced computed tomography revealed a 3.9 × 3.6 cm gas-containing mass infiltrating the third portion of the duodenum. The tumor was heterogeneous, with cysts and air bubbles. It showed heterogeneous weak-to-mild enhancement in the solid part. Postoperative pathological examination confirmed a final diagnosis of mesenteric desmoid-type fibromatosis.
The patient underwent surgical resection of intra-abdominal lesion.
No evidence of local recurrence was noted during the 6 months of follow-up.
Accurate preoperative diagnosis is difficult for an intra-abdominal gas-containing mass on computed tomography scan. The appearance of spiculated infiltrative margin suggests the diagnosis of desmoid-type fibromatosis. Further investigation of imaging evidence and treatment methods is necessary.
硬纤维瘤病（desmoid-type fibromatosis）为一种罕见的良性间叶组织肿瘤，仅 8%发生于腹腔。肠系膜受累少见，需与胃肠道间质瘤（gastrointestinal stromal tumors）相鉴别，尤其当影像学检查提示腹腔内存在含气性肿瘤时。文献中仅报道了少数含气性肠系膜硬纤维瘤病病例。
一位 69 岁男性患者因血便和间歇性上腹痛就诊。
增强 CT 显示一个 3.9×3.6cm 的含气性肿块浸润十二指肠第三段。肿瘤呈异质性，存在囊肿和气泡，实性部分呈不均匀弱-轻度强化。术后病理检查证实为肠系膜硬纤维瘤病。
患者接受了腹腔内病变的手术切除。
随访 6 个月时未发现局部复发迹象。
CT 扫描显示腹腔内含气性肿块时，术前准确诊断较为困难。呈刺状浸润性边缘的表现提示硬纤维瘤病的诊断。需进一步研究影像学证据和治疗方法。
