Khoo Teck Puat Hospital, Singapore.
National University Hospital, Singapore.
Ann R Coll Surg Engl. 2021 Feb;103(2):e53-e55. doi: 10.1308/rcsann.2020.7003.
Inflammatory myofibroblastic tumours (IMTs) are rare tumours with unpredictable biological behaviour ranging from benign to locally invasive and rarely, distant metastasis. While neurofibromatosis type 1 (NF1) may manifest with gastrointestinal soft tissue tumours, this is the first report in the literature that describes an IMT occurring in a NF1 patient who presented with intestinal obstruction. Our patient presented with intestinal obstruction secondary to an obstructing terminal ileum mesenteric tumour. bowel resection was performed, with histology revealing an IMT and an adjacent neurofibroma. The resection margins were clear and the patient was free of recurrence at six months.
炎性肌纤维母细胞瘤(IMTs)是一种罕见的肿瘤,其生物学行为具有不可预测性,从良性到局部侵袭性,甚至罕见的远处转移。神经纤维瘤病 1 型(NF1)可能表现为胃肠道软组织肿瘤,但这是文献中首次描述 NF1 患者出现肠梗阻的 IMT。我们的患者因回肠末端肠系膜肿瘤导致肠梗阻而就诊。进行了肠切除术,组织学显示为 IMT 和相邻的神经纤维瘤。切除边缘清晰,患者在 6 个月时无复发。