肠系膜纤维瘤病:一例罕见的实体病例报告

Swati Sharma, Anupam Sarma, Deep Jyoti Kalita, Shiraj Ahmed, Lopa Mudra Kakoti
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摘要

肠系膜纤维瘤病或硬纤维瘤是深纤维瘤病的一部分。深纤维瘤病是一组增生性,局部侵袭性病变,可浸润性,可复发而无转移。病例报告:一名42岁男性以腹痛不适来医院就诊。影像学上发现肠系膜肿瘤,并考虑胃肠道肿瘤的诊断。组织病理学检查诊断为低级别梭形细胞瘤。IHC结果显示SMA阳性,而DOG1、CD34、desmin和CD117呈阴性。Ki67约为4%。HPE和IHC倾向于诊断肠系膜纤维瘤病。结论:肠系膜间质瘤病与纤维瘤病在临床表现上非常相似。放射学表现与病理表现相当相似。免疫组织化学有助于区分两者。这两个完全不同的实体有不同的管理,因此应区分,以避免不必要的治疗良性纤维瘤病的情况下。
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Mesenteric firomatosis: A rare entity- case report
Mesenteric fibromatosis or desmoids are a part of spectrum of deep fibromatosis. Deep fibromatosis is a group of proliferative, locally aggressive lesions that can be infiltrative and can recur without metastasis. Case report: A 42-year male came to hospital with complains of abdominal pain and discomfort. On imaging a mesenteric tumour was noted and diagnosis of gastrointestinal tumour was considered. On histopathological examination a spindle cell tumour of low grade was diagnosed. IHC came out to be SMA positive and negative for DOG1, CD34, desmin and CD117. Ki67 was around 4%. HPE and IHC favored a diagnosis of mesenteric fibromatosis. Conclusion: mesenteric GIST and fibromatosis are very similar in clinical & radiological presentation and moreover pathological picture is quite similar. To differentiate the two immunohistochemistry helps. The two entirely poles apart entities have different management hence should be differentiated to avoid unnecessary treatment in case of benign fibromatosis.
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