Koechlin Luca, Zettl Andreas, Koeberle Dieter, von Flüe Markus, Bolli Martin
Department of Surgery, St. Claraspital, Basel, Switzerland.
Department of Pathology, Viollier AG, Basel, Switzerland.
Case Rep Surg. 2016;2016:8593242. doi: 10.1155/2016/8593242. Epub 2016 Nov 28.
Inflammatory myofibroblastic tumors (IMT) of the spleen are rare neoplasms and only little is known about the origin and behavior of these tumors. Here we report the case of a 37-year-old woman with an atypical spindle cell neoplasm showing features strongly suggesting an IMT of the spleen with hepatic metastasis. A 37-year-old patient had been complaining about pain in the left upper abdomen for the last two months. A CT scan revealed a tumor mass in her spleen and liver. After complete staging, a splenectomy and atypical liver resection of segments VII and VIII were performed. Literature was screened for similar cases and existing further literature. A R0 resection was achieved. Histological analysis showed a multinodular infiltration of the spleen by an atypical mesenchymal neoplasia. Immunohistochemically there was an expression of histiocytic markers (CD4, CD68) as well as smooth muscle cell markers (SMA, H-Caldesmon) in the tumor cells. A diagnosis of an atypical spindle cell neoplasm showing features most suggestive of an IMT was rendered. Synchronous hepatic metastasis of an IMT of the spleen is a rarity. Therefore no experience in the treatment of these tumors exists. Fibroblastic reticular cell tumor is a differential diagnosis, but differentiation of these two entities is difficult.
脾脏炎性肌纤维母细胞瘤(IMT)是一种罕见的肿瘤,关于这些肿瘤的起源和行为知之甚少。在此,我们报告一例37岁女性,患有非典型梭形细胞瘤,其特征强烈提示为脾脏IMT伴肝转移。一名37岁患者在过去两个月一直抱怨左上腹疼痛。CT扫描显示其脾脏和肝脏有肿瘤肿块。在完成全面分期后,进行了脾切除术以及肝脏VII段和VIII段的非典型肝切除术。对文献进行了筛查,以寻找类似病例及现有其他文献。实现了R0切除。组织学分析显示脾脏被非典型间叶性肿瘤多结节浸润。免疫组化显示肿瘤细胞中存在组织细胞标志物(CD4、CD68)以及平滑肌细胞标志物(SMA、H - 钙调蛋白)的表达。做出了非典型梭形细胞瘤的诊断,其特征最提示为IMT。脾脏IMT的同步肝转移很罕见。因此,目前尚无治疗这些肿瘤的经验。纤维母细胞网状细胞瘤是一种鉴别诊断,但区分这两种实体很困难。