腹膜孤立性纤维性肿瘤:罕见肿瘤的不寻常部位

Lamghari M, Azzaoui Ie, S. M., A. Em, Najih M, M. M, Bonchentouf Sm, Kaoui He, Bounaim A
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摘要

SFT是一种罕见的肿瘤,占所有软组织肉瘤和间质肿瘤的3.7%。最常见的部位是胸膜,其次是腹部。病例报告:一名68岁男性患者因腹痛及腹下区移动包块入住我科。ct扫描显示一个清晰的肿块,在动脉和静脉阶段均有明显的均匀增强特征。采用正中脐下剖腹手术对肿瘤进行完全切除。组织学检查显示为纤维母细胞间充质肿瘤,免疫组化研究显示CD34、CD99和Bcl2表达为孤立性纤维性肿瘤的特异性表达。讨论:SFT是解剖学上普遍存在的由成纤维细胞发展而来的间充质肿瘤。它是一种罕见的肿瘤,占所有软组织肉瘤和间质肿瘤的3.7%。最常见的部位是胸膜,其次是腹部。临床上,腹部SFT通常表现为腹部充盈、胃肠梗阻、体重减轻、黄疸、发热或低血糖。免疫组化发现,SFT细胞典型表达以下标记物:CD34、CD99和Bcl2。完全手术切除肿瘤是金标准治疗,可通过辅助放疗完成。结论:腹膜原发性SFTs极为罕见。临床症状及影像学表现无特异性,故诊断困难。治疗包括手术切除、栓塞治疗、放射治疗、化疗和抗血管生成药物。
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Solitary Fibrous Tumor of the Peritoneum: An Unusual Location of A Rare Tumor
Introduction: SFT is an uncommon tumor representing 3, 7% of all soft tissue sarcomas and mesenchymal tumor. The most common location is pleura followed by the abdomen. Case Report: A 68 years old male patient was admitted to our department with abdominal pain and a mobile masse in his abdominal hypogastric area. CT-scan showed a well-defined mass with vividly homogenous enhancing features in both the arterial and venous phases. A complete resection of the tumor was performed thought a median sub-ombilical laparotomy. The histological examination showed a fibroblastic mesenchymal tumor with expression of CD34, CD99 and Bcl2 in the immunohistochemical study that is specific of the solitary fibrous tumor. Discussion: SFT are anatomically ubiquitous mesenchymal tumors developed from fibroblasts. It is an uncommon tumor represents 3, 7% of all soft tissue sarcomas and mesenchymal tumors. The most common location is the pleura followed by the abdomen. Clinically, abdominal SFT is usually manifest as abdominal fullness, gastrointestinal obstruction, weight loss, jaundice, fever, or hypoglycemia. Immunohistochemistry, the cell of the SFT typically express the following markers: CD34, CD99 and Bcl2. Complete surgical removal of the tumor is the gold standard treatment which can be completed by adjuvant radiotherapy. Conclusion: Primary SFTs in the peritoneum are extremely uncommon. Clinical symptoms and imaging manifestations are nonspecific whish make the diagnosis difficult. Treatment includes surgical resection, embolization therapy, radiation therapy, chemotherapy and anti-angiogenic agents.
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