INFLAMMATORY FIBROSARCOMA OF THE MESENTERY AND RETROPERITONEUM - A TUMOR CLOSELY SIMULATING INFLAMMATORY PSEUDOTUMOR
INFLAMMATORY FIBROSARCOMA OF THE MESENTERY AND RETROPERITONEUM - A TUMOR CLOSELY SIMULATING INFLAMMATORY PSEUDOTUMOR
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DOI：
10.1097/00000478-199112000-00005
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发表时间：
1991-12-01
影响因子：
5.6
通讯作者：
ENZINGER, FM
中科院分区：
文献类型：
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作者：
MEIS, JM;ENZINGER, FM
本文报告38例炎性纤维肉瘤，女性23例，男性15例，年龄2个月至74岁(中位数8.5岁，平均15岁)，有腹痛(17例)、贫血(21例)、发热(14例)、肿块(16例)和胃肠道梗阻(7例)等症状。原发部位分别为肠系膜及腹膜后(31例)、大网膜(2例)、纵隔(2例)、肝脏(1例)、横隔膜(1例)、腹壁(1例)。大小2.4~20 cm，平均9.6 cm。27例随访资料显示10例局部复发，3例多次局部复发，组织学证实有肺(2例)和脑(1例)远处转移。5名患者死于他们的疾病(中位数为20个月)。所有肿瘤，包括转移瘤，都由成纤维细胞、肌成纤维细胞和浆细胞组成，有不同程度的纤维化和钙化。免疫组织化学染色提示肌纤维母细胞分化；20例中有18例(90%)表达肌动蛋白，15例(83%)表达波形蛋白，13例(77%)表达角蛋白(主要位于间皮下部位)。超微结构研究还揭示了肌成纤维细胞的特征。这些肿瘤的局部侵袭性、复发性，以及转移和肿瘤死亡的发生，表明它们是潜在的恶性肿瘤，我们认为更好地将它们归类为炎性纤维肉瘤，而不是细胞性炎性假瘤。
We report 38 cases of inflammatory fibrosarcoma occurring in 23 females and 15 males, 2 months to 74 years of age (median, 8.5 years; mean, 15 years) with symptoms of abdominal pain (17 cases), anemia (21 cases), fever (14 cases), mass (16 cases), and gastrointestinal obstruction (7 cases). Primary tumor sites included mesentery and retroperitoneum (31 cases), omentum (two cases), mediastinum (two cases), liver (one case), diaphragm (one case), and abdominal wall (one case). Sizes ranged from 2.4 cm to 20 cm (mean, 9.6 cm). Follow-up data in 27 cases revealed local recurrences in 10 patients, with multiple local recurrences in three and histologically proven distant metastases to lung (two cases) and brain (one case). Five patients died from their disease (median, 20 months). All tumors, including metastases, consisted of fibroblasts, myofibroblasts, and plasma cells, with variable degrees of fibrosis and calcification. Immunostains indicate myofibroblastic differentiation; 18 of 20 (90%) stained for actin, 15 of 18 (83%) for vimentin, and 10 of 13 (77%) for keratin (primarily in a submesothelial location). Ultrastructural studies also disclosed myofibroblastic features. The locally aggressive, recurrent nature of these neoplasms, as well as the occurrence of metastases and tumor deaths, indicate that they are potentially malignant neoplasms that we believe are better classified as inflammatory fibrosarcomas, not as cellular inflammatory pseudotumors.