Yantiss Rhonda K, Nielsen G Petur, Lauwers Gregory Y, Rosenberg Andrew E
Department of Pathology, University of Massachuestts Memorial Health Care, 55 Lake Avenue North, Worcester, MA 01655, USA.
Am J Surg Pathol. 2003 Apr;27(4):532-40. doi: 10.1097/00000478-200304000-00015.
Although the majority of mesenchymal lesions of the gastrointestinal tract are neoplastic in nature, nonneoplastic reactive processes may involve the gastrointestinal tract and mesentery, causing diagnostic confusion with more aggressive neoplasms, such as fibromatosis or gastrointestinal stromal tumors. In this study, we report a series of fibroinflammatory lesions of the gastrointestinal tract that we think represent a relatively cohesive group of tumors and describe the clinical and pathologic features of this entity, which we have termed "reactive nodular fibrous pseudotumor." The tumors affected five patients (four male and one female patient) who ranged in age from 48 to 71 years (mean 56 years). Two patients presented with acute abdominal pain without a significant past medical history, two had incidental lesions discovered during evaluation for other medical conditions, and one was found to have an abdominal mass. Three patients had a history of abdominal surgery. The tumors were multiple in three patients and solitary in two patients. In four cases, at least one of the tumors involved the small intestine or colon, and the lesion was confined to the peripancreatic soft tissue in one case. The tumors were firm, tan-white, ranged in size from 4.3 to 6.5 cm in greatest dimension, and were grossly well circumscribed. All of the lesions were of low to moderate cellularity and composed of stellate or spindled fibroblasts arranged haphazardly or in intersecting fascicles. Three cases had microscopically infiltrative borders. The stroma was rich in collagen, which was wire-like, keloidal, or hyalinized. Intralesional mononuclear cells were sparse but were more numerous peripherally and frequently arranged in lymphoid aggregates. Immunohistochemical stains demonstrated that all of the tumors stained for vimentin, 80% stained for CD117 or muscle specific actin, 60% stained for smooth muscle actin or desmin, and none of the tumors stained for CD34, S-100 protein, or anaplastic lymphoma kinase-1. Follow-up information was available in all cases: four patients had no residual disease following surgical resection (mean follow-up 16.3 months) and one patient who had an incomplete surgical resection had stable disease at 26 months. In summary, we report a series of distinct intraabdominal fibroinflammatory pseudotumors that we have collectively termed "reactive nodular fibrous pseudotumors." These lesions are uncommon and may infiltrate the bowel wall, thereby mimicking primary bowel neoplasms or intraabdominal fibromatosis. Recognition of these nonneoplastic lesions is important, as they pursue a benign clinical course, but may be confused with other mesenchymal neoplasms that require more aggressive treatment.
虽然胃肠道的大多数间质性病变本质上是肿瘤性的，但非肿瘤性反应性过程可能累及胃肠道和肠系膜，导致与更具侵袭性的肿瘤（如纤维瘤病或胃肠道间质瘤）产生诊断混淆。在本研究中，我们报告了一系列胃肠道纤维炎性病变，我们认为它们代表了一组相对具有一致性的肿瘤，并描述了这一实体的临床和病理特征，我们将其称为“反应性结节性纤维假瘤”。这些肿瘤累及5例患者（4例男性和1例女性），年龄在48至71岁之间（平均56岁）。2例患者表现为急性腹痛，既往无重大病史，2例在评估其他疾病时偶然发现病变，1例发现腹部肿块。3例患者有腹部手术史。3例患者的肿瘤为多发，2例患者为单发。4例患者中，至少有1个肿瘤累及小肠或结肠，1例病变局限于胰周软组织。肿瘤质地硬，呈棕白色，最大直径为4.3至6.5 cm，大体边界清晰。所有病变细胞密度低至中等，由星状或梭形成纤维细胞组成，排列杂乱或呈交叉束状。3例显微镜下边界呈浸润性。间质富含胶原，呈丝状、瘢痕疙瘩样或玻璃样变。瘤内单核细胞稀少，但周边较多，常排列成淋巴样聚集。免疫组化染色显示，所有肿瘤波形蛋白染色阳性，80%的肿瘤CD117或肌肉特异性肌动蛋白染色阳性，60%的肿瘤平滑肌肌动蛋白或结蛋白染色阳性，所有肿瘤CD34、S-100蛋白或间变性淋巴瘤激酶-1染色均为阴性。所有病例均有随访信息：4例患者手术切除后无残留疾病（平均随访16.3个月），1例手术切除不完全的患者在26个月时病情稳定。总之，我们报告了一系列独特的腹腔内纤维炎性假瘤，我们将其统称为“反应性结节性纤维假瘤”。这些病变不常见，可能浸润肠壁，从而酷似原发性肠肿瘤或腹腔内纤维瘤病。认识这些非肿瘤性病变很重要，因为它们临床过程良性，但可能与其他需要更积极治疗的间质性肿瘤混淆。
