Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Am J Surg Pathol. 2010 Apr;34(4):447-53. doi: 10.1097/PAS.0b013e3181d325c0.
Sclerosing mesenteritis (SM), also known as mesenteric lipodystrophy, rarely involves the parenchyma of the pancreas. When SM does involve the pancreas, it can mimic pancreatic carcinoma both clinically and radiographically with pain, obstructive jaundice, a mass lesion, and even the appearance of vascular invasion. We report 6 patients with SM involving the pancreas (mean age 43.2 y, 5 female), and review their clinical presentation, radiographic findings, pathology, and outcome. Five of these 6 patients were originally thought to have a primary pancreatic neoplasm. Initial presenting clinical information was available for each patient: all 6 reported abdominal or epigastric pain, 3 reported weight loss, and 2 reported one or more of the following: back pain, fever, abdominal bloating/distention, nausea with/without vomiting, and anorexia. The lesions formed masses with an infiltrative pattern and all had 3 key histologic features: fibrosis, chronic inflammation, and fat necrosis-without a known etiology. The inflammatory infiltrate was composed of a mixture of lymphocytes, plasma cells, and scattered eosinophils. Of the 5 patients with post-treatment clinical information available, 4 had at least a partial response to treatment with steroids, tamoxifen, azathioprine, resection, or a combination of these, and 1 did not respond. A dramatic response to immunosuppressive therapy is illustrated by the case of a 46-year-old woman who presented with the presumptive diagnosis of an unresectable pancreatic cancer. Distinguishing SM from pancreatic carcinoma is crucial to appropriate management, as patients with SM may benefit from immunosuppressive therapy.
硬化性肠系膜炎(SM),也称为肠系膜脂肪营养不良,很少累及胰腺实质。当 SM 确实累及胰腺时,其临床表现和影像学表现可与胰腺癌相似,表现为疼痛、阻塞性黄疸、肿块病变,甚至出现血管侵犯。我们报告了 6 例累及胰腺的 SM 患者(平均年龄 43.2 岁,5 例女性),并回顾了他们的临床表现、影像学表现、病理学和结局。这 6 例患者中有 5 例最初被认为患有原发性胰腺肿瘤。每位患者均提供了初始临床表现信息:6 例均报告有腹痛或上腹痛,3 例报告有体重减轻,2 例报告有以下一种或多种症状:背痛、发热、腹胀/扩张、恶心伴/不伴呕吐和食欲不振。病变形成肿块,呈浸润性模式,均具有 3 个关键组织学特征:纤维化、慢性炎症和脂肪坏死-无已知病因。炎症浸润由混合淋巴细胞、浆细胞和散在的嗜酸性粒细胞组成。在有治疗后临床信息的 5 例患者中,4 例对皮质类固醇、他莫昔芬、硫唑嘌呤、切除术或这些方法的联合治疗至少有部分反应,1 例无反应。一位 46 岁女性的病例说明了免疫抑制治疗的显著反应,她被诊断为无法切除的胰腺癌。区分 SM 和胰腺癌对于适当的管理至关重要,因为 SM 患者可能受益于免疫抑制治疗。