Nuclear β-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions
Nuclear β-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions
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DOI：
10.1097/01.pas.0000157938.95785.da
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发表时间：
2005-05-01
影响因子：
5.6
通讯作者：
Montgomery, E
中科院分区：
文献类型：
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作者：
Bhattacharya, B;Dilworth, HP;Montgomery, E
深层纤维瘤（硬纤维瘤）是一种无性系肌纤维母细胞增生，易于局部侵袭性复发，但不会转移。它们必须与纤维母细胞和肌纤维母细胞病变以及平滑肌肿瘤区分开来。几乎所有的深层纤维瘤病都有体细胞β -连环蛋白或腺瘤性大肠息肉病（APC）基因突变导致核内β -连环蛋白积累。由于低级别肉瘤通常缺乏P-catenin，而且反应性增生不可能有它，因此我们预测在深部纤维瘤中可以检测到核β -catenin的表达，而在其他实体的鉴别诊断中则没有。我们评估了β -连环蛋白的作用，以帮助区分深纤维瘤从同系物。Formalin-fixed,石蜡包埋部分来自21个病变20例深fibromatoses患者沾单克隆β-连环蛋白抗体(转导实验室),相比之下,低级fibromyxoid肉瘤(n = 12),平滑肌肉瘤(n = 10),其他各种纤维肉瘤变体(n = 13包括3 myofibrosarcomas 3硬化性上皮状的纤维肉瘤,5低级纤维肉瘤,我产生典型的纤维肉瘤dermatofibrosarcoma protuberans,1例炎性黏液透明瘤/黏液炎性纤维母细胞肉瘤)、肌纤维瘤/肌纤维瘤病（n = 12）、结节性筋膜炎（n = 11）和疤痕（n = 9）。进行细胞核和细胞质染色。所有21例深部纤维瘤病均显示核β -连环蛋白（1例局灶性核染色至90%染色）。所有其他检测的病变（n = 67）缺乏β -连环蛋白的核标记，仅显示细胞质积累。β -连环蛋白免疫组化在鉴别诊断中将深部纤维瘤病从实体中分离出来，这一发现可用于诊断。大多数纤维瘤瘤有弥漫性核染色，虽然偶尔也有局部标记。
Deep fibromatoses (desmoid tumors) are clonal myofibroblastic proliferations that are prone to aggressive local recurrences but that do not metastasize. They must be distinguished from a host of fibroblastic and myofibroblastic lesions as well as from smooth muscle neoplasms. Virtually all deep fibromatoses have somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations leading to intranuclear accumulation of beta-catenin. Since low-grade sarcomas in general lack P-catenin and since reactive proliferations would not be expected to have it, we predicted that nuclear beta-catenin expression would be detected in deep fibromatoses but absent in other entities in the differential diagnosis. We evaluated the role of beta-catenin to help differentiate distinguish deep fibromatoses from congeners. Formalin-fixed, paraffin-embedded sections from 21 lesions from 20 patients with deep fibromatoses were stained with monoclonal beta-catenin antibody (Transduction Laboratories) and compared with low-grade fibromyxoid sarcoma (n = 12), leiomyosarcoma (n = 10), various other fibrosarcoma variants (n = 13, including 3 myofibrosarcomas, 3 sclerosing epithelioid fibrosarcomas, 5 low-grade fibrosarcomas, I classic fibrosarcoma arising in dermatofibrosarcoma protuberans, I inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma), myofibroma/myofibromatosis (n = 12), nodular fasciitis (n = 11), and scars (n = 9). Nuclear and cytoplasmic staining was assessed. All 21 examples of deep fibromatosis displayed nuclear beta-catenin (focal nuclear staining in one case to 90% staining). All other lesions tested (n = 67) lacked nuclear labeling for beta-catenin, showing only cytoplasmic accumulation. beta-Catenin immunohistochemistry separates deep fibromatosis from entities in the differential diagnosis, a finding that can be exploited for diagnosis. Most fibromatoses have diffuse nuclear staining although occasional examples only focally label.