Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder
Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder
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DOI：
10.1097/00019606-199704000-00005
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发表时间：
1997-04-01
影响因子：
--
通讯作者：
Wolfe, HJ
中科院分区：
文献类型：
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作者：
Alman, BA;Pajerski, ME;Wolfe, HJ
侵袭性纤维瘤病（也称为深部纤维瘤病或硬纤维瘤）是一种细胞学上表现为良性的纤维细胞增殖，通常导致显著的功能丧失。病变的性质是有争议的：一些证据表明，这是一个反应过程，而其他证据支持肿瘤病因。X染色体失活的模式，使用聚合酶链反应（PCR）扩增的基础上的高变CAG重复区侧翼HHal限制性位点的人雄激素受体基因的技术，确定在4例中，其中冷冻保存的肿瘤和邻近的正常组织。所有四个肿瘤表现出单克隆模式，而相邻的正常组织表现出多克隆模式。这表明侵袭性纤维瘤病是来源于具有生长优势的单个克隆的细胞的增殖，因此可能是肿瘤过程。
Aggressive fibromatosis (also called deep fibromatosis or desmoid tumor) is a proliferation of cytologically benign-appearing fibrocytes, often resulting in significant functional loss. The nature of the lesion is controversial: some evidence suggests that it is a reactive process, whereas other evidence supports a neoplastic etiology. The pattern of X chromosome inactivation, using a technique based on polymerase chain reaction (PCR) amplification of a hypervariable CAG repeat region flanking Hhal restriction sites of the human androgen receptor gene, was determined in four cases in which cryopreserved tumor and adjacent normal tissue were available. All four tumors demonstrated a monoclonal pattern, while the adjacent normal tissues demonstrated a polyclonal pattern. This demonstrates that aggressive fibromatosis is proliferation of cells derived from a single clone with a growth advantage, and thus is likely a neoplastic process.