TUMORAL PRESENTATION OF AMYLOIDOSIS (AMYLOIDOMAS) IN SOFT-TISSUES - A REPORT OF 14 CASES
TUMORAL PRESENTATION OF AMYLOIDOSIS (AMYLOIDOMAS) IN SOFT-TISSUES - A REPORT OF 14 CASES
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DOI：
10.1093/ajcp/100.2.135
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发表时间：
1993-08-01
影响因子：
3.5
通讯作者：
FRIZZERA, G
中科院分区：
文献类型：
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作者：
KRISHNAN, J;CHU, WS;FRIZZERA, G
淀粉样变性在其不同类型（免疫细胞性恶液质相关的，反应性的，或遗传家族性的）中最常表现为系统性形式。局限性淀粉样变性一般不常见，在软组织中更是极为罕见。作者讨论了14例淀粉样变表现为软组织（主要是纵隔和腹膜后）局部肿块（“淀粉样瘤”）的患者，导致大多数病例临床诊断为肿瘤。根据相关的形态学非典型性和表型单克隆细胞群，高锰酸钾预处理的抗性，以及缺乏与抗AA抗血清的反应性，10例可被归类为免疫细胞性恶液质相关的AL-淀粉样变性。 然而，4例病例具有反应性（“继发性”）AA-淀粉样变性的组织病理学、组织学和免疫组化特征。这一比例（28.5%）高于文献报道的散发性AA淀粉样瘤。这两种类型之间的病理学区别是重要的，因为软组织的AA淀粉样瘤患者似乎有更好的预后。
Amyloidosis in its diverse types (immunocytic dyscrasia-associated, reactive, or heredofamilial) most often presents in a systemic form. Localized amyloidosis is uncommon in general and is exceedingly rare in the soft tissues. The authors discuss the cases of 14 patients in whom amyloidosis manifested as a localized mass (''amyloidoma'') in the soft tissues (mostly mediastinal and retroperitoneal), leading to a clinical diagnosis of neoplasm in most cases. On the basis of the associated morphologically atypical and phenotypically monoclonal cell population, the resistance to potassium permanganate pretreatment, and the lack of reactivity with anti-AA antisera, 10 cases could be classified as immunocytic dyscrasia-associated AL-amyloidosis. However, four cases had histopathologic and histo- and immunohistochemical characteristics of reactive (''secondary'') AA-amyloidosis. This proportion (28.5%) was higher than that suggested by the sporadic AA-amyloidomas reported in the literature. The pathologic distinction between these two categories is important because patients with AA-amyloidomas of the soft tissues appear to have a better prognosis.