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Lymphatic-type "Angiosarcoma" With Prominent Lymphocytic Infiltrate.

淋巴细胞浸润明显的淋巴管型 “血管肉瘤”。

  • 影响因子:6.06
  • DOI:10.1097/PAS.0000000000001398
  • 作者列表:"Martinez AP","Zapata M","North PE","Folpe AL","Weiss SW
  • 发表时间:2020-02-01
Abstract

:We report 21 cases of a distinctive and unique vascular tumor which we propose to be a pure lymphatic-type angiosarcoma characterized by architectural and growth characteristics of angiosarcoma, cytologic, and immunohistochemical features of lymphatic differentiation, a prominent lymphocytic infiltrate, and variable nuclear grade. Patients included 12 males and 9 females with a median age of 65 years (range: 32 to 95 y). Tumors involved the head and neck (n=11), lower extremities (n=5), trunk (n=4), and upper extremity (n=1) and were located superficially in the dermis and/or subcutis. Tumors were designated "low grade" (n=10) when the nuclear grade was low, and vascular channel formation was evident throughout but with multilayering of endothelium within the vessels. Cases were designated "high grade" (n=11) when nuclei appeared higher grade with more rounded contours and prominent nucleoli and when solid areas predominated over vascular channel formation. A striking feature of both groups was the presence of a dense, lymphocytic infiltrate with occasional germinal center formation. All cases strongly and diffusely expressed at least 1 lymphatic marker (21/21) with podoplanin (17/19) and Prox-1 (11/11) more commonly expressed than LYVE-1 (5/10). No consistent molecular alteration was identified. Follow-up on 17 patients (median: 41 mo, mean: 54 mo) showed 10 patients were alive without disease, 5 were alive with disease, 1 died of other cause, and 1 died of disease. Local recurrence developed in 9 cases and metastasis in 2 cases, although neither correlated with grade as defined. On the basis of clinical follow-up to date, the natural history of lymphatic-type angiosarcoma appears to be more favorable than other forms of cutaneous angiosarcoma.

摘要

: 我们报告了 21 例独特的血管肿瘤,我们提出是一种纯淋巴型血管肉瘤,其特征是血管肉瘤的结构和生长特征,细胞学,和免疫组织化学特征的淋巴分化,一个突出的淋巴细胞浸润,和可变的核级别。患者包括 12 例男性和 9 例女性,中位年龄 65 岁 (范围: 32 至 95 岁)。肿瘤累及头颈部 (n = 11) 、下肢 (n = 5) 、躯干 (n = 4) 和上肢 (n = 1) 位于真皮和/或皮下。当核级别低时,肿瘤被命名为 “低级别” (n = 10),血管通道形成明显但血管内内皮多层。当细胞核出现更高级别的,轮廓更圆,核仁突出,并且固体区域主导血管通道形成时,病例被指定为 “高级” (n = 11)。两组的一个显著特征是存在密集的淋巴细胞浸润,偶有生发中心形成。所有病例均强烈弥漫表达至少 1 个淋巴标记物 (21/21),podoplanin (17/19) 和Prox-1 (11/11) 比LYVE-1 (5/10) 更常见。未发现一致的分子改变。随访 17 例患者 (中位数: 41 mo mo,平均: 54  mo),10 例患者无病存活,5 例存活,1 例死于其他原因,1 人死于疾病。局部复发 9 例,转移 2 例,尽管与定义的分级均不相关。根据迄今为止的临床随访,淋巴管型血管肉瘤的自然史似乎比其他形式的皮肤血管肉瘤更有利。

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影响因子:0.96
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皮肤肿瘤方向

皮肤肿瘤是发生在皮肤的细胞增生性疾病,是一种常见病。发生于皮内或皮下组织的新生物,种类很多,临床上分良性肿瘤和恶性肿瘤。恶性肿瘤可以不断增殖,引起转移,威胁生命,称为皮肤癌。

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