假肌源性血管内皮瘤6例临床病理分析  

Pseudomyogenic hemangioendothelioma:a clinicopathological study of 6 cases

作　　者：汪庆余[1] 郝华[1] 刘绮颖 喻林[2] 王坚[2] 

机构地区：[1]南昌大学第二附属医院病理科,南昌330006 [2]复旦大学附属肿瘤医院病理科,上海200032

出　　处：《临床与实验病理学杂志》2014年第10期1122-1126,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨假肌源性血管内皮瘤（pseudomyogenic hemangioendothelioma，PHE）的临床病理学特征、免疫表型、诊断及鉴别诊断。方法对6例PHE的临床病理资料和免疫表型进行回顾性复习。结果男性4例，女性2例。平均年龄32岁（10～70岁）。3例原发于骨内，均为多骨性累及，其中1例伴发皮肤结节；另3例分别表现为软组织局部肿块、皮肤和软组织多灶性病变及皮肤黑色斑块。影像学检查：3例骨病变均表现为多骨性骨质破坏，1例皮肤和软组织多灶性病变累及多个组织平面。眼观：送检肿瘤组织呈灰白、灰红或灰褐色，直径0．5～4cm，平均2cm。镜检：病变边界不清，可累及真皮、皮下、肌肉或骨组织。肿瘤主要由疏松条束状、结节状或片状分布的胖梭形细胞组成，部分区域内瘤细胞呈上皮样，间质内可见散在的中性粒细胞。高倍镜下见瘤细胞胞质丰富，嗜伊红色，核染色质呈空泡状，可见小核仁，形态上类似横纹肌母细胞。除1例局部区域外，瘤细胞异型性不明显或仅有轻度异型性，核分裂象为1～2个／10HPF。部分区域内可见炎性或液化性坏死。免疫表型：瘤细胞弥漫强阳性表达CK（AE1／AE3），不同程度表达CD31和FLI1，INI-1标记显示无缺失，Ki-67平均增殖指数约5％。本组6例中2例复发，其中1例在短期内发生双肺转移。结论PHE是一种局部易复发偶有转移的中间性血管内皮瘤。Purpose To investigate the clinicopathological features and immunophenotype of pseudomyogenic hemangioendothelioma （PHE） with emphasis on diagnosis and differential diagnosis. Methods The clinical, pathological and immunophenotypic profiles in six cases of PHE were reviewed. Results Tumors occurred in four males and two females with age at presentation ranging from 10 to 70 years （mean, 32 years）. Three tumors developed primarily in the bones, one of which had a concurrent skin nodule. One arose in the somatic soft tissues, 1 presented as muhiple diseases involving the skin and soft tissues and 1 as a small black papule on the skin. Radiological examination in one ease with both skin and soft tissue lesions revealed multiple nodules involving different tissues, and presented as muhifocal destructive disease in 3 bony cases. Grossly, tumors were soft and solid with gray to tan color, ranging in size from 5 mm to 4 cm （mean, 2 cm）. On histology, they were ill-circumscribed and involved the dermis, subcntis, muscles or bones. They were all composed of loose fascicles or sheets of plump spindled cells with abundant eosinophilic cytoplasm, vesicular nuclei and distinct nucleoli, bearing a close resemblance to rhabdomyoblasts. Apart from focal areas in 1 case, nuclear atypia was not prominent and mitotic figures were scarce （1～2/10 HPF）. By immunohistochemistry, all cases were strongly positive for CK （AE1/AE3） with variable reactivity for CD31 and FLI1, and intact expression of INI-1. The average index of Ki-67 was about 5%. Two patients experi- enced local recurrence, one of whom developed bilateral lung metastases within a short period. Conclusions Awareness of the clinical and pathological characteristics of PHE will help in the distinction from epithelioid neoplasms with overlapping features, especially epi- thelioid sarcoma and epithelioid hemangioendothelioma. Biologically, PHE belongs to the category of intermediate malignancy with a tendency for local recurrence and a low potential of dev

关 键 词：软组织肿瘤 血管内皮瘤 横纹肌母细胞 

分 类 号：R738.6[医药卫生—肿瘤]