一直以来，上华夏网收获很大，今天上传一个有意思的病例，(ˇˍˇ） 想和大家一起讨论一下！

学习

 这个病可以活多久

上皮样血管内皮细胞瘤：是一种以血管为中心的血管肿瘤，有转移潜能，由排列成短条索和巢状结构的上皮样内皮细胞构成，细胞周围有独特的黏液玻璃样间质。

学习

 关键有酶标支持： CD31/Fli-1表达情况？

此例，已经送到肿瘤医院王坚大夫手中，等病人回来，我会把结果告诉大家！

上皮样血管内皮瘤，好病例

见上楼，美国外科病理学上有文章说明皮肤和软组织中的上皮样血管内皮细胞瘤中有45%的病例SMA是阳性的。

中文文章页查到有，就不引用了！

本例SMA阳性就可以解释了！

Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher CD.

Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and  immunohistochemical study of 30 cases.

Am J Surg Pathol. 1997. 21(4): 363-74.

Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the  head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round,  to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10,  more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is  better regarded as a fully malignant, rather than borderline, vascular neoplasm,  albeit the prognosis is better than in conventional angiosarcoma.

 图一：典型的印戒样血管内皮细胞和口含红珠的血管（用战斗机的尖部表示）

图二：华夏病理上确诊肝脏上皮样血管内皮细胞瘤，注意印戒样血管内皮细胞。

实际上，大家仔细看每张图上都能看见印戒样血管内皮细胞！！

 今天把这例片子给肿瘤医院的张仁元教授看过此例，他同意了上皮样血管内皮细胞瘤的诊断，其中多张图谱可以看见印戒样的血管内皮细胞。

今天做了CD31、calponin和CK广，有时间我会上传图片的。

倾向低度恶性肌纤维母细胞肉瘤

考虑：

）低度恶性肌纤维母细胞肉瘤；

2）平滑肌肉瘤；

3）上皮样血管内皮细胞瘤和上皮样血管肉瘤。

 上皮样血管内皮瘤多呈多结节镜下形态，本例呈弥漫性生长。需要标记CD31和Fli-1鉴别是否有血管内皮表型。F8一般是多抗，单一F8的结果难以评价。

细胞形态表现为纤维母/肌纤维母细胞形态。SMA阳性这么强，而MSA和Desmin均为阴性，提示是肌纤维母细胞表型。

理论上，血管内皮将不会表达SMA强阳性。所以，再做内皮标记的意义可能不大。

增加标记caldesmon。排除肌源性。

本例考虑为纤维母/肌纤维母细胞病变，需要仔细观察切片，鉴别：

1）结节性筋膜炎（本例图片中粘液样间质内有片区红细胞漏出现象）；增生性筋膜炎/增生性肌炎；

2）低度恶性肌纤维母细胞肉瘤（属于中间性肿瘤）；

3）假肉瘤性肌纤维母细胞增生性病变。

 掌跖纤维瘤病

 上皮样血管内皮细胞瘤和上皮样血管肉瘤都属于一个肿瘤谱系当中；该例在这个谱系的当中，两头分别是 上皮样血管内皮细胞瘤和上皮样血管肉瘤。

我也曾经考虑够血管肌周细胞瘤，但是核分裂高达10/HPF,应该是个恶性或低度恶性的肿瘤；

本例免疫组化：SMA+、MSA-、Desmin- 符合上皮样血管内皮细胞瘤表型，而肌周细胞瘤：SMA和MSA阳性，不符。

肌周细胞瘤

 目前我仍考虑为上皮样血管内皮瘤，CD34的特异性较差，所以，我今天做F8因子看看！