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！

 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。

 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！！

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。

考虑低度恶性的血管平滑肌肉瘤。

倾向低度恶性肌纤维母细胞肉瘤

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.

1、纤维瘤病

2、低度恶性肌纤维母细胞肉瘤

3、平滑肌肉瘤

学习

1、纤维瘤病：细胞学形态相对温和，细胞异型性小，细胞的密度也不大，MSA也应该表达，本例不是纤维瘤病；

2、低度恶性肌纤维母细胞肉瘤：细胞呈梭形，常常浸润到周围软组织中形成棋盘样结构；

3、平滑肌肉瘤：Desmin和SMA均为阳性才对

 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！

血管源性肿瘤

肌周细胞瘤

 形态特点：

见较多的血管，血管的内皮细胞肿胀，血管周围的细胞呈上皮样（不知道是什么细胞），核分裂易见，5/10hpf，可见单个红细胞，

酶标：Ki67：20%+；SMA++； MSA-；Desmin-；CD34：血管阳性，肿瘤细胞阴性；

诊断考虑：

1、上皮样血管瘤：血管呈上皮样，多有淋巴细胞和嗜酸性粒细胞浸润；本例未见炎细胞浸润，核分裂也不应该见到；

2、上皮样血管内皮细胞瘤：单个内皮细胞呈印戒样，口含红珠（单个红细胞）；本例CD34血管阳性，肿瘤细胞阴性；本例如果是上皮样血管              内皮细胞瘤的话，就是恶性上皮样血管内皮细胞瘤（罕见）

3、多形性血管内皮细胞瘤(pseudomyogenic haemangioendothelioma)：核分裂罕见，本例不像。

目前：补做ck广和CD31

(ˇˍˇ） 想听听大家的意见，非常感谢！

 患者无明显外伤史，发现右手掌肿块一个月；部位较浅；

取材时，见切面较嫩，鱼肉感。

谢谢版主这么快回答！

等补做的免疫组化出来后一起上传酶标结果！图15能看出明显的血管的结构

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。

 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。

 掌跖纤维瘤病

 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。