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- B1422鼻腔肿块-上海市骨与软组织读片2013(8)上交大附属市一医院提供
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图4
| 性别 | 男 | 年龄 | 34岁 | 临床诊断 | 左鼻腔肿块 |
|---|---|---|---|---|---|
| 一般病史 | 发现左鼻腔肿块3月余。 | ||||
| 标本名称 | 鼻腔肿块活检 | ||||
| 大体所见 | 不规则组织1块，大小约0.8×0.6×0.6cm，表面可见黑色毛发，切面灰白，半透明，质中等。 | ||||
图片1、2、3分别为显微镜下低倍、中倍和高倍视野所见。
图4为市一南院外景。
标签：鼻腔 肿块
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本帖最后由 海上明月 于 2013-08-18 12:12:37 编辑
- 王军臣
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（左鼻腔）巨细胞血管纤维瘤。
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Giant cell angiofibroma：1、Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. 2、There was intense immunoreactivity for CD34.
巨细胞血管纤维瘤（Giant cell angiofibroma）鉴别于巨细胞纤维瘤在于强表达CD34，据说是孤立性纤维性肿瘤的一种特殊变异类型。
Ophthal Plast Reconstr Surg. 2009 Sep-Oct;25(5):402-4. doi: 10.1097/IOP.0b013e3181b39a15.
Giant cell angiofibroma, a variant of solitary fibrous tumor, of the orbit in a 16-year-old girl.
Source
Oncology Service and daggerPathology Department, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Abstract
A 16-year-old girl presented with diplopia and gradual-onset, painless proptosis of the left eye. Orbital CT showed a well-circumscribed, enhancing, extraconal mass in the superior orbit, and the surgical excision was performed. Histopathology was interpreted as capillary hemangioma. Five years later, her symptoms recurred, and she was referred to the Oncology Service, Wills Eye Institute. Repeat orbital MRI showed a well-defined, extraconal mass with loculated areas of enhancement in the left orbit superonasally. Complete surgical excision was performed. Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. There was intense immunoreactivity for CD34. After 20 months follow-up, there was no recurrence or development of metastasis. Giant cell angiofibroma, a variant of solitary fibrous tumor, is a rare orbital tumor that presents as a well-circumscribed, enhancing mass and can be found in children.
- 王军臣
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本帖最后由 jcw62 于 2013-08-07 08:10:52 编辑
1、巨细胞血管纤维瘤有2个特点：多核巨细胞和扩张的血管腔。可能与SFT有关。好发部位：眼睑、鼻泪管、泪囊（道）。
2、Gross description（Macroscopy）：Well-circumscribed with variable capsule, median 3 cm (larger in extra-orbital regions), may have hemorrhagic or cystic cut surface。
3、Micro description（Histopathology）：Cellular areas with bland round to spindle cells in myxocollagenous stroma with small-medium thick walled vessels and multinucleated giant stromal cells that line ectatic vascular spaces。
4、免疫组化：阳性：CD34, CD99, vimentin, variable bcl2；阴性：CD31, CD68, c-kit/CD117, muscle specific actin, S100, desmin。
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本帖最后由 jcw62 于 2013-08-04 00:35:41 编辑
病灶位于鼻腔，鳞状上皮下真皮层，肿瘤细胞为梭形或多边形，有异型性，可见明显核仁，间质为纤维组织，血管裂隙内可见少量多核巨细胞，免疫组化：Vimentin、CD34强阳性，KP-1梭形细胞阴性，S100、CD31、 Factor VIII、keratin、desmin、SMA 、HMB45均为阴性，符合巨细胞纤维母细胞瘤（Giant cell fibroblastoma）（ intermediate malignancy ）。主要鉴别诊断：肌周细胞瘤、血管肉瘤、巨细胞血管母细胞瘤、巨细胞血管纤维瘤、纤维组织细胞瘤等。肌周细胞瘤一般无多核巨细胞可以排除；血管肉瘤—
CD31、 Factor VIII均阴性可以排除；巨细胞血管母细胞瘤—可见类似未分化的间叶细胞围绕小血管分布；巨细胞血管纤维瘤—间质可见较多小-中等大小的厚壁血管，KP-1（-）。
