Department of Pathology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Mod Pathol. 2011 Jan;24(1):82-9. doi: 10.1038/modpathol.2010.170. Epub 2010 Sep 17.
Cellular angiofibroma represents a rare benign mesenchymal tumor, occurring mainly in the superficial soft tissue of the genital region. The involvement of 13q14 in some cases confirmed the morphological suggested link with spindle cell lipoma and mammary-type myofibroblastoma. We analyzed the clinicopathological and immunohistochemical features of 25 cases, and performed in a number of cases additional molecular studies. There were 17 female and 8 male patients (age ranged from 27 to 83 years); females tended to be younger. A marked predilection for the vulva (n=13) was observed, and neoplasms in males were predominantly located in the inguinal region (n=4), and one case each in the scrotum, perianal, the knee, and the upper eyelid. The tumors arose most commonly in the superficial soft tissue and were well circumscribed in all but two cases. The tumor size ranged from 1 to 9 cm. All lesions were composed of spindle-shaped cells associated with numerous small- to medium-sized blood vessels; however, a broad morphological variation with foci of lipogenic differentiation in nine cases and sarcomatous transformation in one case was found. By immunohistochemistry, 11 out of 22 cases expressed CD34. A focal reaction for α-smooth muscle actin was observed in 9 out of 22 cases, and two cases each stained weak and focally positive for epithelial membrane antigen and CD99. In all seven cases tested, a monoallelic deletion of RB1 was detected by FISH analysis. Follow-up, available in 14 patients, showed neither local recurrence nor metastasis. In conclusion, we affirm the link between cellular angiofibroma, spindle cell lipoma, and mammary-type myofibroblastoma, showing a spectrum of one entity with morphological variations dependent on anatomic location.
细胞性血管纤维瘤是一种罕见的良性间叶性肿瘤，主要发生于生殖器区域的表浅软组织。部分病例中 13q14 的累及证实了其与梭形细胞脂肪瘤和乳腺型肌纤维母细胞瘤在形态学上的联系。我们分析了 25 例病例的临床病理和免疫组化特征，并对一些病例进行了额外的分子研究。患者中有 17 名女性和 8 名男性（年龄范围为 27-83 岁）；女性倾向于更年轻。观察到明显的外阴偏好（n=13），而男性的肿瘤主要位于腹股沟区（n=4），还有 1 例位于阴囊、肛周、膝关节和上眼睑。肿瘤最常发生于表浅软组织，除 2 例外均边界清楚。肿瘤大小从 1 到 9 厘米不等。所有病变均由梭形细胞组成，伴有大量小至中等大小的血管；然而，在 9 例中发现了广泛的形态学变异，伴有脂肪分化灶，1 例发生肉瘤样转化。免疫组化方面，22 例中有 11 例表达 CD34。22 例中有 9 例α-平滑肌肌动蛋白有局灶性反应，2 例上皮膜抗原和 CD99 呈弱阳性和局灶性阳性。在所有 7 例进行检测的病例中，FISH 分析均显示 RB1 单等位基因缺失。在可获得随访的 14 例患者中，均未出现局部复发或转移。综上所述，我们证实了细胞性血管纤维瘤、梭形细胞脂肪瘤和乳腺型肌纤维母细胞瘤之间的联系，展示了一种具有形态学变异的实体，其形态学变异取决于解剖位置。
