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We hereby describe an unusual case of a primary phyllodes tumor of the breast that metastasized to the vulva. The patient was a 55-year-old woman who was first diagnosed with phyllodes tumors of the breast in 05/2010. She was managed with wide excision of the tumor. One year later she had a recurrence in the right breast (04/2011) and three years later in the small bowel wall (05/2013). She was managed with excision of the tumors and she was commenced on chemotherapy. The patient presented to our institution with an anterior left vulvar mass in 08/2013. She was managed with wide local excision. All the previous excisions pathologies were requested from the outside institutions and reviewed at our center. The diagnosis of borderline phyllodes tumor MS-275 ic50 of the breast was confirmed in the 2010 and 2011 breast excisions. The 2011 breast excision showed more stromal overgrowth and higher stroma/ducts ratio than the 2010 excision with more infiltrative and irregular borders. However, there was no necrosis or sarcomatoid transformation noted. The small bowel wall excision shows frank high grade sarcoma without any specific morphologic differentiation. CD117 was performed to exclude gastrointestinal stromal tumor (although CD117 can be positive in up to 35% of phyllodes tumor) and it was negative. The gross examination of the vulvar mass shows a pink-tan lobulated soft tissue measuring 3.9 �� 2.9 �� 1.5?cm with overlying skin ellipse. The skin shows 2.6 �� 1.8?cm white to tan ulcerated area. Sectioning reveals Quinapyramine tan solid cut surface. The microscopic examination shows proliferation of pleomorphic neoplastic spindle cells with frequent mitotic figures and large atypical forms resembling the small bowel excision morphology. The current tumor is slightly less cellular than the metastatic sarcoma into small bowel which could be explained by the ongoing chemotherapy effect (Figure 1). Figure 1 H&E staining of phyllodes tumor. A large panel of immunohistochemical stains was performed to rule out other spindle cell neoplasms that click here can be seen in the anatomic site. The neoplastic tumor cells are positive for vimentin and negative for AE1/AE3, CK5/6, and CAM 5.2 immunostains. The tumor cells are negative for S100 (positive in melanoma), myogenin, MyoD1 (positive in spindle cell tumors of skeletal muscle differentiation), and SMA (positive in spindle cells tumor of smooth muscle differentiation). CD34 and CD117, which can be positive in phyllodes tumor up to 75% and 35%, respectively, were negative in the vulvar mass as well as the primary breast phyllodes tumor. The overall morphologic features combined with the clinical history and immunohistochemical stains are diagnostic of high grade spindle cell sarcoma and consistent with the clinical history of phyllodes tumor from a breast primary. 3.