Rare Diseases Symptoms Automatic Extraction

Bone and Soft Tissue Pathology: SC02-3 ANGIOFIBROMA OF SOFT TISSUE.

[liposarcoma]

Angiofibroma of soft tissue arising in the thigh of a 54-year-old female will be demonstrated. Grossly, well circumscribed tumor showed grayish white color with focal gelatinous appearance on its cut surface. Histologically, the tumor is composed of oval to short spindle-shaped cells arranged in patternless pattern, accompanied by slit-like or hemangiopericytomatous vessels, fibro-collagenous or myxoid stroma. Immunohistochemically, tumor cells are negative for CD34, myogenic, myoepithelial or epithelial markers. INI1 expression is preserved. Characteristic AHRR-NCOA2 fusion transcript was detected by RT-PCR. This tumor is characterized by two components: uniformly proliferating bland spindle shaped cells in fibro-collagenous or myxoid stroma, and prominent vascular network composed of small, branching hemangiopericytomatous, and thin walled blood vessels. In this slide seminar detailed clinicopathological features of angiofibroma of soft tissue will be demonstrated. Moreover, the differential diagnosis of this peculiar tumor including cellular angiofibroma, solitary fibrous tumor, low-grade fibromyxoid sarcoma and low-grade myxofibrosarcoma or myxoid/round cell liposarcoma will be explained.