Case report of angiomyofibroblastoma in a patient with lynch syndrome

Lynch syndrome (hereditary non-polyposis colorectal cancer) is a cancer predisposition caused by mutations in mismatch repair genes. Carcinoma of the colon, endometrium, ovary and urothelium are most common. Soft tissue tumours not typically associated with syndrome, however, sarcoma has rarely been reported. We present case vulval angiomyofibroblastoma patient known syndrome. The 75-year-old female who presented lump clinically presumed to be lipoma. Histological examination showed partially encapsulated spindle cell lesion small vessels abundant mature adipocytes. No cytological atypia, mitotic activity or necrosis were identified. Lesional cells expressed ER, PR, desmin (focal), BCL2 CD99, negative for SMA, CD34 S100. Lipomatous rare morphological variant which no special prognostic significance, but pathologists should aware this entity avoid misdiagnosis as an adipocytic tumour. differential diagnosis includes aggressive angiomyxoma, liposarcoma, lipoma, superficial angiomyxoma cellular angiofibroma. To our knowledge, first reported Further research required determine whether chance association manifestation