Abstract
Extra-mammary myofibroblastoma is a rare, benign spindle cell lesion mainly found in the axillary and inguinal regions. It strictly resembles its mammary counterpart with few reported cases occurring in the head and neck regions. We present a case of a middle aged woman with right sided anterior neck swelling. A diagnosis could not be made on tissue biopsy and the swelling persistently bothering the patient, thus surgical removal had been done under general anaesthesia. Histological examination showed spindle cells in clusters, with positive CD34 and desmin expression and also positive Rb1 expression. In myofibroblastoma Rb1 expression should be lost in majority of the cases. In view of histopathological dilemma, the sample was sent for fluorescence in situ hybridization (FISH) analysis. The final diagnosis of extra-mammary myofibroblastoma was made. This case is rare due to the unusual location of this tumour. Furthermore, we want to highlight the difficulties in differentiating between its malignant counterpart on pathological examination. Extra-mammary myofibroblastoma carry an excellent prognosis with low reoccurrence rate after surgical removal.