Background A 15-year-old Caucasian male presented with 9-month history of a recurrent nodule on the nasal columella. The previous biopsy was reported as a neurofibroma.
Methods Frozen sections revealed a spindle cell neoplasm. Permanent section immunohistochemistry sections stained positive for vimentin and smooth muscle actin and negative for S100 and CD34, confirming the diagnosis of leiomyosarcoma.
Results The tumor was removed using Mohs micrographic surgery. Radiological work-up revealed no distant metastasis. There has been no local recurrence to date.
Conclusions Leiomyosarcoma is a difficult diagnosis to make clinically and requires histological confirmation. Re-biopsy of a “benign” growth may be necessary if clinicopathological correlation does not match with the clinical behavior of the tumor in question. Finally, Mohs micrographic surgery is a useful treatment modality for leiomyosarcomas, particularly those located in cosmetically-sensitive regions of the body such as the nose.