Several histopathologic variants of cutaneous leiomyomas have been recognized. In our consultation dermatopathology practice, we encountered a variant of cutaneous leiomyoma which, to our knowledge, has not been reported.

We report 3 cases of vascular leiomyomas, all of them manifesting prominent intratumoral calcifications dominating over the residuum of the tumors and occurring on the acral sites.

We conducted a clinicopathological and immunohistochemical study, which is complemented by a literature review.

All 3 patients were women ranging in age from 57 to 72 years. Each presented clinically with a slowly growing, firm mass. The lesion was painful in two cases. None of the patients had renal disease or endocrine abnormalities. Microscopically, the lesions were a well-circumscribed, non-encapsulated neoplasm composed of mature smooth muscle cells and vascular pattern, which was inconspicuous, but focally dilated blood vessels were present. In all cases, prominent calcifications were present. Immunohistochemically the spindle cells were positive for ⍺-smooth muscle actin.

This study utilizes tissue specimens that all came as consultations; therefore, some inherent selection bias exists.

Acral calcified vascular leiomyoma is a rare clinicopathological variant of leiomyoma which has predilection for acral sites and shows massive calcifications prevailing over the tumor itself.