Primary cutaneous vascular leiomyosarcoma: A rare subtype of leiomyosarcoma of the skin K E Y W O R D S : cutaneous leiomyosarcoma, leiomyosarcoma, skin neoplasm, superficial leiomyosarcoma, vascular leiomyosarcoma Primary smooth-muscle malignancies in the skin account for approximately 2% to 3% of all soft tissue sarcomas. 1,2 We read with interest a recent JCP report on a vascular leiomyosarcoma arising from vena saphena magna. 3 We report herein a case of primary cutaneous vascular leiomyosarcoma arising from a small-caliber dermal vein.A 59-year-old male presented with a 6-month history of a slowlyThe prognosis of leiomyosarcoma is associated with depth of tumor invasion, tumor size, distal location, and histological grade. 1,5,12 Cutaneous leiomyosarcoma is considered a low-grade malignancy because of its low risk of aggressive behavior, which led some authors to propose the term "atypical intradermal smooth neoplasm." 13 An excellent outcome has been reported after surgical excision with 1-cm resection margins. 6,14 In contrast to cutaneous leiomyosarcoma, subcutaneous leiomyosarcoma is associated with a poorer prognosis, bearing higher rates of recurrence and a tendency to metastasize earlier, 5,12 probably because its usually larger size and deeper extension make a complete resection less likely. In contrast, cases of superficial cutaneous vascular leiomyosarcoma like ours, smaller in size and more accessible in location, are favorable to an early wide excision.However, its vascular origin would theoretically allow for early hematogeneous metastisation. In sum, although the prognosis of superficial vascular leiomyosarcoma is uncertain, a good clinical outcome is expected.In conclusion, this case of primary vascular leiomyosarcoma shows that cutaneous leiomyosarcoma, which generally arises from the arrector pili muscle, on extremely rare occasions, may also have a vascular origin. A high index of suspicion for malignancy is paramount in middle-aged patients with cutaneous nodules arising de novo. A pathological diagnosis of clinically non-specific and inconspicuous lesions should be sought because early surgical intervention may avoid recurrence, metastasis, and death.