Malignant Eccrine Acrospiroma of the Scalp-Case Report and Literature Review

Abstract

Background:Malignant eccrine acrospiroma (MEA) is a rare malignancy of sweat glands, mostly arising from face, trunk and extremities. MEAs arising from scalp are very rare.Aim and objective:We report a rare case of MEA arising from the scalp and discuss the treatment and outcome.Material and Methods:A 43-year-old man with Sjogren's syndrome and polyarthritis presented with a 10-year history of a painless slow-growing tumor on his right occipital area. After excisional biopsy, pathology confirmed malignant eccrine acrospiroma. Due to narrow resection margin, wide excision with neck dissection was done and the scalp defect was reconstructed with a free rectus femoris muscle flap and split-thickness skin graft. Post-operative radiation therapy was done.Results:There was no evidence of local recurrence and distant metastasis at the one-year follow-up.Conclusion:Malignant eccrine acrospiroma is rarely seen on the scalp. There's no known risk factor for this malignancy. Wide excision of MEA is the treatment of choice. Adjuvant chemotherapy or radiotherapy have been controversial. The prognosis of MEA is poor if frequent local recurrence or metastasis was noted. Early detection and radical tumor resection can promote survival.