Introduction: Malignant mixed tumor (MMT) is an exceedingly rare cutaneous adnexal carcinoma with a significant risk for aggressive behavior and a propensity for metastasis. This tumor occurs in a wide age range and is twice as common in woman than in men. MMT shows a predilection for the trunk and the extremist’s foremost hands and feet. MMT may be confused clinically with many benign and malignant lesions. Therefore the histopathological and immunohistochemical examination is required for the correct diagnosis and treatment. Purpose: To present a case of MMT localized in the left toe with a special attention focused on its histopathology and differential diagnosis. Case presentation: A 56-year-old female was admitted with a nodular lesion on the left toe. A painless mass had been presented for one year with a significant increase in size within the past three month. The patient underwent wide surgical excision. Histopathologically for the lesion was diagnosed it as a malignant mixed tumor. Immunohistochemical examination revealed positive reaction for cytokeratin AE1/AE3/PCK26, vimentin and S-100. Histochemical reaction for PAS in the chondromyxoid tumor stroma was negative. Conclusion: Correct histopathological diagnosis and complete excision with wide disease-free margins before metastasis result in MMT free survival.
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