What causes true malignant mixed tumor (carcinosarcoma)?

Updated: Dec 01, 2015
  • Author: M Sherif Said, MD, PhD; Chief Editor: M Sherif Said, MD, PhD  more...
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No clear etiology is known. However, accumulation of genetic mutations could be a factor. A history of exposure to irradiation of pleomorphic adenoma has been reported. [11, 17] The reported interval between the irradiation and the onset of carcinosarcoma varies from 1 to 36 years.

Two schools of thought exist as to the origin of carcinosarcomas. The convergence hypothesis maintains that multiclonal stem cells of the epithelial and mesenchymal components play a causative role. The divergence hypothesis postulates a monoclonal origin from a single totipotent stem cell with divergent differentiation. [18, 19] The latter hypothesis is more favored.

Gotte et al hypothesized that the tumor originates from a myoepithelial cell precursor. [20] Other investigators have postulated that the tumor originates from inner ductal cells [5] or a pluripotent primitive cell. [14]

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