Synonyms
Malignant mixed tumor (not appropriate: see differential diagnosis)
Definition
Carcinoma ex pleomorphic adenoma (CEPA) is a carcinoma which arises from epithelial cells in pleomorphic adenoma (Barnes et al. 2005; Ellis and Auclair 2008). Clinically, CEPA presents in two forms: de novo or recurrent with in situ or infiltrative pattern. The clinical data and previous history of salivary (lacrimal) tumor are important in the diagnostic process.
Although CEPA belongs to high-grade malignancy, clinical prognosis depends on histological type of carcinoma arising in pleomorphic adenoma.
Clinical Features
Incidence
CEPA is a relatively common malignancy and represent approximately 2% of all salivary gland lesions and more than 6% of salivary gland carcinomas. It develops 12 years in average after a diagnosis of underlying pleomorphic adenoma.
Age
CEPA occurs at any age, but its peak of incidence is in the sixth and eighth decades of life.
Sex
There is a slight female-to-male...
References and Further Reading
Anand, A., & Brockie, E. S. (1999). Cytomorphological features of salivary duct carcinoma ex pleomorphic adenoma: Diagnosis by fine-needle aspiration biopsy with histologic correlation. Diagnostic Cytopathology, 20, 375–378.
Antony, J., Gopalan, V., Smith, R. A., & Lam, A. K. (2012). Carcinoma ex pleomorphic adenoma: A comprehensive review of clinical, pathological and molecular data. Head and Neck Pathology, 6, 1–9.
Barnes, L., Eveson, J. W., Reichert, P., & Sidransky, D. (Eds.). (2005). World Health Organization classification of tumours. Pathology and genetics of head and neck tumours. Lyon: IARC Press.
Ellis, G. L., & Auclair, P. L. (2008). Tumors of the salivary glands. AFIP atlas of tumor pathology. Fourth series, Fascicle 9. Washington, DC: American Registry of Pathology, ARP Press.
El-Naggar, A. K., Callender, D., Coombes, M. M., Hurr, K., Luna, M. A., & Batsakis, J. G. (2000). Molecular genetic alterations in carcinoma ex-pleomorphic adenoma: A putative progression model? Genes, Chromosomes & Cancer, 27, 162–168.
Ersöz, C., Cetik, F., Aydin, O., Cosar, E. F., & Talas, D. U. (1998). Salivary duct carcinoma ex pleomorphic adenoma: Analysis of the findings in fine-needle aspiration cytology and histology. Diagnostic Cytopathology, 19, 201–204.
Klijanienko, J., El-Naggar, A. K., Servois, V., Rodriguez, J., Validire, P., & Vielh, P. (1998). Mucoepidermoid carcinoma ex pleomorphic adenoma: Nonspecific preoperative cytologic findings in six cases. Cancer, 84, 231–234.
Klijanienko, J., El-Naggar, A. K., & Vielh, P. (1999a). Fine-needle sampling findings in 26 carcinoma ex pleomorphic adenomas: Diagnostic pitfalls and clinical considerations. Diagnostic Cytopathology, 21, 163–166.
Klijanienko, J., El-Naggar, A. K., Servois, V., et al. (1999b). Histologically similar, synchronous or metachronous, lacrimal salivary-type and parotid gland tumors: A series of 11 cases. Head & Neck, 21, 512–516.
Nigam, S., Kumar, N., & Jain, S. (2004). Cytomorphologic spectrum of carcinoma ex pleomorphic adenoma. Acta Cytologica, 48, 309–314.
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Klijanienko, J., Vielh, P. (2017). Carcinoma ex Pleomorphic Adenoma, Cytological Findings. In: Schmitt, F. (eds) Cytopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-33286-4_914
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DOI: https://doi.org/10.1007/978-3-319-33286-4_914
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