We highlight the importance of keeping this rare tumor in mind and the early detection of symptoms because such a malignant transformation could occur in pleomorphic adenomas, with the. We report the case of a 62yearold male who presented with a swelling over his left temple. Pleomorphic adenoma pa, the most frequent tumour in salivary glands, can undergo malignant transformation. Our goal was to investigate the prognosis of cxpa in the major salivary glands and factors influencing it.
Within the background of pleomorphic adenoma, the carcinoma cells sometimes replaced ductal luminal cells while retaining an intactlike myoepithelial layer. Carcinoma ex pleomorphic adenoma of the lacrimal gland. The current definition of ca ex pa became widely accepted in the second half of the twentieth century. Ex pleomorphic adenoma is a malign neoplasm of difficult to diagnose. Pleomorphic adenoma is the most common salivary gland neoplasm, constituting up to 60% of all benign salivary gland tumors, regardless of major or minor tumors.
The use of strict pathological criteria may underestimate the frequency of carcinoma ex pleomorphic adenoma because the malignant cells in some cases may. Carcinoma ex pleomorphic adenoma cxpa is an uncommon malignant tumor with highly aggressive biological behavior. Rare spectrum of carcinomas demonstrating abrupt transition from benign tumor usually pleomorphic adenoma. Histopathology remains the gold standard for the diagnosis of ca ex pa, with only a handful of cases reported on cytology.
Because of several highrisk factors for metastasis i. The clinical findings typical of this tumor include history of a slow growing. The chart showing pdf series, word series, html series, scan qr codes. Carcinoma ex pleomorphic adenoma is an epithelial malignant neoplasm arising from a primary or recurrent pleomorphic adenoma and is a diagnostic challenge for cytopathologists. Carcinoma ex pleomorphic adenoma is the most common variety of mixed malignant tumor where carcinoma develops from the epithelial elements of a preexisting pleomorphic adenoma. Usually defined as carcinoma that develops in association with benign primary or recurrent pleomorphic adenoma rare spectrum of carcinomas demonstrating abrupt transition from benign tumor usually pleomorphic adenoma. The proposed criteria for defining carcinoma ex pleomorphic adenoma by nagao et al.
Diffusionweighted images demonstrated well complex tissue. Ca ex pa has been named as carcinoma ex mixed tumour, carcinoma ex adenoma, and carcinoma ex benign pleomorphic adenoma. Pathology of true malignant mixed tumor carcinosarcoma. They are benign except for a rare carcinoma ex pleomorphic adenoma but are not always completely encapsulated and show a definite tendency to recur after excision, in which case recurrence represents local growth because of incomplete excision and not malignancy. Meanwhile, the imaging findings of carcinoma ex pleomorphic adenoma are often nonspecific and are. The other 2 forms are carcinoma ex pleomorphic adenoma and metastasizing benign pleomorphic adenoma by definition, carcinosarcoma is a biphasic malignant tumor with a carcinomatous component and a sarcomatous component.
Pleomorphic adenomas are the commonest tumors of the salivary glands. This case report documents the clinical, radiological and histopathological. Carcinoma expleomorphic adenoma cxpa is a rare malignant tumor that affects the salivary glands, corresponding to 3. Mutation and overexpression of tp53 are also frequent events in carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma ca ex pa is a rare malignant transformation of a benign primary pleomorphic adenoma pa. Pineal and leptomeningeal metastases from a parotid. Pleomorphic adenoma is the most common salivary gland tumor and accounts for approximately 80% of parotid tumors. Carcinoma ex pleomorphic adenoma is an uncommon malignant salivary gland tumor with high aggressive behavior and poor prognosis 3. Carcinoma ex pleomorphic adenoma affects glandular structures, occurring mostly in major salivary glands and less commonly in the lacrimal gland. The proper examination, a complete physical examination and request imaging studies are very important in patients with suspect of parotid mass to avoid the malignant. A case of carcinoma ex pleomorphic adenoma arising from. Prognostic factors affecting the clinical outcome of. Ductal adenocarcinoma ex pleomorphic adenoma of the. Only three cases of cxpa of the trachea have been reported in the literature.
Pleomorphic adenoma surgery, symptoms, causes, treatment. We report a case of carcinoma ex pleomorphic adenoma in a patient presenting with left facial. A preexisting benign tumour cannot be distinguished so it is designated as carcinoma ex pleomorphic adenoma. These tumors occasionally undergo malignant transformation, with potentially devastating consequences. In carcinoma ex pa, the recommended therapy is wide local excision with lymph node dissection, followed by radiation therapy for widely invasive tumors. This tumor develops in primary or recurrent pleomorphic adenoma. Most malignant mixed tumors are carcinoma ex pleomorphic adenoma, whereas the latter 2 types are extremely unusual. The current definition of ca ex pa became widely accepted in the second half of the.
Maspin expression in carcinoma ex pleomorphic adenoma. Pdf carcinoma ex pleomorphic adenoma ca ex pa is a malignant neoplasm arising from primary or recurrent benign pleomorphic. Transformation to malignancy, carcinoma ex pleomorphic adenoma. In 3 of the 4 cases, diffusionweighted and apparent diffusion coefficient adc mapping images clearly revealed carcinoma as a hypercellular area with low adc values and pleomorphic adenoma as a hypocellular area with high adc values.
Similar tumor arising in a benign mixed tumor has only been reported once. Carcinoma ex pleomorphic adenoma is a malignant tumor arising from a transformed benign mixed tumor or in a patient who has undergone previous resection. Pa is a rare epithelial malignancy arising from primary or recurrent pa. Historical reports list 5year survivals ranging from 30% to 70% 4. Despite of that, ca ex pa is uncommon and has a prevalence rate of 5. Diagnosis requires that elements from the benign pleomorphic adenoma and the malignant component need to be seen. Primary pulmonary pleomorphic adenoma is a very rare benign tumor that tends to arise in the large airways tumors tend to show small, branching, double layered ductules rather than ducts and less cartilaginous stroma as compared to pleomorphic adenomas arising in the salivary gland.
Effective chemotherapy for submandibular gland carcinoma ex. Pleomorphic adenoma an overview sciencedirect topics. Invasive salivary duct carcinoma ex pleomorphic adenoma of. The typical clinical presentation in a patient with carcinoma ex pleomorphic adenoma is a longstanding history average, 1015 years of pleomorphic adenoma and a sudden period of rapid growth average, 36 months. Carcinoma ex pleomorphic adenoma of the parotid gland. Carcinoma ex pleomorphic adenoma diagnostic histopathology. Carcinoma ex pleomorphic adenoma ca ex pa is a type of cancer typically found in the parotid gland. The prognosis of carcinoma ex pa depends on its extension. A case of squamous cell carcinoma ex pleomorphic adenoma in a palate is presented and comments on diagnostic criterias are described.
Report of three cases suggesting a relationship to metaplastic carcinoma of matrixproducing type received. Malignant pleomorphic adenoma, salivary gland tumors. The most common benign salivary tumour is a pleomorphic adenoma. Cns metastases of carcinoma ex pleomorphic adenoma of the. Malignant degeneration of an adenoma increases from 1. Carcinoma ex pleomorphic adenoma of the maxillary sinus. Carcinoma ex pleomorphic adenoma is rare and comprises approximately 3.
Etiology carcinoma ex pleomorphic adenoma of salivary gland develops from a preexisting pleomorphic adenoma that is present for a long duration typically over 3 years. It arises from the benign tumour pleomorphic adenoma its prognosis depends on the stage. Carcinoma expleomorphic adenoma cxpa is a rare, aggressive, poorly understood malignancy of the salivary gland. Carcinoma ex pleomorphic adenoma, an uncommon neoplasm of the parotid gland, accounts for less than 4% of salivary gland tumors. It originates from a previous pa, usually in the 6th and 7th decades of life, as a slow. The patient was 36yearold male presenting with an ovoid. Though it is classified as a benign tumor, pleomorphic adenomas have the capacity to grow to large proportions and may undergo malignant transformation, to form carcinoma expleomorphic adenoma, a risk that increases with time 9. Regional metastasis is common and mortality is high4. Invasive myoepithelial carcinoma ex pleomorphic adenoma of. Primary carcinoma ex pleomorphic adenoma, tracheobronchial system, primary salivary glandtype tu mors, pleomorphic adenoma.
More importantly, they have a proven risk 515% of undergoing malignant transformation carcinoma ex pleomorphic adenoma, or malignant mixed tumor 3. It is researched that several genetic factors play a role in their formation. Carcinoma ex pleomorphic adenoma cepa is an uncommon complication of an untreated pleomorphic adenoma pa, but one that has a lifethreatening significance. What are the causes of carcinoma ex pleomorphic adenoma of salivary gland. Usually defined as carcinoma that develops in association with benign primary or recurrent pleomorphic adenoma. Carcinoma ex pleomorphic adenoma accounts for 11% of all salivary malignancies 1.
Therefore, we finally made a diagnosis of invasive salivary duct carcinoma ex pleomorphic adenoma. Cytomorphologic spectrum of carcinoma ex pleomorphic. Carcinoma ex pleomorphic adenoma is defined as a pleomorphic adenoma from which derived an epithelial malignancy. Superficial parotid gland resection was performed in 20.
Case report open access invasive salivary duct carcinoma. Cellular proliferation index between department of. Carcinoma ex pleomorphic adenoma an overview sciencedirect. Imaging revealed a lytic lesion over the left orbital wall with soft tissue extension suggestive of malignancy. He underwent an en bloc resection of the mass with orbital. Only a few cases of carcinoma ex pleomorphic adenoma arising in the. The authors present a case of carcinoma ex pleomorphic adenoma. We highlight the importance of keeping this rare tumor in mind and the early detection of symptoms because such a malignant transformation could occur in pleomorphic adenomas, with the proportion.
The tumour often arises from the background of a recurrent pleomorphic adenoma and can be composed of various histologic subtypes, including highgrade salivary duct carcinoma, adenocarcinoma not otherwise specified, myoepithelial. We present this as a unique account of a primary parotid gland carcinoma, arising from myoepithelial cells, without a known precursor lesion, in a 28yearold woman. Carcinoma ex pleomorphic adenoma arises from pleomorphic adenoma in a multistep process with genetic alterations of, for example, plag1, hmga2, or mdm2. It believed that the inhibitory effects on cell divi. Case report primary carcinoma ex pleomorphic adenoma of the. Carcinoma ex pleomorphic adenoma ca ex pa is a malignant neoplasm arising from primary or recurrent benign pleomorphic adenoma. Case report primary carcinoma ex pleomorphic adenoma of. Different patterns of malignant change occur in pleomorphic adenoma, of which true malignant mixed tumor carcinosarcoma is one form. Benign mixed tumors bmts, is a benign or a noncancerous growth in the salivary gland. Carcinoma ex pleomorphic adenoma cxpa is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma. Although rare, this condition has been reported to be responsible for 4. The continued clinical evaluation and longterm followup seems to be of utmost importance for better diagnosis and treatment of. The difference in the clinical features between carcinoma.
It arises from a benign pleomorphic adenoma presenting in the sixth to eighth decades of life. However, there is evidence to support the more conservative approach of. Immunohistochemical expression of p21 in normal tissues of. A large pleomorphic adenoma can be disfiguring or exert mass effect on important structures. This case report presents the clinical and radiographic features of a rare case of biopsy proved brain and spinal cord metastases arising from carcinoma ex pa of the. Pleomorphic adenomas pas, also known as benign mixed tumors, are common tumors of the parotid gland. We present 4 cases of carcinoma ex pleomorphic adenoma of the parotid gland. Pdf pineal and leptomeningeal metastases from a parotid. We should be aware that owing to its characteristic. We report a case of salivary duct carcinoma arising from a pleomorphic adenoma ex pleomorphic adenoma in a parotid salivary gland of a 70yrold man.