Despite of that, ca ex pa is uncommon and has a prevalence rate of 5. Pleomorphic adenoma surgery, symptoms, causes, treatment. 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. Our case of salivary duct carcinoma ex pleomorphic adenoma of the palate is the first complete report, to our knowledge, in englishlanguage scientific studies. Carcinoma ex pleomorphic adenoma ca ex pa is a type of cancer typically found in the parotid gland. The proportion of benign versus malignant components can be variable. Cytomorphologic spectrum of carcinoma ex pleomorphic. A report has suggested that the median onset of pleomorphic adenoma is 10 years of age.
Prognostic factors affecting the clinical outcome of. Carcinoma ex pleomorphic adenoma caexpa of salivary gland is a malignancy of the major salivary glands mostly that arises from a pleomorphic adenoma it is generally observed in elderly adults. Carcinoma ex pleomorphic adenoma an overview sciencedirect. The parotid lymph node did not show any evidence of malignancy.
Carcinoma expleomorphic adenoma is exceedingly rare in sites other than the major salivary glands and the oral cavity. Carcinoma ex pleomorphic adenoma ca ex pa is a carcinoma arising from a primary or recurrent benign pleomorphic adenoma. Pleomorphic adenoma occurs commonly in the major salivary glands but is uncommonly encountered in the breast. Carcinoma ex pleomorphic adenoma cepa is defined as a pleomorphic adenoma from which an epithelial malignancy is derived 1. Among all salivary gland neoplasms, carcinoma ex pleomorphic adenoma ca ex pa accounts for approximately 3. We present the case of a 60yearold woman with a cxpa of the left parotid gland who required systemic therapy for locally recurrent disease. Pleomorphic adenoma definition of pleomorphic adenoma by. All tu mours showed areas of pleomorphic adenoma adjacent to typical areas of malignant transformation. The tumour cells demonstrate a squamoid appearance with abundant cytoplasm and form large dilated ducts containing central comedonecrosis. A 55yearold male patient presented with an asymptomatic mass of the upper lip that had noticed 8 years previously.
We present 4 cases of carcinoma ex pleomorphic adenoma of the parotid gland. The generally accepted frequency for malignant transformation of a pleomorphic adenoma is 57 % age. Pleomorphic adenomas pas are the most common benign tumors arising in salivary glands and their malignant transformation to carcinoma ex pleomorphic adenomas cxpas accounts for between 4. Metastatic pleomorphic adenoma to the supraspinatus muscle. A case of carcinoma ex pleomorphic adenoma arising from. The epidemiology of pleomorphic adenoma can cover all ages and both genders but is more prevalent among women from thirty to sixty years of age. Pleomorphic adenoma shows malignant changes during its natural history and occurs in three forms, i. On histopathological examination, the tumor comprises a wide morphological spectrum with a variable proportion between the benign and malignant components with the latter often predominating and overlapping the pa, which may cause misdiagnosis. We present the case of a 58yearold man who was referred to our hospitals oral and maxillofacial surgery clinic for evaluation of an exophytic mass of the right palate. Carcinoma ex pleomorphic adenoma is a highgrade salivary gland carcinoma in which malignant transformation occurs in a previously benign pleomorphic adenoma.
Carcinoma ex pleomorphic adenoma of the palate composed of. Carcinoma ex pleomorphic adenoma of the salivary glands. Ca ex pa has been named as carcinoma ex mixed tumour, carcinoma ex adenoma, and carcinoma ex benign pleomorphic adenoma. The final diagnosis of myoepithelial carcinoma expleomorphic adenoma was made. Carcinoma ex pleomorphic adenoma, abbreviated ca ex pa, is a rare malignant salivary gland tumour that arises from a pleomorphic adenoma. The average age of this tumor is 10 years later as the average age occurrence of the benign mixed tumor. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtypes are possible. Superficial parotid gland resection was performed in 20. When recurrence and distant metastasis occur, survival is so low. Carcinoma expleomorphic adenoma of parotid radiology. It derives its name from the architectural pleomorphism variable. Caexpa, carcinosarcoma and metastasizing carcinoma. Pathology outlines carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma arises from pleomorphic adenoma in a multistep process with genetic alterations of, for example, plag1, hmga2, or mdm2.
Carcinoma ex pleomorphic adenoma in a minor salivary gland. Pleomorphic adenoma synonyms, pleomorphic adenoma pronunciation, pleomorphic adenoma translation, english dictionary definition of pleomorphic adenoma. Pa is a rare epithelial malignancy arising from primary or recurrent pa. Because of several highrisk factors for metastasis i. Carcinoma ex pleomorphic adenoma is an uncommon malignant salivary gland tumor with high aggressive behavior and poor prognosis 3.
Carcinoma ex pleomorphic adenoma of the sublingual gland. Carcinoma ex pleomorphic adenoma affects glandular structures, occurring mostly in major salivary glands and less commonly in the lacrimal gland. Metastasizing pleomorphic adenoma in the multiple organs. Thoracic intramedullary spinal metastasis from carcinoma. It arises from the benign tumour pleomorphic adenoma its prognosis depends on the stage. From the department of pathology, university of texas, m.
Only a few cases of carcinoma ex pleomorphic adenoma arising in the submandibular gland have ever been reported, all with a poor prognosis. Pleomorphic adenoma, the most common tumor of the salivary glands, is usually benign. These pathologies, which should not be confused, are the carcinoma ex pleomorphic adenoma. The first two types are generally treated with surgical resection and radiation, and carry a fair prognosis. In the 1991 who classification of tumours of the salivary glands, the term malignant mixed tumour comprises three different pathologies. Carcinoma ex pleomorphic adenoma can be classified as noninvasive, minimally invasive, or widely invasive 2.
Abstract three cases of carcinoma expleomorphic ad enoma of the breast are reported. It often poses a diagnostic challenge to clinicians and pathologists. This tumor most often develops in the parotid gland, where it represents 2% to 3% of all parotid neoplasms. The pleomorphic adenoma is the most common tumour of glandular origin in the head and neck 1. Effective chemotherapy for submandibular gland carcinoma ex. Invasive salivary duct carcinoma ex pleomorphic adenoma of. The authors present the cytologic and clinical findings for six examples of these rare tumors. 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. 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. Squamous cell carcinoma scc as the malignant component of carcinoma ex pleomorphic adenoma cxpa occurring in upper lip is rare. Carcinoma ex pleomorphic adenoma of the parotid gland ajnr. Carcinoma ex pleomorphic adenoma cxpa is a rare and poorly understood malignancy that develops from either a longstanding primary or a recurrent pleomorphic adenoma pa.
Carcinoma ex pleomorphic adenoma of the sublingual gland is extremely rare and frequently misdiagnosed. We present the first two histopathologically documented examples to occur in the nasal cavity. Regional metastasis is common and mortality is high4. The clinical findings typical of this tumor include history of a slow growing. Carcinoma ex pleomorphic adenoma is the most common of three malignant mixed tumors of salivary glands, and are thought to arise from preexisting pleomorphic adenomas or benign mixed tumors 1. Files are available under licenses specified on their description page. Our goal was to investigate the prognosis of cxpa in the major salivary glands and factors influencing it.
Pleomorphic adenomas pas, also known as benign mixed tumors, are common tumors of the parotid gland. This case report presents the clinical and radiographic features of a rare case of biopsy proved brain and spinal cord metastases. Invasive carcinoma is wellcircumscribed with a pushing border. There are 3 distinct histologic types of malignant mixed tumor. Carcinoma ex pleomorphic adenoma is the most common variety of mixed malignant tumor where. Carcinoma ex pleomorphic adenoma article about carcinoma. Different patterns of malignant change occur in pleomorphic adenoma, of which true malignant mixed tumor carcinosarcoma is one form.
Pathology of true malignant mixed tumor carcinosarcoma. Carcinoma expleomorphic adenoma cxpa is a rare, aggressive, poorly understood malignancy of the salivary gland. Sections of parotid gland show a salivary duct carcinoma with evidence of intermixed pleomorphic adenoma carcinoma ex pleomorphic adenoma. More rarely pleomorphic adenomas can metastasize without histological malignant transformation. The objective of this study was to investigate the clinicopathologic features of carcinoma ex pleomorphic adenoma cxpa and comprehensively. Cns metastases of carcinoma ex pleomorphic adenoma of the. An intracapsular carcinoma ex pleomorphic adenoma with lung metastases composed exclusively of benign elements. Carcinoma ex pleomorphic adenoma radiology reference. In both of these locations, the tumor is typically grossly circumscribed and has a mixed histological appearance, being composed of myoepithelial and epithelial components amid a myxochondroid matrix. Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of parenchymatous glandular cells along with myoepithelial components, having a malignant potentiality. The cause of formation of this salivary gland malignancy is believed to be due to genetic factors.
All structured data from the file and property namespaces is available under the creative commons cc0 license. Among salivary gland tumors, cxpa is unique and interesting because it is histologically diverse and contains both benign pleomorphic adenoma and carcinomatous components. Surgical resection is curative, but approximately 6% of these tumours transform into carcinoma ex pleomorphic adenoma cxpa, also known as mixed malignant tumour 1. It is well known, however, that pleomorphic adenomas occasionally undergo malignant transformation to carcinoma ex pleomorphic adenoma and can metastasize.
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. These tumors occasionally undergo malignant transformation, with potentially devastating consequences. Carcinoma ex pleomorphic adenoma cxpa is an aggressive, poorly understood salivary gland malignancy. Carcinoma ex pleomorphic adenoma of the lacrimal gland.
We presen 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 dependent on the duration of the tumor. Preoperatively, a diagnosis of pleomorphic adenoma of the parotid gland metastasizing to the cervical lymph node was made, but postoperatively it was reported. In contrast to most major salivary gland malignancies, mucoepidermoid carcinoma has been rarely documented and tentatively accepted as carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma of the parotid gland. Pleomorphic adenoma, also referred to as benign mixed tumors bmts, is a benign or a noncancerous growth in the salivary gland, the most commonly occurring parotid tumor. The diagnosis of cxpa is based on the coexistence of epithelial malignancy with histologically benign pa. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. Invasive myoepithelial carcinoma ex pleomorphic adenoma of. The difference in the clinical features between carcinoma ex. Salivary duct carcinoma ex pleomorphic adenoma of the. Carcinoma ex pleomorphic adenoma was observed at higher rates in the minor salivary glands 24. Dna aneuploidy as a topographic malignant transformation. Meanwhile, the imaging findings of carcinoma ex pleomorphic adenoma are often nonspecific and are. Carcinoma ex pleomorphic adenoma cxpa is an uncommon malignant tumor with highly aggressive biological behavior.
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