Cytological features of carcinoma ex pleomorphic adenoma of. It arises from a benign pleomorphic adenoma presenting in the sixth to eighth decades of life. 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. 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. Aug 12, 2018 carcinoma ex pleomorphic adenoma ca ex pa 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. 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.
Among salivary gland tumors, cxpa is unique and interesting because it is histologically diverse and contains both benign pleomorphic adenoma and carcinomatous components. Find out information about carcinoma ex pleomorphic adenoma. There are 3 distinct histologic types of malignant mixed tumor. 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. A case report hengxing gao, quan li, wenli chang, yalong zhang, xiaozhi wang, xuexue zou hengxing gao, xiaozhi wang, respiratory department, binzhou medical university hospital, binzhou 256603, shandong province, china.
We report a case of carcinoma ex pleomorphic adenoma in a patient presenting with left facial. The mass was clinically suspected to be a benign salivary gland tumor based on palpation and magnetic resonance imaging. Carcinoma ex pleomorphic adenoma, an uncommon neoplasm of the parotid gland, accounts for less than 4% of salivary gland tumors. Carcinoma ex pleomorphic adenoma arises from pleomorphic adenoma in a multistep process with genetic alterations of, for example, plag1, hmga2, or mdm2. Despite of that, ca ex pa is uncommon and has a prevalence rate of 5. The tumour cells demonstrate a squamoid appearance with abundant cytoplasm and form large dilated ducts containing central comedonecrosis.
Ductal adenocarcinoma ex pleomorphic adenoma of the lacrimal. Carcinoma expleomorphic adenoma cxpa arises in pa and is supposed maintenance of plag1 expression from pa to cxpa. Surgery is the most common form of treatment for pleomorphic adenoma. 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. Study and implications for diagnostics and therapy. Carcinoma ex pleomorphic adenoma cxpa is a rare parotid malignancy and until today no standardized concept exists for its therapy apart from recommendations for parotid carcinoma in general. Intracapsular carcinoma ex pleomorphic adenoma in the. Carcinoma ex pleomorphic adenoma of the parotid gland ajnr. Carcinoma ex pleomorphic adenoma cxpa is an aggressive, poorly understood salivary gland malignancy.
Prognosis is thought to be poorer than for other parotid malignancies. It derives its name from the architectural pleomorphism variable appearance seen by. Pdf ossifying parotid carcinoma ex pleomorphic adenoma. We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70yearold man. Ductal adenocarcinoma ex pleomorphic adenoma of the. Some choose to leave them untreated but if not treated some of the pleomorphic adenoma can experience malignant changes because of them recurrence. Invasive myoepithelial carcinoma ex pleomorphic adenoma of. These cancerous transformations are known as carcinoma ex pleomorphic adenoma or malignant mixed tumor. Case report open access an unusual case of spleen metastasis from carcinoma ex pleomorphic adenoma of the parotid gland luigi rossi1, erika giordani1, antonella fontana2, claudio di cristofano3, giuseppe cavallaro4, oreste bagni5, luca filippi5, loredana bianchi1, giulia rinaldi1, francesca perrone congedi1, anselmo papa1, davide caruso1, monica verrico1, gianfranco silecchia4 and silverio tomao1. Carcinoma ex pleomorphic adenoma ca ex pa is a rare malignant transformation of a benign primary pleomorphic adenoma pa. We report the case of a 62yearold male who presented with a swelling over his left temple.
Among all salivary gland neoplasms, carcinoma ex pleomorphic adenoma ca ex pa accounts for approximately 3. Pleomorphic adenoma symptoms, causes, treatment, surgery. Full text sinonasalnasopharyngeal pleomorphic adenoma. The difference in the clinical features between carcinoma. Metastasis of carcinoma ex pleomorphic adenoma to the. Carcinoma ex pleomorphic adenoma cxpa is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma. Carcinoma ex pleomorphic adenoma affects primarily adults and presents at a. 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 parotid gland. Carcinoma ex pleomorphic adenoma cxpa is an uncommon malignant tumor with highly aggressive biological behavior. In most instances 75%, the luminal epithelial cells undergo malignant change. 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. The mass was clinically suspected to be a benign salivary gland tumor based on palpation and magnetic resonance imaging findings.
Prognostic factors affecting the clinical outcome of. The cause of formation of this salivary gland malignancy is believed to be due to genetic factors. Maspin expression was studied by means of immunohistochemistry in 16 cases of cxpa, using the labelled polymer method. Pdf pleomorphic adenoma pa is the most common benign neoplasm affecting the major salivary glands. Cytomorphologic spectrum of carcinoma ex pleomorphic adenoma. Maspin expression in carcinoma ex pleomorphic adenoma. A 55yearold male patient presented with an asymptomatic mass of the upper lip that had noticed 8 years previously. It arises from the benign tumour pleomorphic adenoma its prognosis depends on the stage. Among these types of adenoma, carcinoma ex pleomorphic adenoma is the most commonly encountered. After extensive search of literature in english, there are only 3 previous reports of primary carcinoma ex pleomorphic adenoma of the trachea. The most common clinical presentation of ca ex pa is of a firm mass in. Ca ex pa has been named as carcinoma ex mixed tumour, carcinoma ex adenoma, and carcinoma ex benign pleomorphic adenoma.
Intraductal carcinoma is the precursor of carcinoma ex pleomorphic adenoma and is often associated with dysfunctional p53. Myoepithelial carcinoma mc, also known as malignant myoepithelioma, shows an. Pathology outlines carcinoma ex pleomorphic adenoma. It often poses a diagnostic challenge to clinicians and pathologists. Pdf carcinoma expleomorphic adenoma of submandibular. Pathological assessment is the gold standard for making the diagnosis. Jul 09, 2011 carcinoma ex pleomorphic adenoma ca ex pa is a carcinoma arising from a primary or recurrent benign pleomorphic adenoma. The aim of this study was to evaluate the plag1 expression maintenance by immunohistochemistry in a series of pas and cxpas.
The entity is difficult to diagnose preoperatively. Under a clinical diagnosis of benign salivary gland tumor, excision of. Carcinoma ex pleomorphic adenoma is an epithelial malignant neoplasm arising from a primary or recurrent pleomorphic adenoma and is a diagnostic challenge for cytopathologists. It accounts for 34% of salivary neoplasms and approximately 12% of. Pdf carcinoma ex pleomorphic adenoma ca ex pa is a malignant neoplasm arising from primary or recurrent benign. Carcinoma ex pleomorphic adenoma ca ex psa is a rare and poorly understood malignancy of salivary glands. Incidence pleomorphic adenoma86% adenoid cystic carcinoma25% mucoepidermoid carcinoma18% palate68% benign 62% malignant 38% parotid 60% submandibular 16% sublingual 12% minor 22% krishnaraj subhashraj, salivary gland tumors. Pdf carcinoma ex pleomorphic adenoma of the palate a case.
Pleomorphic adenoma surgery, symptoms, causes, treatment. Pleomorphic adenoma pa, also called mixed tumor is the most common tumor in the lacrimal gland, consisting 50% of epithelial lacrimal gland tumors. Carcinoma ex pleomorphic adenoma is the malignant counterpart of pleomorphic adenoma. We present 4 cases of carcinoma ex pleomorphic adenoma of the parotid gland. These tumors occasionally undergo malignant transformation, with potentially devastating consequences. Aug 29, 2018 carcinoma ex pleomorphic adenoma is an epithelial malignant neoplasm arising from a primary or recurrent pleomorphic adenoma and is a diagnostic challenge for cytopathologists. 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.
Although rare, this condition has been reported to be responsible for 4. Thoracic intramedullary spinal metastasis from carcinoma. This image shows the colocalisation of both benign and malignant components of carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma of the parotid gland. Only three cases of cxpa of the trachea have been reported in the literature. Pleomorphic adenomas pas, also known as benign mixed tumors, are common tumors of the parotid gland. The current definition of ca ex pa became widely accepted in the second half of the. History of a pleomorphic adenoma at the same site 25% of cases features of a pleomorphic adenoma and a carcinoma. Because of several highrisk factors for metastasis i. Carcinoma ex pleomorphic adenoma of the parotid gland nobuo. In all cases of carcinoma ex pleomorphic adenoma, a pa ghost was identified, and the malignant element was either undifferentiated carcinoma or.
Carcinoma ex pleomorphic adenoma affects glandular structures, occurring mostly in major salivary glands and less commonly in the lacrimal gland. Prognostic factors affecting the clinical outcome of carcinoma ex. Carcinoma expleomorphic adenoma cxpa is a rare, aggressive, poorly understood malignancy of the salivary gland. He underwent an en bloc resection of the mass with orbital. Carcinoma ex pleomorphic adenoma ca ex pa is a type of cancer typically found in the parotid gland. Carcinoma ex pleomorphic adenoma of the salivary glands. Under a clinical diagnosis of benign salivary gland tumor, excision of the mass with the sublingual. Our goal was to investigate the prognosis of cxpa in the major salivary glands and factors influencing it. Immunohistochemical features of carcinoma ex pleomorphic. Sep 17, 20 squamous cell carcinoma scc as the malignant component of carcinoma ex pleomorphic adenoma cxpa occurring in upper lip is rare. Caexpa, carcinosarcoma and metastasizing carcinoma. Only a few case reports describing the radiologic features of cxpa have been published.
Cytomorphologic spectrum of carcinoma ex pleomorphic. Carcinoma ex pleomorphic adenoma of the palate a case report article pdf available in journal of korean medical science 121. Salivary glands tumors are rare, and the most common benign salivary gland tumor is the pleomorphic adenoma, which comprises approximately 50% of all salivary gland tumors and 65% of parotid gland tumors. Sections of parotid gland show a salivary duct carcinoma with evidence of intermixed pleomorphic adenoma carcinoma ex pleomorphic adenoma. 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.
Quantitative promoter methylation differentiates carcinoma ex. Malignancy arising from a pleomorphic adenoma diagnosis either 1 or 2. Nov 22, 2015 carcinoma ex pleomorphic adenoma cxpa. Several features of this site will not function whilst javascript is disabled. Cns metastases of carcinoma ex pleomorphic adenoma of the. Jan 12, 2012 we report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70yearold man. 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 ex pleomorphic adenoma, a risk that increases with time 9. Immunohistochemical expression of p53 in pleomorphic adenoma. Plag1 expression from pleomorphic adenoma to carcinoma ex. 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 article about carcinoma ex. Pleomorphic adenoma mixed tumor, a benign tumor of salivary gland type containing epithelial, myoepithelial, and stromal components, is the most commonly diagnosed lesion of the salivary glands. Carcinoma ex pleomorphic adenoma cxpa of the salivary glands is a relatively rare carcinoma. Metastasis of carcinoma ex pleomorphic adenoma to the brain.
Meanwhile, the imaging findings of carcinoma ex pleomorphic adenoma are often nonspecific and are. 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. 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. Vaciamiento cervical radical sin presencia tumoral figs. Evaluate the clinicopathologic characteristics of pa of the major salivary glands, particularly tumors showing signs of malignant transformation. Carcinoma ex pleomorphic adenoma may be asymptomatic 2 and has similar clinical presentations as benign pleomorphic adenoma. Oct 28, 2016 mec ex pleomorphic adenoma pa was suspected. Carcinoma expleomorphic adenoma of parotid radiology. Pleomorphic adenoma pa, the most frequent tumour in salivary glands, can undergo malignant transformation. Sinonasalnasopharyngeal pleomorphic adenoma and carcinoma ex pleomorphic adenoma. 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.
Although lacrimal pa is benign, it is inclined to recur after incomplete surgical resection, and has the possibility to transform into carcinoma ex pleomorphic adenoma ca ex pa with a poor prognosis. Pleomorphic adenoma shows malignant changes during its natural history and occurs in three forms, i. We report a case of carcinoma ex pleomorphic adenoma in a patient presenting with left facial nerve palsy. Carcinoma ex pleomorphic adenoma radiology reference. Clear cell myoepithelial carcinoma ex pleomorphic adenoma of. Invasive carcinoma is wellcircumscribed with a pushing border.
Carcinoma ex pleomorphic adenoma ca ex pa is a carcinoma arising from a primary or recurrent benign pleomorphic adenoma. In imaging examination, there is no calcification can be seen in the primary tracheal cxpa of the previous cases, but ours can. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtypes are possible. Carcinoma ex pleomorphic adenoma affects primarily adults and presents at a mean age of approximately 60 years. Carcinoma ex pleomorphic adenoma article about carcinoma. Cytological features of carcinoma ex pleomorphic adenoma. Pdf carcinoma ex pleomorphic adenoma of the palate a. Although salivary gland tumors are common, intracapsular carcinoma ex pleomorphic adenoma is a rare entity especially within the buccal space with no similar case report in the current literature. Squamous cell carcinoma scc as the malignant component of carcinoma ex pleomorphic adenoma cxpa occurring in upper lip is rare. It is defined as a carcinoma that arises in the epithelial andor myoepithelial component of a pleomorphic adenoma. Clear cell myoepithelial carcinoma ex pleomorphic adenoma of parotid gland.
The most common clinical presentation of ca ex pa is of a firm mass in the. Full text sinonasalnasopharyngeal pleomorphic adenoma and. Diagnosis requires that elements from the benign pleomorphic adenoma and the malignant component need to be seen. The difference in the clinical features between carcinoma ex. Carcinoma ex pleomorphic adenoma of the lacrimal gland.
It arises from the benign tumour pleomorphic adenoma. Pulmonary salivary glandtype tumors with features of. Carcinoma ex pleomorphic adenoma diagnostic histopathology. It accounts for 34% of salivary neoplasms and approximately 12% of all salivary. Superficial parotid gland resection was performed in 20. According to the extent of invasion, the tumours were subdivided into. To investigate the presence and distribution of the protein maspin in carcinoma ex pleomorphic adenoma cxpa. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. Carcinoma ex pleomorphic adenoma cxpa is a rare aggressive epithelial malignancy arising from a primary or recurrent benign mixed tumor.
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