He underwent an en bloc resection of the mass with orbital. Sections of parotid gland show a salivary duct carcinoma with evidence of intermixed pleomorphic adenoma carcinoma ex pleomorphic adenoma. What are the causes of carcinoma ex pleomorphic adenoma of salivary gland. We report a case of carcinoma ex pleomorphic adenoma in a patient presenting with left facial. These tumors occasionally undergo malignant transformation, with potentially devastating consequences. All tumours showed areas of pleomorphic adenoma adjacent to typical areas of malignant transformation. Following a negative systemic workup, the patient underwent radiation therapy and remains free of clinical or radiographic disease 24 months following surgery. Herein the presentation, radiography, histopathology, and treatment outcome of a rare case of lacrimal gland epithelialmyoepithelial carcinoma ex pleomorphic.
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. 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. Pleomorphic adenoma is the most common salivary gland tumor and accounts for approximately 80% of parotid tumors. 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. The presence of these different elements accounts for the name pleomorphic tumor or mixed one characteristic of a pleomorphic adenoma is the presence of microscopic projections of tumor outside of the. 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. Carcinoma ex pleomorphic adenoma ca ex pa accounts for about 4% of all salivary tumors and about 12% of salivary malignancies. Pathology outlines carcinoma ex pleomorphic adenoma. Therefore, we finally made a diagnosis of invasive salivary duct carcinoma ex pleomorphic adenoma. Only two cases were ever reported with brain metastases, with absence of good evidence guiding management of such cases.
Only a few cases of carcinoma ex pleomorphic adenoma arising in the. Carcinoma ex pleomorphic adenoma of the sublingual gland is extremely rare and frequently misdiagnosed. Invasive carcinoma is wellcircumscribed with a pushing border. 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 falls under the category of malignant transformations that can occur in a benign pleomorphic adenoma. Primary carcinoma ex pleomorphic adenoma, tracheobronchial system, primary salivary glandtype tu mors, pleomorphic adenoma. Pdf squamous cell carcinoma ex pleomorphic adenoma a.
The cause of formation of this salivary gland malignancy is believed to be due to genetic factors. Ca ex pa is a carcinomatous change as a primary or as a recurrence of pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is an epithelial malignant neoplasm arising from a primary or recurrent pleomorphic adenoma and is a diagnostic challenge for cytopathologists. Pleomorphic adenoma presenting a 2x2cm well circumscribed lesion on the palate of a 67year old female iarc screening group. A case of squamous cell carcinoma ex pleomorphic adenoma in a palate is presented and comments on diagnostic criterias are described. The chart showing pdf series, word series, html series, scan qr codes. Pa is a rare epithelial malignancy arising from primary or recurrent pa. Carcinoma ex pleomorphic adenoma, with particular emphasis.
Pdf carcinoma ex pleomorphic adenoma of the parotid. The difference in the clinical features between carcinoma. Preoperatively, a diagnosis of pleomorphic adenoma of the parotid gland metastasizing to the cervical lymph node was made, but postoperatively it was reported. The sites of origin are usually the parotid 67%, minor glands 18 %, and submandibular 15% and sublingual glands pleomorphic adenoma may be difficult to obtain but necessary for diagnosis unless benign tumor coexists with malignant tumor note.
Pleomorphic adenoma pa, the most frequent tumour in salivary glands, can undergo malignant transformation. Case report abstract carcinoma ex pleomorphic adenoma is a rare malignant tumor of the major salivary glands, derived from a preexisting pleomorphic adenoma. Surgery is the most common form of treatment for pleomorphic adenoma. Pdf mucoepidermoid carcinoma ex pleomorphic adenoma. In an attempt to elucidate the malignant behavior of. Under a clinical diagnosis of benign salivary gland tumor, excision of. Case report primary carcinoma ex pleomorphic adenoma of the. Ductal adenocarcinoma ex pleomorphic adenoma of the. Carcinoma expleomorphic adenoma cxpa is an aggressive neoplasm that arises from a pleomorphic adenoma. Due to resemblance of clinical symptoms of ca ex pa and benign pleomorphic adenoma, it is mandatory for surgeons to keep high degree of clinical alertness. A 61yearold woman presenting with facial paralysis was found to have carcinoma ex pleomorphic. These cancerous transformations are known as carcinoma ex pleomorphic adenoma or malignant mixed tumor. The most common clinical presentation of ca ex pa is of a firm mass in. Carcinoma ex pleomorphic adenoma cxpa is an uncommon malignant tumor with highly aggressive biological behavior.
Carcinoma ex pleomorphic adenoma in the parapharyngeal space article pdf available in philippine journal of otolaryngology head and neck surgery 281 june 20 with 59 reads. We report the case of a 62yearold male who presented with a swelling over his left temple. The most common clinical presentation of ca ex pa is of a firm mass in the. The spectrum of malignancy in pleomorphic adenoma comprises three distinct entities carcinoma ex pleomorphic adenoma, carcinosarcoma and benign metastasising pleomorphic adenoma. 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 soft palate with. Mar 30, 2010 carcinoma ex pleomorphic adenoma cxpa is an aggressive salivary gland malignancy and rare in minor salivary gland. Carcinoma ex pleomorphic adenoma, an uncommon neoplasm of the parotid gland, accounts for less than 4% of salivary gland tumors. Carcinoma ex pleomorphic adenoma pa is the most familiar model for tumour progression in the salivary glands. Dec 14, 2019 there are 3 main patterns of malignant change that occur in pleomorphic adenoma. It arises from the benign tumour pleomorphic adenoma its prognosis depends on the stage. A case of carcinoma ex pleomorphic adenoma arising from.
Former lecturer, mar baselios dental college, kerala, india. Salivary duct carcinoma and the concept of early carcinoma ex. Pulmonary salivary glandtype tumors with features of. Malignant degeneration of an adenoma increases from 1. Prognostic factors affecting the clinical outcome of. Pdf carcinoma ex pleomorphic adenoma in the parapharyngeal. Pdf cyclin d1 and p16 expression in pleomorphic adenoma. When recurrence and distant metastasis occur, survival is so low. We present 4 cases of carcinoma ex pleomorphic adenoma of the parotid gland. Immunohistochemistry was used to examine cyclin d1 and p16 expression in 43 parotid. Carcinoma ex pleomorphic adenoma diagnostic histopathology. These cases add to the spectrum of tumours shared by breast and salivary gland.
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. It arises from the benign tumour pleomorphic adenoma. It is the most common of the transformations, with carcinosarcoma malignant mixed tumor and metastasizing adenoma being much less likely. It arises from a benign pleomorphic adenoma presenting in the sixth to eighth decades of life.
Metastatic deposits of carcinoma ex pleomorphic adenoma are either composed exclusively of malignant elements or contain mixed benign and malignant components. Some choose to leave them untreated but if not treated some of the pleomorphic adenoma can experience malignant changes because of them recurrence. Although rare, this condition has been reported to be responsible for 4. Carcinoma ex pleomorphic adenoma is a malignant transformation of the common benign neoplasm of the salivary glands, pleomorphic adenoma. In all cases of carcinoma ex pleomorphic adenoma, a pa ghost was identified, and the malignant element was either undifferentiated carcinoma or. Immunohistochemical expression of p53 in pleomorphic adenoma. This tumor represents less than 4% of malignant salivary gland tumors and 11. We describe the case of a 63yearold man with carcinoma ex pleomorphic adenoma of the submandibular gland and metastases to the.
Pdf carcinoma ex pleomorphic adenoma of the palate a case. Pleomorphic adenomas pas, also known as benign mixed tumors, are common tumors of the parotid gland. Only three cases of cxpa of the trachea have been reported in the literature. Cns metastases of carcinoma ex pleomorphic adenoma of the. Thoracic intramedullary spinal metastasis from carcinoma ex. Our data suggest that inactivation of p53 may play an important role in the evolution of pleomorphic salivary adenoma and carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is a rare malignancy of the head and neck, in particular, in the minor salivary glands. Carcinoma ex pleomorphic adenoma of the lacrimal gland. Carcinoma ex pleomorphic adenoma is transformed at the incidence of 120% in pleomorphic adenoma and frequently recurred. Carcinoma ex pleomorphic adenoma ca ex pa is extremely unusual in minor salivary glands of oral cavity. To compare cyclin d1 and p16ink4 p16 expression in normal tissue, pleomorphic adenoma pa and carcinoma ex pleomorphic adenoma cxpa of the parotid gland. Thoracic intramedullary spinal metastasis from carcinoma ex pleomorphic adenoma of the submandibular gland py waghmare, k abhaya department of neurosurgery, kokilaben dhirubhai ambani hospital, mumbai, india. History of a pleomorphic adenoma at the same site 25% of cases features of a pleomorphic adenoma and a carcinoma. Carcinomas ex pleomorphic adenoma and metastasizing benign mixed tumor are two varients of this tumor undergoing malignant transformation.
Diagnosis requires that elements from the benign pleomorphic adenoma and the malignant component need to be seen. Carcinoma ex pleomorphic adenoma cxpa is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma. May 30, 2012 within the background of pleomorphic adenoma, the carcinoma cells sometimes replaced ductal luminal cells while retaining an intactlike myoepithelial layer. Pleomorphic adenoma usually develops as a nontender nodular lesion with a slow growth rate persisting for years. 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. The present paper reports a case of pleomorphic adenoma pa in submandibular gland that, after more than 30 years of initial treatment, recurred and metastasized to ipsilateral neck lymph nodes and parotid gland. Malignancy arising from a pleomorphic adenoma diagnosis either 1 or 2. Pleomorphic adenoma, the most common salivary gland tumor, consists of epithelial. Carcinoma ex pleomorphic adenoma, with particular emphasis on. Pdf carcinoma ex pleomorphic adenoma of the palate a. A 16yearold female patient presented with right cheek swelling.
Fine needle aspiration cytology showed squamous cells, basaloid cells, and foamy cells, along with. Carcinoma expleomorphic adenoma of parotid radiology. It may cause difficulty in cytological interpretation, due to limited and selective sampling. Pleomorphic adenoma an overview sciencedirect topics. A soft palate cxpa initially presenting as direct cavernous sinus cs invasion is very rare. Find out information about carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma of parotid gland with. However, there is evidence to support the more conservative approach of. Figure 2 these glands exhibited nuclear pleomorphism, luminal necrosis, and mitoses compatible with an adenocarcinomatous proliferation. Carcinoma ex pleomorphic adenoma cxpa is a rare epithelial malignancy that arises from a primary or recurrent pleomorphic adenoma pa. Invasive salivary duct carcinoma ex pleomorphic adenoma of. Imaging revealed a lytic lesion over the left orbital wall with soft tissue extension suggestive of malignancy.
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. Carcinoma ex pleomorphic adenoma, abbreviated ca ex pa, is a rare malignant salivary gland tumour that arises from a pleomorphic adenoma. It accounts for 10% of all malignant salivary tumors and its average age. The patient was 36yearold male presenting with an ovoid. Maspin expression in carcinoma ex pleomorphic adenoma. Immunohistochemical features of carcinoma ex pleomorphic.
Cellular proliferation index between carcinoma expleomorphic. Carcinoma ex pleomorphic adenoma cxpa has been considered an interesting model of carcinogenesis, presenting various histological subtypes and invasiveness phase. Carcinoma ex pleomorphic adenoma caexpa is a carcinoma that shows histologic evidence of arising in a pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is a malignant epithelial tumor of salivary glands arising in preexisting pleomorphic adenoma and accounts for approximately 12% of all malignant salivary gland tumors. N2 pathologic factors of predictive value for carcinoma ex pleomorphic adenoma cxpa, an aggressive salivary gland malignancy, are poorly defined. Carcinoma ex pleomorphic adenoma cxpa is the malignant transformation of. Salivary duct carcinoma ex pleomorphic adenoma of the palate. The tumour cells demonstrate a squamoid appearance with abundant cytoplasm and form large dilated ducts containing central comedonecrosis. Alternative names are intracapsular, in situ, or focal carcinoma. Mar 30, 2012 carcinoma ex pleomorphic adenoma is a rare neoplasm of the submandibular gland with a poor prognosis. Figure 3 based on these features, the case was signed out as an intracapsular carcinoma ex pleomorphic adenoma. A 55yearold male patient presented with an asymptomatic mass of the upper lip that had noticed 8 years previously. A 60yearold male had a 3month history of a small soft palatal mass with progressing left cheek numbness, proptosis, and disturbed vision. Carcinoma ex pleomorphic adenoma of the sublingual gland.
Carcinoma expleomorphic adenoma of parotid radiology case. Abstract background carcinoma ex pleomorphic adenoma cxpa is an aggressive, poorly understood salivary gland malignancy. The objective was to determine the proliferative index of cxpa and comparing to pleomorphic adenoma pa. Carcinoma ex pleomorphic adenoma ca ex pa is a carcinoma arising from a primary or recurrent benign pleomorphic adenoma. Carcinoma ex pleomorphic adenoma article about carcinoma ex. Carcinoma ex pleomorphic adenoma cxpa is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic. Carcinoma expleomorphic adenoma derived from recurrent. Carcinoma ex pleomorphic adenoma is a carcinoma arising from a preexisting pleomorphic adenoma. Pleomorphic adenoma, though a benign tumor of salivary gland, should be diagnosed at an early stage and surgically excised.
The majority of these neoplasms originate from the mucosa of the bony or cartilaginous septum. Ca ex pa has been named as carcinoma ex mixed tumour, carcinoma ex adenoma, and carcinoma ex benign 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. Pleomorphic adenoma pa, also called mixed tumor is the most common tumor in the lacrimal gland, consisting 50% of epithelial lacrimal gland tumors. Noninvasive carcinoma ex pleomorphic adenoma of the parotid. The diagnosis is made on identification of benign tumor or carcinoma arising in a site previously involved by pleomorphic adenoma. Malignant pleomorphic adenoma, salivary gland tumors. Histological diversity is the hallmark of pleomorphic adenoma, the most common salivary gland tumor.
Pleomorphic adenoma pa is the most common benign neoplasm affecting the major salivary glands. 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. Most cases arise in the major salivary glands, most commonly, the parotid gland followed by the submandibular gland. Carcinoma ex pleomorphic adenoma of the parotid gland. Frontiers trastuzumab emtansine for the treatment of her. Patients were 82, 60 and 56 years old and presented with a breast lump. Among salivary gland neoplasms, metastasizing pleomorphic adenoma mpa constitutes an extremely rare group of tumors. Dec 26, 2014 the epithelial cells make up a trabecular pattern that is contained within a stroma. The stroma may be chondroid, myxoid, osteoid, or fibroid. Abstract three cases of carcinoma ex pleomorphic adenoma of the breast are reported.
It is more common in parotid gland and sometimes occurs in submandibular and other minor salivary glands. Carcinoma ex pleomorphic adenoma of the maxillary sinus. 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. Carcinoma ex pleomorphic adenoma ca ex pa is a rare malignant transformation of a benign primary pleomorphic adenoma pa. Carcinoma ex pleomorphic adenoma affects glandular structures, occurring mostly in major salivary glands and less commonly in the lacrimal gland. 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. Herein, we report a case of a highgrade mucoepidermoid carcinoma ex pleomorphic adenoma within the deep lobe of the parotid gland. It often poses a diagnostic challenge to clinicians and pathologists. Pleomorphic adenoma surgery, symptoms, causes, treatment. Effective chemotherapy for submandibular gland carcinoma ex. Carcinoma ex pleomorphic adenoma cxpa is a broad category of carcinomas of the salivary glands which includes at least 2 clinically relevant categories. 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.
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 ca ex pa is a type of cancer typically found in the parotid gland. Pleomorphic adenoma originally called the mixed tumour is a neoplasm commonly involving major salivary glands. In imaging examination, there is no calcification can be seen in the primary tracheal cxpa of the previous cases, but ours can. Pathologic analysis of 73 cases, human pathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. The current definition of ca ex pa became widely accepted in the second half of the. The average age is 46 years, but the range is since the first to the fifth decade with a predilection for. After extensive search of literature in english, there are only 3 previous reports of primary carcinoma ex pleomorphic adenoma of the trachea. 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. This paper aims to present a clinical case of a patient with carcinoma ex pleomorphic adenoma with long term evolution, which reached remarkable size. Carcinoma ex pleomorphic adenoma radiology reference. Pleomorphic adenoma symptoms, causes, treatment, surgery. Carcinoma expleomorphic adenoma of submandibular salivary. Our goal was to investigate the prognosis of cxpa in the major salivary glands and factors influencing it.
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