Carcinoma ex pleomorphic adenoma affects glandular structures, occurring mostly in major salivary glands and less commonly in the lacrimal gland. Carcinoma ex pleomorphic adenoma cxpa is an uncommon malignant tumor with highly aggressive biological behavior. Carcinoma ex pleomorphic adenoma ca ex pa accounts for about 4% of all salivary tumors and about 12% of salivary malignancies. We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70yearold man.
Squamous cell carcinoma scc as the malignant component of carcinoma ex pleomorphic adenoma cxpa occurring in upper lip is rare. Pleomorphic adenoma, though a benign tumor of salivary gland, should be diagnosed at an early stage and surgically excised. The tumour cells demonstrate a squamoid appearance with abundant cytoplasm and form large dilated ducts containing central comedonecrosis. Sload, peter carbone, christopher johnson and terence johnson naval medical center san diego, ent, san diego, ca, usa abstract carcinoma ex pleomorphic adenoma ca ex pa is a rare salivary gland malignancy arising from a previously benign pleomorphic adenoma. These tumors occasionally undergo malignant transformation, with potentially devastating consequences. Carcinoma ex pleomorphic adenoma ca ex pa is extremely unusual in minor salivary glands of oral cavity. Under a clinical diagnosis of benign salivary gland tumor, excision of.
The cause of formation of this salivary gland malignancy is believed to be due to genetic factors. Only three cases of cxpa of the trachea have been reported in the literature. They represent 60 to 80% of all benign tumors in major salivary glands and. Sections of parotid gland show a salivary duct carcinoma with evidence of intermixed pleomorphic adenoma carcinoma ex pleomorphic adenoma.
Most malignant mixed tumors are carcinoma ex pleomorphic adenoma, whereas the latter 2 types are extremely unusual. Pdf ossifying parotid carcinoma ex pleomorphic adenoma. The mass was clinically suspected to be a benign salivary gland tumor based on palpation and magnetic resonance imaging. Pleomorphic adenoma an overview sciencedirect topics. 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. Ki67 is a marker for showing cells dna synthesis before mitosis. Carcinoma ex pleomorphic adenoma of the parotid gland ryan l.
Cns metastases of carcinoma ex pleomorphic adenoma of the. Carcinoma ex pleomorphic adenoma of the salivary glands. Our goal was to investigate the prognosis of cxpa in the major salivary glands and factors influencing it. A 55yearold male patient presented with an asymptomatic mass of the upper lip that had noticed 8 years previously. Diagnosis requires that elements from the benign pleomorphic adenoma and the malignant component need to be seen. Carcinoma ex pleomorphic adenoma radiology reference. 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. Invasive myoepithelial carcinoma ex pleomorphic adenoma of. Carcinomas ex pleomorphic adenoma and malignant mixed. Pdf mucoepidermoid carcinoma ex pleomorphic adenoma. 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 in a young woman delaram j taghipour, md1, bonnie c davis, md2, babak shokrani, md3, blair a simon, bs4, william r bond, jr. It arises from a benign pleomorphic adenoma presenting in the sixth to eighth decades of life. Maspin expression in carcinoma ex pleomorphic adenoma.
Carcinoma ex pleomorphic adenoma cxpa is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma. Pdf squamous cell carcinoma ex pleomorphic adenoma. Only one of the patients with lowgrade terminal duct carcinomas died of his disease during followup periods extending to. Management of pleomorphic adenomas is similar to most benign sgts, with the exception. 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. 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. Superficial parotid gland resection was performed in 20. Carcinoma ex pleomorphic adenoma of the maxillary sinus. Carcinoma ex pleomorphic adenoma of the lacrimal gland.
The pleomorphic adenoma is the most common tumour of glandular origin in the head and neck 1. Carcinoma ex pleomorphic adenoma ca ex pa is a type of cancer typically found in the parotid gland. It arises from the benign tumour pleomorphic adenoma its prognosis depends on the stage. Carcinoma ex pleomorphic adenoma is an epithelial malignant neoplasm arising from a primary or recurrent pleomorphic adenoma and is a diagnostic challenge for cytopathologists. The sites of origin are usually the parotid 67%, minor glands 18 %, and submandibular 15% and sublingual glands carcinoma ex pleomorphic adenoma cxpa is an aggressive, poorly understood salivary gland malignancy. Perineural and lymphovascular invasion and gross extraglandular extension were identified. Pathology outlines carcinoma ex pleomorphic adenoma. Carcinoma ex pleomorphic adenoma diagnostic histopathology. Pleomorphic adenoma pa, the most frequent tumour in salivary glands, can undergo malignant transformation. Pa is a rare epithelial malignancy arising from primary or recurrent pa. Pdf a rare case of squamous cell carcinoma ex pleomorphic adenoma in maxillary sinus is presented. Carcinoma ex pleomorphic adenoma, abbreviated ca ex pa, is a rare malignant salivary gland tumour that arises from a pleomorphic adenoma. Although rare, this condition has been reported to be responsible for 4. Carcinoma ex pleomorphic adenoma, an uncommon neoplasm of the parotid gland, accounts for less than 4% of salivary gland tumors.
Salivary gland tumors are rare and make up to 3% of head and neck tumors1. In the 1991 who classification of tumours of the salivary glands, the term malignant mixed tumour comprises three different pathologies. The antecedent benign tumor may either be a longstanding one, often with a history measured in decades, or characterized by a protracted history of excisions and multiple recurrences. 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. Carcinoma expleomorphic adenoma of parotid radiology.
It arises from the benign tumour pleomorphic adenoma. Carcinoma ex pleomorphic adenoma cxpa is defined as a carcinoma arising from a preexisting benign pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is a carcinoma arising from a preexisting pleomorphic adenoma. Immunohistochemical features of carcinoma ex pleomorphic. A giant submandibular gland carcinoma expleomorphic adenoma introduction. Because of several highrisk factors for metastasis i. Recognize the presentation of pleomorphic adenoma pa and carcinoma ex pa caxpa 2.
Immunohistochemical evaluation of androgen receptor, her2. Carcinoma ex pleomorphic adenoma is defined as a pleomorphic adenoma from which derived an epithelial malignancy. Case report primary carcinoma ex pleomorphic adenoma of. Prognostic factors affecting the clinical outcome 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. Md, mba5 department of surgery1, department of radiology2, department of pathology3, medical school4, department of otolaryngology5, howard university college of medicine, washington dc 20059. Carcinoma ex pleomorphic adenoma is rare and comprises approximately 3. Carcinoma ex pleomorphic adenoma cxpa is an infrequent but aggressive salivary gland malignancy that develops in association with a primary or recurrent benign pleomorphic adenoma pa. These pathologies, which should not be confused, are the carcinoma ex pleomorphic adenoma. Pleomorphic adenomas pas, also known as benign mixed tumors, are common tumors of the parotid gland. Carcinoma ex pleomorphic adenoma of the parotid gland. Caexpa is a kind of infiltrative carcinoma arising in a pa.
However, there is evidence to support the more conservative approach of. Prognostic factors of recurrence in salivary carcinoma ex pleomorphic adenoma, with emphasis on the carcinoma histologic subtype. Herein, we report a case of a highgrade mucoepidermoid carcinoma ex pleomorphic adenoma within the deep lobe of the parotid gland. Carcinomas ex pleomorphic adenoma and metastasizing benign mixed tumor are two varients of this tumor undergoing malignant transformation. Carcinoma ex pleomorphic adenoma of the sublingual gland. Kim jw, kwon gy, roh jl, choi sh, nam sy, kim sy, et al. The authors present a case of carcinoma ex pleomorphic adenoma. The most common salivary gland tumor is pleomorphic adenoma pa. 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 caexpa with a poor prognosis. The deep and diffuse distribution of salivary glands in the soft tissue of the head and neck region is one common limitation to the early diagnosis of pleomorphic adenoma.
Pleomorphic adenoma is the most common salivary gland tumor and accounts for approximately 80% of parotid tumors. Prognostic factors of recurrence in salivary carcinoma ex. Ca ex pa is a carcinomatous change as a primary or as a recurrence of pleomorphic adenoma. 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. 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 sites of origin are usually the parotid 67%, minor glands 18 %, and submandibular 15% and sublingual glands pleomorphic adenoma rare spectrum of carcinomas demonstrating abrupt transition from benign tumor usually pleomorphic adenoma. Carcinoma ex pleomorphic adenoma cxpa, a rare malignancy of the salivary glands. Invasive carcinoma is wellcircumscribed with a pushing border. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtypes are possible. Though its pathogenesis remains unclear, it is hypothesized to be the result of. A case of carcinoma ex pleomorphic adenoma arising from. This tumor develops in primary or recurrent 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. It often poses a diagnostic challenge to clinicians and pathologists.