The preferential sites of metastases are the lung and bone, followed by the brain and liver. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. Finally, one case reported in cytology as benign salivary gland neoplasm turned out to be adenoid cystic carcinoma. Their recognition is important particularly when the component tumours have different biological behaviours. Adenoid cystic carcinoma of the head and neck literature. Review and updates of immunohistochemistry in selected. The disease needs early detection and complete treatment because of its tendency to recur, vicinity to sensitive organs and lower response to radiation. An unusual presentation of adenoid cystic carcinoma of the. Another significant feature is the incorporation of important knowledge gained during the past few years regarding the genetics of salivary gland tumors.
Jul 25, 2014 myoepithelial cell adenoid cystic carcinoma pleomorphic adenoma salivary gland tumour minor salivary gland these keywords were added by machine and not by the authors. Its innoceous clinical presentation remains a diagnostic challenge. Adenoid cystic carcinomas arise more commonly in the minor salivary glands 55% than in the major salivary glands. Adenoid cystic carcinoma nord national organization for. Jun 21, 2017 adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. This process is experimental and the keywords may be updated as the learning algorithm improves. Adenoid cystic carcinoma typically arises within salivary glands. Adenoid cystic carcinoma, tongue adenoid cystic carcinoma, tongue. Adenoid cystic carcinoma acc is a rare salivary gland malignant neoplasm. Overview adenoid cystic carcinoma research foundation. Summary of data38 patients with adenoid cystic carcinoma. May 14, 2007 histopathology salivary gland adenoid cystic carcinoma. Intracranial acc is even more infrequent and could be primary or secondary occurring either by direct invasion, hematogenous spread, or perineural spread. Adenoid cystic carcinoma acc is a rare malignant tumor of the salivary glands, with higher frequency in the submandibular gland, a prolonged clinical course, and poor long.
Adenoid cystic carcinoma of the salivary glands radiology. Histopathology images of adenoid cystic carcinoma, tongue by. Update in salivary gland pathology arup university of utah. The cribriform pattern, which is the most common, is characterized by nests of cells containing small, circular cystlike spaces. Adenoid cystic carcinoma is a rare salivary glandtype tumor that arises in exocrine glands including the salivary, lacrimal, mammary, and bronchial glands. Histology of adenoid cystic carcinoma subtypes tubular and cribriform and. Adenoid cystic carcinoma acc is a salivary gland malignancy that is an extremely rare neoplasm salivary gland or minor salivary gland of upper aerodigestive tract carcinoma characterized by its biphasic ductal and myoepithelial differentiation, tubular cribriform solid architecture head neck 2017. Two cases of mucoepidermoid carcinoma diagnosed in cytology were later confirmed by histopathology. Often palate is the most commonly involved site, tongue being the second most common. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. Diagnostic difficulties and pitfalls on diagnostic. Adenoid cystic carcinoma an overview sciencedirect topics. Adenoid cystic carcinoma of submandibular gland metastatic to.
Adenoid cystic carcinoma of the external auditory a case. The adenoid cystic carcinoma can occur in any salivary gland, however 50% develop in the minor salivary glands. View a short video led by an asco expert that provides information about what comes next after finishing treatment. Myoepithelial cell adenoid cystic carcinoma pleomorphic adenoma salivary gland tumour minor salivary gland these keywords were added by machine and not by the authors. In primary cutaneous adenoid cystic carcinoma, the tumour involves the dermis and may invade the subcutis figure 1.
The minor salivary glands consist of 800 small mucussecreting glands located throughout the lining of the oral cavity. Histopathology of the salivary glands will be of interest not only to. Adenoid cystic carcinoma acc of the head and neck is one of the most prevalent malignant salivary gland neoplasms 1. Adenoid cystic carcinoma genetic and rare diseases. Anna buda nowak, malgorzata swiader, miroslawa puskulluoglu, grzegorz dyduch, maciej krupinski, krzysztof krzemieniecki 2015 adenoid cystic carcinoma of the external auditory canal with metastases to lymph nodes and lungs problematic diagnosis and treatment based on a case report. An important feature of accs is the long clinical course with a high rate of distant metastases.
They are locally aggressive with a propensity for perineural spread 5. Adenoid cystic carcinoma, cribriform pattern, parotid gland. Adenoid cystic carcinoma is an epithelial malignancy composed of epithelial and myoepithelial cells variably arranged in tubular, cribriform, and solid patterns. Immunohistochemical studies were performed on 29 adenoid cystic carcinoma and 23 polymorphous lowgrade adenocarcinoma archival cases with bcl2 and ckit antibodies. Adenoid cystic carcinoma of salivary glands diagnostic. There are tumors of the minor salivary glands that derive directly from the pluripotent cells of the ductal system of the gland the above cells could be differentiated to squamous, mucous or cylindrical as well as sebaceous cells mucoepidermoid carcinoma mucous, intermediate, epidermoid sebaceous adenoma or carcinoma lymhadenoma. Primary external auditory canal eac malignancies are very rare with adenoid cystic carcinoma acc representing approximately 5% of these tumors. Adenoid cystic carcinoma organization international faq. Though acc is the most common malignant tumor of the submandibular, sublingual and minor salivary glands, it accounts for only 15% of parotid cancers. Investigators have worked together across academia, government agencies and private industry to move forward the various projects within the research agenda. Adenoid cystic carcinoma of the parotid metastasizing to. Adenoid cystic carcinoma of the submandibular gland. The salivary glands are classified as major or minor.
Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. Histopathology of the salivary glands springerlink. Although most adenoid cystic carcinomas are straight forward to diagnose by fna, occasional diagnostic challenges arise. Histopathology salivary glandadenoid cystic carcinoma youtube. Mr of parotid tumor that radiologically was diagnosed as a pleomorphic adenoma, but fine needle aspiration biopsy showed adenoid cystic carcinoma. Adenoid cystic carcinoma acc is a relatively rare form of cancer that most commonly develops in the salivary glands or other regions of the head and neck. The results were correlated to clinicopathological profile of the patients. This neoplasm is defined by its distinctive histologic appearance. Pdf management of salivary gland adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinomaand adenoid cystic carcinoma of the breast are rare but well recognized histology of adenoid cystic carcinoma.
Overall, adenoid cystic carcinoma acc is a rare tumor, accounting for only 1% of all malignant tumors of the oral and maxillofacial region. To evaluate the immunohistochemical expression of bcl2 and ckit proteins in the salivary gland tumors, adenoid cystic carcinoma, and polymorphous lowgrade adenocarcinoma with a view to tumor differentiation. There is insufficient knowledge of the natural behavior of acc in eac. Acc has a higher tendency to occur in hundreds of minor salivary glands than in the major salivary glands, which are the two large parotid glands in the cheek areas and the submandibular and sublingual glands towards the bottom of the mouth. Understanding acc adenoid cystic carcinoma acc is a rare malignancy of secretory glands, typically originating in the salivary glands though it does appear in other primary sites, including the palate, nasopharynx, tongue base, larynx, trachea, lacrimal gland, breast and vulva. Aug, 2007 adenoid cystic carcinoma acc is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region. Adenoid cystic carcinoma acc is an uncommon form of malignant neoplasm that arises within secretory glands, most commonly the major and minor salivary glands of the head and neck. Salivary gland neoplasms constitute 3% of cancers of all sites, of which, 1015% are malignant 1, 2. Adenoid cystic carcinoma parotid mr salivary gland pathology. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. May 31, 2016 adenoid cystic carcinoma acc is a rare tumor constitutes for less than 1% of head and neck malignancies and 10% of all salivary gland tumors. Salivary gland carcinomas sgcs are rare, accounting for adenoid cystic carcinoma of the salivary gland authors mj ashraf 1, negar azarpira 2, b khademi 1, s shaghasemi 1, n bagheri 1. Oct 29, 2004 morphologic distinction of highgrade adenoid cystic carcinoma from basaloid squamous cell carcinoma can be difficult. The tumors originating from both acinarductal epithelial cells and myoepithelialbasal cells are designated biphasic tumors, and this category includes tumors such as pleomorphic adenoma, epithelial myoepithelial carcinoma, and adenoid cystic carcinoma.
Histopathology images of adenoid cystic carcinoma, tongue. On routinelyprepared histological sections of resection speci. Other sites of origin include the trachea, lacrimal gland, breast, skin, and vulva. Adenoid cystic carcinoma is the most common malignant tumor of minor salivary glands occurring in the oral cavity and tongue. Adenoid cystic carcinoma of the external auditory a case report. Overview since accrf was founded in december 2005, a vibrant community of researchers, supported by patients, has sprung into action. Similar to the salivary gland counterparts, adenoid cystic carcinomas of the breast are characterized by t6. Histopathological grading of adenoid cystic carcinoma of the head. Acc can occur in other parts of the body, such as the breast, skin, cervix in females, prostate gland in males, and various other areas. Adenoid cystic carcinoma of the parotid gland medind. Adenoid cystic carcinoma of submandibular gland metastatic.
Lowgrade tumors have 10year survival rates of 8095%, while 10year survival rates for highgrade tumors range from 2550%. Clinically it represents as an indolent yet a persistent lesion, which shows propensity for late distant metastases, involving vital tissues often leading to the death of the patient. Fine needle aspiration cytology of salivary gland lesions. Palate is the most common site to be involved in the oral cavity followed by parotid gland and submandibular gland.
Pdf salivary gland adenoid cystic carcinoma researchgate. Five salivary gland tumours are currently known to harbour translocations and fusion oncogenes, namely, pleomorphic adenoma pa, mucoepidermoid carcinoma mec, adenoid cystic carcinoma adcc. One case of cystic lesion diagnosed in cytology turned out to be a warthins tumor. Of all salivary gland tumors only about 10% to 15% are of minor salivary gland origin.
Surgical removal of the tumor is the best treatment for adcc as long as it can be done safely and is likely to have a good outcome. It can also occur in the breast, uterus, or other locations in the body. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Adenoid cystic carcinoma acc is a rare malignant neoplasm of the salivary gland. Salivary gland tumors may cause painless masses in the mouth or face. Adenoid cystic carcinoma of salivary glands diagnostic and. Comprehensive skin examination and appropriate follow. Adenoid cystic carcinoma the oral cancer foundation. Histopathological grading of adenoid cystic carcinoma of the. In minor salivary glands, the frequency of adenoid cystic carcinoma is less than previously thought, with the recognition of polymorphous lowgrade adenocarcinoma as a distinct pathologic entity. The booklet is available as a pdf, so it is easy to print out. Cd117 kit expression is present in the luminal ductal epithelial cell component.
Acc occurs most often in minor salivary glands and the submandibular gland, and less frequently in the sublingual and parotid glands. The characteristic acellular spheres of adenoid cystic carcinoma are seen in a variety of other salivary gland tumors, including pleomorphic adenoma, basal cell adenoma, polymorphous lowgrade adenocarcinoma, and epithelialmyoepithelial carcinoma, 3 but usually they are. Adenoid cystic carcinoma is a rare salivary gland type tumor that arises in exocrine glands including the salivary, lacrimal, mammary, and bronchial glands. Adenoid cystic carcinoma, perineural invasion adenoid cystic carcinoma ihc ckit and bcl2 positive epithelial cells. These are under your tongue and on each side of your jaw below the jawbone. It most commonly arises in the major and minor salivary glands of the head and neck. A primary salivary gland or minor salivary gland of upper aerodigestive tract carcinoma characterized by its biphasic ductal and myoepithelial differentiation, tubular cribriform solid architecture head neck 2017. Histopathological grading of adenoid cystic carcinoma of.
A clinical, histological, and immunocytochemical comparison with adenoid cystic carcinoma of salivary glands and adenoid basal cell carcinoma. Assessment of p63 expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous lowgrade adenocarcinoma, and basal cell and canalicular adenomas. It tends to metastasize to lungs, bone, liver, brain, and rarely to skin. Adenoid cystic carcinoma acc of the head and neck is one of the most prevalent malignant salivary gland neoplasms. Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate. Though acc is the most common malignant tumor of the submandibular, sublingual and minor salivary glands, it accounts for only 15% of parotid cancers 3. During surgery, the surgeon will remove the tumor and an area of healthy tissue surrounding it, called a margin.
In 9 out of these 10 cases pathology specimens were available for revision and. In view of the cytologic diagnosis, proposed treatment includes resection of overlying skin which clinically was felt to be tethered and level ii cervical nodes. The adenoid cystic carcinoma constitutes approximately 29. Adenoid cystic carcinoma of the maxilla the value of histopathology in diagnosing a second primary volume 110 issue 5 p. Most of the tumors arose from the major salivary glands or the. Department of oral and maxillofacial pathology terna dental. Adenoid cystic carcinoma of the head and neck quality in. Adenoid cystic carcinoma of the maxilla the value of. Patients usually complain of a constant, lowgrade, dull ache, which gradually increase in intensity.
They are the most common sinonasal tumors of salivary origin. You will read about how to cope with challenges in everyday life after a cancer diagnosis. Adenoid cystic carcinoma adcc is one of the most common salivary gland malignancies and the long. The excision shows an adenoid cystic carcinoma, which is a rare subtype of mammary carcinoma representing adenoid cystic carcinoma. It is notorious for its poor disease free survival due to frequent local recurrences and often indolent distant metastases. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses. Pembrolizumab for the treatment of advanced salivary gland. It is slow growing but a highly invasive cancer with a high recurrence rate. Jul 20, 2016 adenoid cystic carcinoma acc is a rare malignant tumor of the salivary glands, with higher frequency in the submandibular gland, a prolonged clinical course, and poor long. Adenoid cystic carcinoma is an uncommon salivary gland tumour that. Salivary gland tumours or neoplasms are tumours that form in the tissues of salivary glands. The major salivary glands consist of the parotid, submandibular, and sublingual glands. Pdf salivary gland tumor management requires longterm.
Adenoid cystic carcinoma is an uncommon salivary gland tumor that may arise in a wide variety of anatomical sites in the head and neck, often with an advanced stage at diag nosis. Their recognition is important particularly when the. Highgrade tumors include adenoid cystic carcinoma, highgrade mucoepidermoid carcinoma, carcinoma expleomorphic adenoma, squamous cell carcinoma, and adenocarcinoma. Facial nerve paralysis may develop in parotid tumors. Understanding acc adenoid cystic carcinoma research.
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