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. We retrospectively studied 35 operations in 34 patients who had had a pathologic diafnosis of a cc of the salivary glands and who had been treated over a 20year period and followed for a. 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. Salivary gland carcinomas are notoriously resistant to therapy and no standard of care exists.
Systemic therapy for recurrent or metastatic salivary gland. Recurrentmetastatic rm accs are generally incurable, due to a lack of active systemic therapies. Adenoid cystic carcinoma of submandibular gland metastatic to. Familial adenoid cystic carcinoma of sublingual salivary.
Expression of runx3 in salivary adenoid cystic carcinoma. It is the fifth most common salivary gland malignancy 10 and accounts for approximately 10% of all salivary gland neoplasms3, 8, 11, 12. Survival from salivary glands adenoid cystic carcinoma in european populations. The neck and head are the most common sites of occurrence of this type of cancer. Adenoid cystic carcinoma definition of adenoid cystic. The sample consisted of 44 cases of acc diagnosed between 2008 and 2016 at a. It most commonly arises in the major and minor salivary glands of the head and neck. Craniofacial pain symptoms are often complex in nature and can represent a variety of unrelated conditions.
Adenoid cystic carcinoma of salivary glands diagnostic and. Histology adenoid cystic carcinoma is usually low grade but has often associated with pni and distant metastases are common carcinoma ex pleomorphic adenoma this case is a carcinomatous transformation within a primary or recurrent pleomorphic adenoma. Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. A form of cancer, carcinoma makes up the majority of the cases of malignancy of the breast, uterus, intestinal tract, skin, and tongue. You will read about how to cope with challenges in everyday life after a cancer diagnosis. Adenoid cystic carcinoma acc is a rare tumor accounting for approximately less than 1% of all malignant tumors of the head and neck. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. A fragment of parotid is present in one corner large arrow with tumor double arrows invading the thick fibrous parotid capsule.
Like most cancers, acc appears to develop from genetic changes that are caused by something in a persons environment. Complementary histoenzymological investigations and electron microscopic study were performed on specimens from 7 and patients respectively. Adenoid cystic carcinoma of salivary glands diagnostic. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. They are the most common sinonasal tumors of salivary origin. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. But other factors, such as the grade of the cancer how likely it is to grow and spread quickly. Carcinomas of the major salivary glands constitute a heterogeneous group of rare malignant neoplasms, accounting for less than 5% of newly diagnosed head and neck cancers. These are under your tongue and on each side of your jaw below the jawbone.
Whilst over 70% of cases originate from within the salivary glands, up to 20% of cases arise within the. Local control after treatment was determined for all patients and analyzed with respect to extent of surgery and dose of radiation. Pdf management of salivary gland adenoid cystic carcinoma. The underlying cause of adenoid cystic carcinoma acc is not yet known, but it appears to develop from noninherited, genetic changes that occur during a persons lifetime. Of all salivary gland tumors only about 10% to 15% are of minor salivary gland origin. There are approximately 1,200 new cases of acc diagnosed each. 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. Dec 22, 2017 adenoid cystic carcinoma acc is a rare type of cancer mainly located within secretory glands, most commonly the minor but also the major salivary glands of the head and neck. 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. Primary cutaneous adenoid cystic carcinoma acc is a rare tumor, with less than 50 cases reported to date. Adenoid cystic carcinoma, external auditory canal, outer onehalf. Original article prognostic value of quantitative p63. 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.
Outcomes of patients with adenoid cystic carcinoma of the. They are locally aggressive with a propensity for perineural spread 5. Agulnik m, cohen ew, cohen rb, chen ex, vokes ee, hotte sj, winquist e, laurie s, hayes dn, dancey je, et al. Salivary gland neoplasms constitute 3% of cancers of all. The distinctive histologic appearance is the main characteristic of this type of neoplasm. Hybrid salivary gland carcinoma, adenoid cystic carcinoma component a note small cells with scant cytoplasm in solid and microcystic growth patterns. 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. The sample consisted of 44 cases of acc diagnosed between 2008 and 2016 at a reference hospital in oncology. During surgery, the surgeon will remove the tumor and an area of healthy tissue surrounding it, called a margin. An unusual presentation of adenoid cystic carcinoma of the. Adenoid cystic carcinoma of submandibular gland metastatic. Major or minor salivary glands may be involved, presenting as single or multiple masses. The tumors have a notable tendency for perineural spread. Review and updates of immunohistochemistry in selected.
These cancers are small and still within the salivary gland. Adenoid cystic carcinoma minor salivary gland origin. These genetic changes are present only in the cancer cells, not in the cells with the genetic material that is passed on to offspring the egg and sperm cells. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Histologic and ultrastructural features of adenoid cystic. A case report294 acc cases occur in the salivary glands, among which acc of the minor salivary gland has the worst prognosis. Just a few from a long list include cardiac ischemia and acute myocardial infarction, 1 ernest syndrome and temporal tendonitis, 25 temporomandibular joint pain, and salivary gland adenoid cystic carcinoma acc. Adenoid cystic carcinoma of the parotid gland ncbi. It is slow growing but a highly invasive cancer with a high recurrence rate. Pdf salivary gland tumor management requires longterm followup because of tumor indolence and possible late recurrence and distant. Adenoid cystic carcinoma acc is a rare type of cancer mainly located within secretory glands, most commonly the minor but also the major salivary glands of the head and neck. Despite its slow growth, it has a grave prognosis characterized by frequent local recurrences, distant. First described in 1853,1 adenoid cystic carcinoma.
Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to. There were 25 men and 25 women with a mean age of 52 years age range, 21 to 88 years. Each year, about 1200 people are diagnosed with acc in the united states. Adenoid cystic carcinoma genetic and rare diseases. Cases 3 and 4, and the consultation files of 1 of the. Adenoid cystic carcinoma is among the most frequent malignancies in the salivary and lacrimal glands and has a grave prognosis characterized by frequent local recurrences, distant metastases, and. Adenoid cystic carcinoma causes, symptoms, treatment. Pathology adenoid cystic carcinomas are generally considered low grade 4. Adenoid cystic carcinoma organization international faq. Adenoid cystic carcinoma acc is a rare malignancy most often affecting the salivary glands.
Minor salivary gland carcinoma consider the lip and oral cavity, nasal cavity and paranasal sinuses. Mucoepidermoid carcinoma adenoid cystic carcinoma polymorphous lowgrade adenocarcinoma epithelialmyoepithelial carcinoma clear cell carcinoma, nos basal cell adenocarcinoma malignant sebaceous tumors cystadenocarcinoma lowgrade cribriform cystadenocarcinoma mucinous adenocarcinoma oncocytic carcinoma salivary duct. Adenoid cystic carcinoma acc is a tumour of mucous secreting glandular cells 12 representing less than 1% of all malignant head and neck tumours3, 11, 19. Adenoid cystic carcinoma acc of salivary gland comprises approximately 10% of all epithelial salivary gland tumors. A retrospective analysis was performed of 50 patients with adenoid cystic carcinoma who were seen in the department of radiation oncology, university of witwatersrand, johannesburg, south africa, in the past 10 years. Jun 21, 2017 adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues.
Adenoid cystic carcinoma nord national organization for. Understanding acc adenoid cystic carcinoma research. Nine normal adult salivary glands and seven adenoid cystic carcinomas frozen tissues were obtained in 2007, and 73 formalin. Comprehensive skin examination and appropriate follow. Dec 23, 2012 adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. Prognostic value of quantitative p63 immunostaining in. 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. Adenoid cystic carcinoma is a rare salivary glandtype tumor that arises in exocrine glands including the salivary, lacrimal, mammary, and bronchial glands. Diagnostic accuracy of 18 ffdg petct and mr imaging in.
Adenoid cystic carcinoma acc is a rare malignant neoplasm of the salivary gland. Thompson and others published salivary gland adenoid cystic carcinoma find, read and cite all the. Adenoid cystic carcinoma, parotid, capsular invasion. Case report liver metastasis from adenoid cystic carcinoma. Basaloid squamous cell carcinoma of the esophagus with or. Publications home of jama and the specialty journals of. Sep 04, 2019 adenoid cystic carcinoma acc is a rare malignancy arising in salivary glands and other sites, characterized by high rates of relapse and distant spread. Adenoid cystic carcinoma acc is a rare malignancy arising in salivary glands and other sites, characterized by high rates of relapse and distant spread. Due to the rarity of these malignancies, various histologies, and wide ranging clinical behavior it has been difficult to standardize systemic therapy.
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, salivary glands of springerlink. Patients suffering from adenoid cystic salivary gland carcinoma have variable prognosis and longterm follow up is recommended in 35 years follow up it was noted that of those patients suffering from adenoid cystic carsinoma acc of salivary glands, 54% of major salivary glands and 42% of minor salivary glands carcinoma patients were alive 10. View a short video led by an asco expert that provides information about what comes next after finishing treatment. It tends to metastasize to lungs, bone, liver, brain, and rarely to skin.
Basaloid squamous cell carcinoma bscc is an uncommon variant of squamous cell carcinoma that most commonly affects the upper aerodigestive tract in the region of the hypopharynx, the oral cavity, and the larynx. Adenoid cystic carcinoma is a basaloid tumor composed of epithelial and myoepithelial cells arranged in variable morphologic configurations, including tubular, cribriform, and solid patterns elnaggar and huvos 2005. The tumor extends well beyond the visible and palpable limits of the salivary gland. Liver metastasis from adenoid cystic carcinoma of the submandibular gland. The booklet is available as a pdf, so it is easy to print out. It can also occur in the breast, uterus, or other locations in the body.
Among 117 adenoid cystic carcinomas, 86 were located in minor salivary glands and were selected for a clinicopathological analysis. It is the most common malignant neoplasm observed in the major and minor salivary glands. Adenoid cystic carcinoma manifests as a slowly growing mass often accompanied by pain and, in some cases, by facial paralysis rousseau and badoual 2010. Adcc usually affects the major as well as the minor salivary glands. Adenoid cystic carcinoma acc is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region.
Distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during followup. Adenoid cystic carcinoma of the salivary glands radiology. Adenoid cystic carcinoma of the parotid metastasizing to liver. Intracranial acc is even more infrequent and could be primary or secondary occurring either by direct invasion, hematogenous spread, or perineural spread. Adenoid cystic carcinomas of the salivary gland, lacrimal. Jun 21, 2017 adenoid cystic carcinoma acc is not considered to be an inherited cancer. A rare presentation of adenoid cystic carcinoma of parotid. Independent prognostic predictors include ajcc tnm staging, margin status, high mitotic index defined as. Histogenesis of adenoid cystic carcinoma of the salivary glands.
It may involve the parotid, submandibular, or minor salivary glands. Genetic hallmarks of recurrentmetastatic adenoid cystic. The treatment options for salivary gland cancer depend largely on the stage extent of the cancer. A nodule structurally resembling a benign mixed tumor of salivary gland origin material within cystic spaces characterizing the tumor x140. Systemic therapy for recurrent or metastatic salivary. Adenoid cystic carcinoma radiology reference article. 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. The behavior of adenoid cystic carcinoma acc of lhe salivary glands has been shown to be ttnpredictable in lenns of local and distant spread and mortality. Adenoid cystic carcinoma of the submandibular gland. The cellular composition of adenoid cystic carcinoma is similar to that in the terminal. Adenoid cystic carcinoma of salivary glands diagnostic and prognostic factors and treatment outcome karoliina hirvonen academic dissertation to be presented, with the permission of the faculty of medicine, university of helsinki, for public examination in the richard faltin lecture hall at surgical hospital, kasarmikatu 11.
Report of a case of adenoid cystic and mucoepidermoid carcinoma, an unusual combination 3 of 6 figure 4 fig. Management of salivary gland adenoid cystic carcinoma scielo. Risk factors and prognosis for salivary gland adenoid cystic. Adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. 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. An important feature of accs is the long clinical course with a high rate of distant metastases. Salivary gland tumors may cause painless masses in the mouth or face. Dedifferentiation occurring in adenoid cystic carcinoma of the tongue. Adenoid cystic carcinoma tends to metastasize to lungs, bone, liver, and brain.
Adenoid cystic carcinoma are relatively less common type of salivary gland tumour including parotid gland, and is the only histological type having propensity to spread distantly by lymphohematogenous and other classical perineural spread. 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. Adenoid cystic carcinomas are a rare histological subtype of adenocarcinoma. Adenoid cystic carcinomas are only poorly encapsulated at best. Adenoid cystic carcinomas arise more commonly in the minor salivary glands 55% than in the major salivary glands. Phase ii study of lapatinib in recurrent or metastatic epidermal growth factor receptor andor erbb2 expressing adenoid cystic carcinoma and non adenoid cystic carcinoma malignant tumors of the salivary glands. Adenoid cystic carcinoma as a primary salivary gland tumor arising in bronchus. The preferential sites of metastases are the lung and bone, followed by the brain and liver. 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. Pdf salivary gland adenoid cystic carcinoma researchgate.
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