Among 117 adenoid cystic carcinomas, 86 were located in minor salivary glands and were selected for a clinicopathological analysis. The sample consisted of 44 cases of acc diagnosed between 2008 and 2016 at a reference hospital in oncology. Understanding acc adenoid cystic carcinoma research. 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. Of all salivary gland tumors only about 10% to 15% are of minor salivary gland origin. 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.
Despite its slow growth, it has a grave prognosis characterized by frequent local recurrences, distant. Diagnostic accuracy of 18 ffdg petct and mr imaging in. 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. Dedifferentiation occurring in adenoid cystic carcinoma of the tongue. Adenoid cystic carcinoma of the salivary glands radiology. 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 manifests as a slowly growing mass often accompanied by pain and, in some cases, by facial paralysis rousseau and badoual 2010. 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. But other factors, such as the grade of the cancer how likely it is to grow and spread quickly. 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. Local control after treatment was determined for all patients and analyzed with respect to extent of surgery and dose of radiation. Adenoid cystic carcinoma of salivary glands diagnostic and. 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.
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. 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. Thompson and others published salivary gland adenoid cystic carcinoma find, read and cite all the. Adenoid cystic carcinoma as a primary salivary gland tumor arising in bronchus. The distinctive histologic appearance is the main characteristic of this type of neoplasm. Adenoid cystic carcinoma of the parotid metastasizing to liver.
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 malignant tumor of the salivary glands, with higher frequency in the submandibular gland, a prolonged clinical course, and poor long. 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. 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. They are the most common sinonasal tumors of salivary origin. 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. Major or minor salivary glands may be involved, presenting as single or multiple masses. Case report liver metastasis from adenoid cystic carcinoma. Adenoid cystic carcinoma, external auditory canal, outer onehalf. 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. Complementary histoenzymological investigations and electron microscopic study were performed on specimens from 7 and patients respectively. Management of salivary gland adenoid cystic carcinoma scielo. There were 25 men and 25 women with a mean age of 52 years age range, 21 to 88 years. 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.
An unusual presentation of adenoid cystic carcinoma of the. Due to the rarity of these malignancies, various histologies, and wide ranging clinical behavior it has been difficult to standardize systemic therapy. Adenoid cystic carcinoma, salivary glands of springerlink. Adenoid cystic carcinoma tends to metastasize to lungs, bone, liver, and brain. Adenoid cystic carcinoma acc of salivary gland comprises approximately 10% of all epithelial salivary gland tumors. Adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body.
Primary cutaneous adenoid cystic carcinoma acc is a rare tumor, with less than 50 cases reported to date. A fragment of parotid is present in one corner large arrow with tumor double arrows invading the thick fibrous parotid capsule. Adenoid cystic carcinoma minor salivary gland origin. Independent prognostic predictors include ajcc tnm staging, margin status, high mitotic index defined as. Histologic and ultrastructural features of adenoid cystic.
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. The cellular composition of adenoid cystic carcinoma is similar to that in the terminal. 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. Review and updates of immunohistochemistry in selected. You will read about how to cope with challenges in everyday life after a cancer diagnosis. The tumors have a notable tendency for perineural spread. 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. Dec 23, 2012 adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. 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. Risk factors and prognosis for salivary gland adenoid cystic. Recurrentmetastatic rm accs are generally incurable, due to a lack of active systemic therapies.
A nodule structurally resembling a benign mixed tumor of salivary gland origin material within cystic spaces characterizing the tumor x140. Craniofacial pain symptoms are often complex in nature and can represent a variety of unrelated conditions. It is the fifth most common salivary gland malignancy 10 and accounts for approximately 10% of all salivary gland neoplasms3, 8, 11, 12. Adenoid cystic carcinoma of the submandibular gland.
The tumor extends well beyond the visible and palpable limits of the salivary gland. A rare presentation of adenoid cystic carcinoma of parotid. Salivary gland carcinomas are notoriously resistant to therapy and no standard of care exists. 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 genetic and rare diseases. Adenoid cystic carcinoma causes, symptoms, treatment.
Adenoid cystic carcinoma of submandibular gland metastatic. It is slow growing but a highly invasive cancer with a high recurrence rate. They are locally aggressive with a propensity for perineural spread 5. Histogenesis of adenoid cystic carcinoma of the salivary glands. Comprehensive skin examination and appropriate follow. 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. It most commonly arises in the major and minor salivary glands of the head and neck. 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. Intracranial acc is even more infrequent and could be primary or secondary occurring either by direct invasion, hematogenous spread, or perineural spread. The treatment options for salivary gland cancer depend largely on the stage extent of the cancer. Adenoid cystic carcinoma radiology reference article. Basaloid squamous cell carcinoma of the esophagus with or.
Adenoid cystic carcinomas of the salivary gland, lacrimal. During surgery, the surgeon will remove the tumor and an area of healthy tissue surrounding it, called a margin. 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. The preferential sites of metastases are the lung and bone, followed by the brain and liver. Adenoid cystic carcinoma definition of adenoid cystic. Systemic therapy for recurrent or metastatic salivary. 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. Genetic hallmarks of recurrentmetastatic adenoid cystic. Nine normal adult salivary glands and seven adenoid cystic carcinomas frozen tissues were obtained in 2007, and 73 formalin.
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. Adenoid cystic carcinoma organization international faq. Jun 21, 2017 adenoid cystic carcinoma acc is not considered to be an inherited cancer. Adenoid cystic carcinoma acc is a rare malignancy arising in salivary glands and other sites, characterized by high rates of relapse and distant spread. 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. Original article prognostic value of quantitative p63. 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, parotid, capsular invasion. These are under your tongue and on each side of your jaw below the jawbone. Publications home of jama and the specialty journals of. Pdf salivary gland adenoid cystic carcinoma researchgate. 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. Adenoid cystic carcinomas arise more commonly in the minor salivary glands 55% than in the major salivary glands. Prognostic value of quantitative p63 immunostaining in. Adenoid cystic carcinoma is a rare salivary glandtype tumor that arises in exocrine glands including the salivary, lacrimal, mammary, and bronchial glands. Salivary gland neoplasms constitute 3% of cancers of all. 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 booklet is available as a pdf, so it is easy to print out. View a short video led by an asco expert that provides information about what comes next after finishing treatment. Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to. First described in 1853,1 adenoid cystic carcinoma. A case report294 acc cases occur in the salivary glands, among which acc of the minor salivary gland has the worst prognosis. Survival from salivary glands adenoid cystic carcinoma in european populations. There are approximately 1,200 new cases of acc diagnosed each.
Adcc usually affects the major as well as the minor salivary glands. 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. 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. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Adenoid cystic carcinoma acc is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region. Minor salivary gland carcinoma consider the lip and oral cavity, nasal cavity and paranasal sinuses. 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. 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. Distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during followup. Hybrid salivary gland carcinoma, adenoid cystic carcinoma component a note small cells with scant cytoplasm in solid and microcystic growth patterns. Expression of runx3 in salivary adenoid cystic carcinoma.
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. Report of a case of adenoid cystic and mucoepidermoid carcinoma, an unusual combination 3 of 6 figure 4 fig. Jun 21, 2017 adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. The changes may be caused by exposures in a persons. A form of cancer, carcinoma makes up the majority of the cases of malignancy of the breast, uterus, intestinal tract, skin, and tongue. Adenoid cystic carcinoma acc is a rare malignant neoplasm of the salivary gland. These cancers are small and still within the salivary gland. Cases 3 and 4, and the consultation files of 1 of the. Pdf management of salivary gland adenoid cystic carcinoma. Adenoid cystic carcinoma acc is a rare malignancy most often affecting the salivary glands. The sample consisted of 44 cases of acc diagnosed between 2008 and 2016 at a.
Outcomes of patients with adenoid cystic carcinoma of the. Adenoid cystic carcinoma of the parotid gland ncbi. Adenoid cystic carcinoma acc is a rare tumor accounting for approximately less than 1% of all malignant tumors of the head and neck. Pathology adenoid cystic carcinomas are generally considered low grade 4. The neck and head are the most common sites of occurrence of this type of cancer. Each year, about 1200 people are diagnosed with acc in the united states. Agulnik m, cohen ew, cohen rb, chen ex, vokes ee, hotte sj, winquist e, laurie s, hayes dn, dancey je, et al. Adenoid cystic carcinoma of submandibular gland metastatic to. Adenoid cystic carcinoma of salivary glands diagnostic.
Whilst over 70% of cases originate from within the salivary glands, up to 20% of cases arise within the. Adenoid cystic carcinomas are only poorly encapsulated at best. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. Adenoid cystic carcinomas are a rare histological subtype of adenocarcinoma. Familial adenoid cystic carcinoma of sublingual salivary. Like most cancers, acc appears to develop from genetic changes that are caused by something in a persons environment. It tends to metastasize to lungs, bone, liver, brain, and rarely to skin. It is the most common malignant neoplasm observed in the major and minor salivary glands. Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. It can also occur in the breast, uterus, or other locations in the body. Liver metastasis from adenoid cystic carcinoma of the submandibular gland. 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. It may involve the parotid, submandibular, or minor salivary glands.