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. Adenoid cystic carcinoma, perineural invasion adenoid cystic carcinoma ihc ckit and bcl2 positive epithelial cells. Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to. The eurocare data provide a good opportunity to study the survival of this uncommon cancer in a large population. Review and updates of immunohistochemistry in selected. 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. Adenoid cystic carcinoma is the most common malignancy of the minor salivary glands, and its biological behaviour is characterised by slow and indolent growth. For adenoid cystic carcinoma, the distant metastasis rate is nearly 50%. 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. Thompson and others published salivary gland adenoid cystic carcinoma find, read and cite all the. Systemic therapy in the management of metastatic or. During surgery, the surgeon will remove the tumor and an area of healthy tissue surrounding it, called a margin.
Pdf akt3 drives adenoid cystic carcinoma development in. Adenoid cystic carcinoma of salivary glands diagnostic. Adenoid cystic carcinoma of the parotid gland medind. Adenoid cystic carcinoma is a tumour of glandular cells responsible for 10% of salivary gland neoplasms. The preferential sites of metastases are the lung and bone, followed by the brain and liver. Adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. Adenoid cystic carcinoma acc is the second most common primary salivary gland epithelial malignancy, comprising approximately 10% of all salivary gland neoplasms and 0. Adenoid cystic carcinoma acc is the second most common malignant tumor of the major salivary glands, accounting for approximately 22%. There are approximately 1,200 new cases of acc diagnosed each. Adenoid cystic carcinoma acc of salivary gland origin is rare. It most commonly arises in the major and minor salivary glands of the head and neck. It includes approximately 10% of all salivary neoplasms and 30% of minor salivary gland tumors. Adcc usually affects the major as well as the minor salivary glands. Adenoid cystic carcinoma of the sublingual gland imaging science.
Adenoid cystic carcinoma acc of the minor salivary glands is rare and presents as a unique subset of minor salivary gland tumors msgts that. Pdf outcomes of patients with adenoid cystic carcinoma. Adenoid cystic carcinoma of the head and neck quality in. Salivary gland cancer is an aggressive and painful cancer, but a rare tumor type accounting for only 0. 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.
Adenoid cystic carcinoma of submandibular gland metastatic. Mitochondrial mutations in adenoid cystic carcinoma of the. They are the most common sinonasal tumors of salivary origin. Adenoid cystic carcinoma manifests as a slowly growing mass often accompanied by pain and, in some cases, by facial paralysis rousseau and badoual 2010.
Histologic grading of adenoid cystic carcinoma of the salivary glands philip a. A rare case of adenoid cystic carcinoma of ceruminous. Understanding acc adenoid cystic carcinoma research. These are under your tongue and on each side of your jaw below the jawbone. Pdf adenoid cystic carcinomas of minor salivary glands.
It can be followed by pain, due to the trend for perineural invasion. White, phdt seventynine patients with adenoid cystic carcinoma arising in salivary glands were studied to determine. Salivary gland tumors may cause painless masses in the mouth or face. Twentyfour percent to 52% of accs of the salivary glands result in distant metastasis, primarily involving the lungs, bones, liver, and brain. Adenoid cystic carcinoma causes, symptoms, treatment. Acc adenoid cystic carcinoma is a rare and unique form of cancer that is known to be unpredictable in nature, with a typical growth pattern of being slow and gradual, but over time can be progressive, insidious and relentless. Here, an asymptomatic acc case presented with extensive pm and local bone erosion was reported. Adenoid cystic carcinoma is a malignant tumor with a. 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. The site of origin was thought to be the sublingual gland in three instances.
Perzin k h, gullane p, clairmont ag 1978 adenoid cystic carcinomas arising in salivary glands. Adenoid cystic carcinoma is a slowgrowing but aggressive neoplasm with a remarkable capacity for recurrence. Histologic grading of adenoid cystic carcinoma of the salivary gland. Overall, adenoid cystic carcinoma acc is a rare tumor, accounting for only 1% of all malignant tumors of the oral and maxillofacial region. Minor salivary gland adccs seem to have a worse prognosis than the major salivary glands ones. A surgical oncologist is a doctor who specializes in treating cancer using surgery. The neck and head are the most common sites of occurrence of this type of cancer. Tumors of the salivary gland exhibit heterogeneous histologic and genetic features and they are subdivided into different subtypes, with adenoid cystic carcinomas acc being one of the most abundant. 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. 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. Adenoid cystic carcinoma organization international. Adenoid cystic carcinoma is a rare salivary glandtype tumor that arises in exocrine glands including the salivary, lacrimal, mammary, and bronchial glands. It has been described as one of the most biologically destructive and unpredictable tumours of the head and neck conley, 1975.
In 44 cases the tumour arose in the major salivary glands and, of these, 77% had an incomplete surgical excision and 23% had only a tumour biopsy before radiotherapy. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. Adenoid cystic carcinoma of the submandibular gland. The distinctive histologic appearance is the main characteristic of this type of neoplasm. Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate. In 68 others 28 per cent the lesion in volved the parotid or submandibular glands. It is the most common malignant neoplasm observed in the major and minor salivary glands.
Adenoid cystic carcinoma of salivary origin the american journal. The oral area includes the major and minor salivary glands, roof of mouth palate, floor of mouth, gums, tongue. Adenoid cystic carcinomas of the salivary glands are rare head and neck malignancies, characterized by a slow but aggressive and destructive growth. Adenoid cystic carcinoma of the minor salivary glands oral surgery. Performance characteristics of adenoid cystic carcinoma of. 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.
It can also occur in the breast, uterus, or other locations in the body. Early cutaneous metastasis of adenoid cystic carcinoma of. Adenoid cystic carcinoma acc is a rare tumor, which is the most common malignancy that occurs in the salivary glands. Predictors of outcome in adenoid cystic carcinoma of. A single report has demonstrated scattered mitochondria and a paucity of endoplasmic reticulum in adenoid cystic carcinoma derived from minor salivary glands 29, though this was only a histologic analysis. Adenoid cystic carcinoma of intraoral minor salivary glands. Adenoid cystic carcinoma, salivary glands of springerlink. 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. Cervical lymph node metastasis in adenoid cystic carcinoma. They are locally aggressive with a propensity for perineural spread 5. Chemotherapy is generally reserved for the palliative treatment of symptomatic locally recurrent or metastatic disease that is not. Pulec jl, parkhill em, devine kd 1963 adenoid cystic carcinoma of external auditory canal.
Their recognition is important particularly when the. For accreditation purposes, this protocol should be used for the following procedures and tumor types. Adenoid cystic carcinoma is a rare, slowgrowing, salivary gland malignant tumor with poor longterm prognosis. Adenoid cystic carcinoma genetic and rare diseases. Akt3 drives adenoid cystic carcinoma development in. It tends to metastasize to lungs, bone, liver, brain, and rarely to skin. 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. June 2017 includes ptnm requirements from the 8th edition, ajcc staging manual. Adenoid cystic carcinoma occurs in the major salivary glands, as well as in the other mucous and serous glands ubiquitous in the oral cavity and upper respiratory tract.
Histologic grading of adenoid cystic carcinoma of the. Adenoid cystic carcinoma of the major salivary glands is associated with a significant prevalence of cervical nodemetastasis, andelectiveneck treatmentisindicated fort 3 andt 4 primarytumours, aswellastumours with other histological risk factors. 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. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. Independent prognostic predictors include ajcc tnm staging, margin status, high mitotic index defined as. Adenoid cystic carcinoma of intraoral minor salivary. Protocol for the examination of specimens from patients with carcinomas of the major salivary glands. Adenoid cystic carcinoma of salivary gland with mandible.
Local control after treatment was determined for all patients and analyzed with respect to extent of surgery and dose of radiation. Because the tumor is uncommon, any prospective study would be best done by a cooperative group, and should recognize the efficacy of adriamycin, and perhaps 5 fluorouracil, in the treatment of the disease. Adenoid cystic carcinoma acc of the salivary glands is a malignant tumor characterized by slow but relentless progression that is plagued by local recurrence, late metastases, and ultimately fatal outcomes. It has a high rate of perineural spread but limited involvement of regional lymphatics even in late stage disease. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. An important feature of accs is the long clinical course with a high rate of distant metastases. Comprehensive skin examination and appropriate follow. Adenoid cystic carcinoma of the major salivary glands is associated with a significant prevalence of cervical node metastasis, and elective neck treatment is indicated for t 3 and t 4 primary tumours, as well as tumours with other histological risk factors. Although patients with metastases from adenoid cystic carcinoma may survive longer than 10 years because of the slow growth of. Pdf salivary gland adenoid cystic carcinoma researchgate.
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