Adenoid cystic carcinoma, salivary glands of springerlink. Mitochondrial mutations in adenoid cystic carcinoma of the. Adenoid cystic carcinoma is a distinctive neoplasm of the salivary gland and is the most common malignant tumor of submandibular gland. The preferential sites of metastases are the lung and bone, followed by the brain and liver. Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to. Introduction adenoid cystic carcinoma acc is a rare malignant tumor arising from salivary glands of head and neck region. 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 acc is a malignant neoplasm of salivary glands that, with slow growth and infiltrative tumor, clinically is characterized by a firm mass. The authors report a complete remission following treat ment with 5fluorouracil, adriamycin doxorubicin. Recurrent or metastatic adenoid cystic carcinoma rm acc is a malignant neoplasm of predominantly salivary gland origin for which effective therapies are lacking. Adenoid cystic carcinoma of the head and neck quality in.
Local recurrence and distant metastases are also very rare. It can also occur in the breast, uterus, or other locations in the body. Pdf akt3 drives adenoid cystic carcinoma development in. Adenoid cystic carcinoma of salivary origin the american journal. 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. Adenoid cystic carcinoma of salivary gland with mandible.
Pdf adenoid cystic carcinoma of parotid salivary glanda. Data were obtained from the national cancer data base ncdb. A 49yearold brown female patient sought dental care with complaints of headaches and nasal congestion over 10 months. Adenoid cystic carcinoma kidtik the breast constitutes approximately 0. Adenoid cystic carcinoma of salivary glands diagnostic. Adenoid cystic carcinoma genetic and rare diseases.
Adenoid cystic carcinoma of the minor salivary glands oral surgery. These are under your tongue and on each side of your jaw below the jawbone. We conducted a phase ii trial evaluating the multitargeted tyrosine kinase inhibitor lenvatinib in patients with rm acc. Background adenoid cystic carcinomas accs are rare malignant neoplasms of exocrine glands that account for up to 10%20% of all salivary gland tumours. Adcc usually affects the major as well as the minor salivary glands.
A concern with all surgeries of the parotid gland is that the surgery may damage facial nerves, which can cause the muscles in the face to droop. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. Adenoid cystic carcinoma of the submandibular gland is a slowgrowing, insidious lesion with a prolonged natural history that includes a tendency toward local recurrence and distant metastasis. Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. Adenoid cystic carcinoma manifests as a slowly growing mass often accompanied by pain and, in some cases, by facial paralysis rousseau. 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. 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. They are locally aggressive with a propensity for perineural spread 5. Adenoid cystic carcinoma of the parotid gland 179 a b fig. Adenoid cystic carcinoma causes, symptoms, treatment. Perzin k h, gullane p, clairmont ag 1978 adenoid cystic carcinomas arising in salivary glands. Adenoid cystic carcinoma as a primary salivary gland tumor arising in bronchus.
They are the most common sinonasal tumors of salivary origin. Due to the lack of understanding of the molecular mechanisms in. Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate. 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. Cervical lymph node metastasis in adenoid cystic carcinoma. Pembrolizumab for the treatment of advanced salivary gland. The most common subtype of sgc was adenocarcinoma 38%, followed by mucoepidermoid 12%, undifferentiated 8%, squamous cell 8%, and adenoid cystic 8% carcinoma. The concept of cure may be difficult to assess until the patient is disease free for 10 years or longer.
In 68 others 28 per cent the lesion in volved the parotid or submandibular glands. Adenoid cystic carcinomas of minor salivary glands. The results were correlated to clinicopathological profile of the patients. Right parotidectomy was recommended as a definitive course of action, and the patient underwent surgery in october 2010. In order to study this tumor type, a reliable model system exhibiting the molecular features of. Their recognition is important particularly when the component tumours have different biological behaviours. Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to local recurrence and late distant. 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. Management of recurrent, advanced, or persistent disease with hyperthermia and radiation therapy. 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. June 2017 includes ptnm requirements from the 8th edition, ajcc staging manual. 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.
Facial nerve sacrifice and radiotherapy in parotid adenoid cystic carcinoma. Systemic therapy for recurrent or metastatic salivary. While initial presentation specifics are unavailable, the patient was evaluated and found to have a 2 x 2 centimeter mass in the right parotid gland. Adenoid cystic carcinoma acc is a rare salivary gland malignant neoplasm.
The incidence is relatively rare and the optimal treatment strategy is still unclear. Phase ii study of lenvatinib in patients with progressive. Adenoid cystic carcinomas arise more commonly in the minor salivary glands 55% than in the major salivary glands. This ability enables an adenoid cystic carcinoma to extend along the nerves and spread long distances. A multiplex preclinical model for adenoid cystic carcinoma. Review and updates of immunohistochemistry in selected.
An important feature of accs is the long clinical course with a high rate of distant metastases. Pdf salivary gland adenoid cystic carcinoma researchgate. What are some symptoms of adenoid cystic carcinoma. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. The site of origin was thought to be the sublingual gland in three instances. Local control after treatment was determined for all patients and analyzed with respect to. Here, an asymptomatic acc case presented with extensive pm and local bone erosion was reported. For accreditation purposes, this protocol should be used for the following procedures and tumor types. Adenoid cystic carcinoma of parotid salivary glanda case. Adenoid cystic carcinoma adcc is one of the most common salivary gland malignancies and the long. Salivary gland carcinomas are notoriously resistant to therapy and no standard of care exists. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. Histologic grading of adenoid cystic carcinoma of the salivary gland. Adenoid cystic carcinoma of the submandibular gland.
Adenoid cystic carcinomas of the salivary gland, lacrimal. Adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. Thompson and others published salivary gland adenoid cystic carcinoma find, read and cite all the. Adenoid cystic carcinoma acc of salivary gland origin is rare. Adenoid cystic carcinoma, parotid gland, malignant salivary gland tumor 1.
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. Its innoceous clinical presentation remains a diagnostic challenge. Pulec jl, parkhill em, devine kd 1963 adenoid cystic carcinoma of external auditory canal. There are approximately 1,200 new cases of acc diagnosed each.
The eurocare data provide a good opportunity to study the survival of this uncommon cancer in a large population. Adenoid cystic carcinoma is among the most frequent malignancies in the salivary and lacrimal gland, and is clinically characterized by. This type of neoplasm was first elaborated by billroth in 1856 as a benign neoplasm and was called cylindroma for cribriform. Case report in october 1980, a 29yearold white woman was referred. Adenoid cystic carcinoma acc of the minor salivary glands is rare and presents as a unique subset of minor salivary gland tumors msgts. Adenoid cystic carcinoma of the head and neck literature. Surgical management of adenoid cystic carcinoma in the parotid gland. Minor salivary gland adccs seem to have a worse prognosis than the major salivary glands ones.
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 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. Salivary gland carcinomas sgcs are rare, accounting for carcinoma, and salivary duct carcinoma. This study was conducted with a twostage minimax design. Pdf adenoid cystic carcinomas of minor salivary glands. Predictors of outcome in adenoid cystic carcinoma of. Early cutaneous metastasis of adenoid cystic carcinoma of. Adenoid cystic carcinoma of the parotid gland medind. Adenoid cystic carcinoma stages up adenoid cystic carcinoma. There were 25 men and 25 women with a mean age of 52 years age range, 21 to 88 years. 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 acc is a rare tumor, which is the most common malignancy that occurs in the salivary glands. 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. It is the thirdmost common malignant salivary gland tumor overall.
Understanding acc adenoid cystic carcinoma research. Publications home of jama and the specialty journals of. The classification applies only to carcinomas of the major salivary glands. 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. 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. A rare case of adenoid cystic carcinoma of ceruminous.
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. Malignant salivary gland tumors of the base of the tongue. Pdf adenoid cystic carcinoma of the submandibular gland. It most commonly arises in the major and minor salivary glands of the head and neck. Adenoid cystic carcinoma of the sublingual gland imaging science. The neck and head are the most common sites of occurrence of this type of cancer. Protocol for the examination of specimens from patients with carcinomas of the major salivary glands.