A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinoma of the salivary glands is the second most common malignancy involving the minor salivary glands behind mucoepidermoid carcinoma and the second most common malignancy involving the parotid gland. 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. An unusual presentation of adenoid cystic carcinoma of the.
Adenoid cystic carcinoma of salivary glands diagnostic. Adenoid cystic carcinoma of sublingual salivary gland obstructing. Currently, amifostine ethyol, a drug to protect tissues during radiation therapy. This study was conducted with a twostage minimax design. Adenoid cystic carcinoma acc is a relatively rare tumour of the salivary glands, accounting for approximately 5%10% of all salivary gland tumours. 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.
Adenoid cystic carcinoma of the breast sciencedirect. Adenoid cystic carcinoma is a rare, slowgrowing, salivary gland malignant tumor with poor longterm prognosis. Adenoid cystic carcinoma acc is a relatively rare epithelial tumor of the salivary glands that accounts for approximately 5 to 10% of all salivary gland neoplasms. Adenoid cystic carcinoma of parotid salivary glanda case study.
It most commonly arises in the major and minor salivary glands of the head and neck. Adenoid cystic carcinoma of the salivary gland sustained complete response to chemotherapy g. Adenoid cystic carcinoma the oral cancer foundation. 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. Performance characteristics of adenoid cystic carcinoma of. Salivary gland malignancies are morphologically and clinically varied neoplasms and account for approximately 57 % of head and neck cancers. Independent prognostic predictors include ajcc tnm staging, margin status, high mitotic index defined as. Latedeveloping tongue adenoid cystic carcinoma after.
Clinical presentation it often presents with orbital pain and paresthesia, since this type of tum. Salivary gland carcinomas are notoriously resistant to therapy and no standard of care exists. In the afip case files, more than 60% of tumors occurred in the mucosa of either the soft or hard palates, approximately 16% occurred in. Introduction adenoid cystic carcinoma acc is a rare malignant tumor arising from salivary glands of head and neck region. Adenoid cystic carcinoma of the parotid gland ncbi. Adenoid cystic carcinoma acc of the base of the tongue has been reported in the literature. Adenoid cystic carcinoma of salivary glands diagnostic and. Histogenesis of adenoid cystic carcinoma of the salivary glands. We have shown that this translocation results in a fusion in which the 3. Pdf salivary gland adenoid cystic carcinoma researchgate. Kolja freier, christa flechtenmacher, axel walch, sibylle ohl, frauke devens, bert burke, stefan hassfeld, peter lichter, stefan joos, christof hofelecopy number gains on 22q in adenoid cystic carcinoma of the salivary gland revealed by comparative genomic hybridization and tissue microarray analysis. 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. Treatment options for patients with unresectable or metastatic salivary gland carcinoma sgc are limited. These are under your tongue and on each side of your jaw below the jawbone.
During surgery, the surgeon will remove the tumor and an area of healthy tissue surrounding it, called a margin. Cervical lymph node metastasis in adenoid cystic carcinoma of. Approximately 50% of patients develop distant metastases and up to. Salivary gland cancers account for adenoid cystic carcinomas acc comprise. Adenoid cystic carcinoma of the salivary glands dr henry knipe and dr maxime stamant et al. Shallow whole genome sequencing of adenoid cystic carcinomas of the salivary glands identifies specific chromosomal aberrations related to tumor progression author links open overlay panel r. Procedure biopsy primary resection specimen with no residual cancer eg, following neoadjuvant therapy cytologic specimens. The neck and head are the most common sites of occurrence of this type of cancer. 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. The results are combined to determine the stage of cancer for each person. The histology specimen was examined and proved to be an adenoid cystic carcinoma of the deep lobe of the parotid gland.
Management of salivary gland adenoid cystic carcinoma scielo. Targeting the oncogenic transcriptional regulator myb in. There were 25 men and 25 women with a mean age of 52 years age range, 21 to 88 years. Adcc usually affects the major as well as the minor salivary glands. 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. Adenoid cystic carcinoma, parotid gland, malignant salivary gland tumor 1. Twentyfour percent to 52% of accs of the salivary glands result in distant metastasis, primarily involving the lungs, bones, liver, and brain. A form of cancer, carcinoma makes up the majority of the cases of malignancy of the breast, uterus, intestinal tract, skin, and tongue. The major salivary glands consist of the parotid, submandibular, and sublingual glands. Adenoid cystic carcinoma of the submandibular gland. Acc account for less than 1% of all malignant tumors in the larynx. Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate.
No studies have investigated mitochondrial mutation in salivary gland adenoid cystic carcinomas. Local control after treatment was determined for all patients and analyzed with respect to extent of surgery and dose of radiation. Among 117 adenoid cystic carcinomas, 86 were located in minor salivary glands and were selected for a clinicopathological analysis. Their recognition is important particularly when the.
Subsequently, an ent head and neck surgeon removed the tumor. In some cases, a drug to stimulate the parotid salivary gland and increase or restore saliva levels may be given. Salivary gland cancers account for oct 27, 2014 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. 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 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.
Adenoid cystic carcinoma of lacrimal glands radiology. Systemic therapy for recurrent or metastatic salivary. Adenoid cystic carcinoma acc is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region. One of the first carcinomaspecific translocations identified was the t6. Adenoid cystic carcinoma tends to metastasize to lungs, bone, liver, and brain. Background the treatment of patients with recurrent andor metastatic rm salivary gland adenoid cystic carcinoma acc remains an unmet. Dec 23, 2012 adenoid cystic carcinoma adcc is a very rare form of cancer which can occur in various parts of the body. Mammary analog secretory carcinoma is a recently described entity characterized by a t12. Other sites of origin include the trachea, lacrimal gland, breast, skin, and vulva. Adenoid cystic carcinomas of the salivary gland, lacrimal. Adenoid cystic carcinoma acc is the second most common malignant tumor of the major salivary glands, accounting for approximately 22%.
Akt3 drives adenoid cystic carcinoma development in. Complementary histoenzymological investigations and electron microscopic study were performed on specimens from 7 and patients respectively. 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. The minor salivary glands consist of 800 small mucussecreting glands located throughout the lining of the. Salivary gland cancer is an aggressive and painful cancer, but a rare tumor type accounting for only 0. Thompson and others published salivary gland adenoid cystic carcinoma find, read and cite all the. 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. Pembrolizumab for the treatment of advanced salivary gland. Adenoid cystic carcinoma radiology reference article. Adenoid cystic carcinoma of intraoral minor salivary glands. 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. Salivary gland tumours or neoplasms are tumours that form in the tissues of salivary glands. 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. The preferential sites of metastases are the lung and bone, followed by the brain and liver.
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. Cervical lymph node metastasis in adenoid cystic carcinoma. Health, general cancer care and treatment chemotherapy health aspects research cancer treatment lung tumors salivary gland diseases salivary glands diseases. We conducted a phase ii trial evaluating the multitargeted tyrosine kinase inhibitor lenvatinib in patients with rm acc. Adenoid cystic carcinoma acc is a malignant tumor that arises from the secretory epithelial cells of salivary glands of the head and neck and accounts for less than 1% of all head and neck malignancies and approximately 10% of all salivary neoplasms. Safety and efficacy of pembrolizumab for sgc expressing programmed death ligand 1 pdl1 were explored.
Pdf mitochondrial mutations in adenoid cystic carcinoma. The tumors have a notable tendency for perineural spread. 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. The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments. Adenoid cystic carcinomas acc of the salivary glands are challenging to understand, treat, and cure. Adenoid cystic carcinoma, salivary glands of springerlink. It is slow growing but a highly invasive cancer with a high recurrence rate. 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. Here are more details on each part of the tnm system. Recurrent or metastatic adenoid cystic carcinoma rm acc is a malignant neoplasm of predominantly salivary gland origin for which effective therapies are lacking. Pac cells may be arranged insingle files or narrow trabeculae forming concentric whorls targetoid appearance around nerves 16.
The aim of this study was to evaluate predictive factors for localregional lr recurrence, dm, and survival in acc. This cohort included 54 major salivary gland adenoid cystic carcinoma maacc patients and 68 minor salivary gland carcinoma acc miacc patients. Adenoid cystic carcinoma acc is a salivary gland malignancy that is an extremely rare neoplasm gland in 15%30% of all cases, in the minor salivary gland in 30% of cases, and in the parotid gland in 2%15% of cases. 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 is a rare type of cancer that can exist in many different body sites. Understanding acc adenoid cystic carcinoma research. Systemic therapy for recurrent or metastatic salivary gland. The palate is the most common site of occurrence in 50% cases of adenoid cystic carcinoma involving minor salivary glands.
Adenoid cystic carcinoma of submandibular gland metastatic to. Intracranial acc is even more infrequent and could be primary or secondary occurring either by direct invasion, hematogenous spread, or perineural spread. Hybrid salivary gland tumor of the upper lip or just an. Clinicopathologic predictors and impact of distant. First described in 1853, named adenoid cystic in 1953. Pathology adenoid cystic carcinomas are generally considered low grade 4. 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. This protocol is not required for accreditation purposes for the following. Acc occurs most commonly in the salivary glands and, less commonly, at other sites such as the breast, skin, uterine cervix, upper aerodigestive tract, and lung. The salivary glands are classified as major or minor. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues.
Adcc comprise approximately 10% of all epithelial salivary neoplasms and most frequently involve the parotid, submandibular and minor salivary glands. Adenoid cystic carcinomas are a rare histological subtype of adenocarcinoma. There are approximately 1,200 new cases of acc diagnosed each. Adenoid cystic carcinoma acc is characterized by slow growth, frequent local recurrences, and high incidence of distant metastasis dm. Patients with adenoid cystic carcinomas of the salivary glands. Hyalinizing clear cell carcinoma is a lowgrade tumor with infrequent nodal and. In minor gland sites, plga is twice as frequent as adenoid cystic carcinoma, and among all benign and malignant salivary gland neoplasms, only pleomorphic adenoma and mucoepidermoid carcinoma are more common. We present a retrospective multicentric study of 67 patients with adenoid cystic carcinomas of the intraoral minor salivary glands, who were diagnosed and treated between 1986 and 2004 in the. Groppe, md a patient developed widely metastatic adenoid cystic carcinoma of the parotid gland after several local. The minor salivary glands consist of 800 small mucussecreting glands located throughout the lining of the oral cavity. 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. Salivary gland malignancies can occur throughout the upper aerodigestive tract but the majority of tumors occur in the parotid gland and other major salivary glands.
Early cutaneous metastasis of adenoid cystic carcinoma of. Shallow whole genome sequencing of adenoid cystic carcinomas. Epidemiology and treatment of adenoid cystic carcinoma of the. 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. Salivary gland tumors may cause painless masses in the mouth or face. Adenoid cystic carcinoma acc of the lacrimal glands is an extraconal malignancy usually originating from the orbital lobe of the lacrimal gland. Adenoid cystic carcinoma acc of salivary glands is a slowgrowing malignant tumor, characterized by wide local infiltration, perineural spread, a propensity to. But it also can occur in other parts of your mouth and throat or other areas of your body, such as your sweat glands or tear glands. Adenoid cystic carcinoma of the salivary glands radiology. A quitesmall adenoid cystic carcinoma in a patient with. Wholegenome sequencing of salivary gland adenoid cystic. Pdf adenoid cystic carcinoma is a malignant tumor with a deceptively. 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. This neoplasm is defined by its distinctive histologic appearance.
This type of neoplasm was first elaborated by billroth in 1856 as a benign neoplasm and was called cylindroma for cribriform. Adenoid cystic carcinoma acc is a rare salivary gland malignant neoplasm. Can arise anywhere within the upper respiratory tract. Adenoid cystic carcinoma causes, symptoms, treatment. Review and updates of immunohistochemistry in selected. 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. Slowgrowing epithelial tumor of salivary glands, thought to arise from intercalated mucoussecreting ducts.
The concept of cure may be difficult to assess until the patient is disease free for 10 years or longer. The cellular composition of adenoid cystic carcinoma is similar to that in the terminal. 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. Its innoceous clinical presentation remains a diagnostic challenge. To better understand the genetic alterations underlying the pathogenesis of these tumors, we performed comprehensive genome analyses of 25 freshfrozen tumors, including wholegenome sequencing and expression and pathway analyses. Adenoid cystic carcinomas accs are the most common malignant tumors that occur in minor salivary glands.
Adenoid cystic carcinoma of parotid salivary glanda case. Adenoid cystic carcinoma genetic and rare diseases. Adenoid cystic carcinoma adcc is a malignant tumor occurring in many types of tissue, including salivary gland. Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. Adenoid cystic carcinoma definition of adenoid cystic. 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.
An external file that holds a picture, illustration, etc. Phase ii study of lenvatinib in patients with progressive. The typical clinical and pathological findings of this tumor include slow indolent growth, common local recurrence, and late distant metastasis to lung, brain, bone, liver, thyroid, and spleen. Risk factors and prognosis for salivary gland adenoid. Case report a 65 yearold male patient consulted his physician be. Adenoid cystic carcinoma nord national organization for. Adenoid cystic carcinoma of the head and neck literature. Pdf adenoid cystic carcinoma of parotid salivary glanda. A case report294 acc cases occur in the salivary glands, among which acc of the minor salivary gland has the worst prognosis.
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