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