They are typically treated first with surgery and then radiation therapy. Aug, 2007 background 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 acc is a rare malignant tumor arising from salivary glands of head and neck region. Histopathological grading of adenoid cystic carcinoma of the. I it can also occur in the breast, uterus, or other locations in the body. Salivary gland tumors an update american society for. This protocol is not required for accreditation purposes for the following. Salivary gland neoplasms constitute 3% of cancers of all sites, of which, 1015% are malignant 1, 2.
Mostly ductal cells larger cells with more vesicular chromatin, stain with cd117 and ck. An adenoid cystic carcinoma arising from the salivary gland. We conducted a retrospective study limited to a period of 10 years in a single medical institution to investigate the morphoclinical profile of this tumor. Adenoid cystic carcinoma is the most common malig nant tumor arising from the submandibular, sublingual, and lacrimal glands. 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 head and necka 20 years experience. Metastasis can occur even a decade or more after initial treatment of the primary. Minor salivary gland malignant neoplasms are most often adenoid cyst. An unusual presentation of adenoid cystic carcinoma of the. Due to its rarity, primary lung acc remains incompletely understood. We present here a 12yearold girl initially diagnosed as pleomorphic adenoma on fine needle aspiration biopsy, and adenoid cystic carcinoma acc after the lesion was excised and examined by histopathology. Polymorphous lowgrade adenocarcinoma 50% of salivary tumors on palate are malignant, but if you consider lymphomas and other things that can appear on the palate, its malignant over 50% of the time. More frequent developing in minor salivary gland, this is a slowgrowing tumor with a longlasting natural evolution, quite aggressive locally, but which has a tendency toward local recurrence and even for distant metastasis. Epiregulin promotes lung metastasis of salivary adenoid.
Adenoid cystic carcinoma acc of salivary gland origin is rare. Reduced ecadherin expression correlates with unfavorable. Adenoid cystic carcinoma is the 4 th most common carcinoma of salivary glands incidence is 10% of all salivary gland carcinomas site most common is parotid, can occur in minor salivary glands among which more common is palate. Intrinsically graded tumors can still be updowngraded usually up based on atypia, etc highgrade transformation. Recognize the presentation of pleomorphic adenoma pa and carcinoma ex pa caxpa 2. Others have a variable grade and must be specifically graded. Malignant tumours of the salivary glands in children are extremely rare. This type of neoplasm was first elaborated by billroth in 1856 as a benign neoplasm and was called cylindroma for cribriform. Adenoid cystic carcinoma, parotid gland, malignant salivary gland tumor 1. Pathology reporting should follow a standard format for salivary gland neoplasms.
Pdf akt3 drives adenoid cystic carcinoma development in. Management of adenoid cystic carcinoma of minor salivary glands. It is notorious for its poor disease free survival due to frequent local recurrences and often indolent distant metastases. The major salivary glands consist of the parotid, submandibular, and sublingual glands. Adenoid cystic carcinoma of the submandibular gland. Ckit expression in the salivary gland neoplasms adenoid. Adenoid cystic carcinoma acc is a malignancy affecting the salivary glands and rarely involving the lung. Frontiers perineural invasion in adenoid cystic carcinoma. Full text full text is available as a scanned copy of the original print version. Adenoid cystic carcinoma of the parotid metastasizing to liver.
Adenoid cystic carcinoma of the tongue the journal of. Adenoid cystic carcinoma, parotid, capsular invasion. Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands. Adenoid cystic carcinoma, cribriform pattern, salivary gland malignancy. Data were extracted from the seer 18 database surveillance, epidemiology, and end results of the national cancer institute. 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. Cancers of the major salivary gland jco oncology practice. Acinic cell carcinoma adenoid cystic carcinoma cribriform, tubular or solid growth 2 cell types. Complementary histoenzymological investigations and electron microscopic study were performed on specimens from 7 and patients respectively. Perineural invasion in adenoid cystic carcinoma of the. Stanford is a global referral center for the diagnosis and treatment of adenoid cystic carcinoma. Histological changes during progression of adenoid cystic carcinoma volume 106 issue 11.
Other important prognostic features of the adenoid cystic carcinoma were its primary site, its presence or absence at surgical margins, and the anatomic structures it involved. A fragment of parotid is present in one corner large arrow with tumor double arrows invading the thick fibrous parotid capsule. The minor salivary glands consist of 800 small mucussecreting glands located throughout the lining of the oral cavity. Jun 21, 2017 adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. Management of adenoid cystic carcinoma of minor salivary. Salivary gland adenoid cystic carcinoma my cancer genome. Seven years later, an abdominal computed tomography scan performed in the context.
It is more frequently diagnosed in females, affecting all age groups and often arising from the minor salivary glands 3, 67. Adenoid cystic carcinoma of parotid salivary glanda case. Choroidal metastasis from submandibular salivary gland. It comprises about 1% of all salivary gland tumors. The immunohistochemical expression ki67 in adenoid cystic. This study aims to evaluate the diagnostic accuracy of acca on fine needle aspiration fna material and the associated challenges. For accreditation purposes, this protocol should be used for the following procedures and tumor types. It is uncommonly found outside the major or minor salivary glands and is especially rare when located in the nasal cavity.
It is slow growing but a highly invasive cancer with a high recurrence rate. The histopathology is marked by sheets of large swollen polyhedral epithelial oncocytes, which are. Acc is the second most common malignant salivary neoplasm, accounting for around one quarter of cases. Ckit expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous lowgrade adenocarcinoma, and monomorphic adenoma. Clinical outcomes and prognostic factors of salivary gland. Adenoid cystic carcinomas are rare cancers that develop from the salivary glands and may spread along nerve fibers to the base of the skull or brain. A case of choroidal metastasis from an adenoid cystic carcinoma of submandibular salivary gland is described. Adenoid cystic carcinoma acc is a relatively rare tumor of epithelial cell origin, most commonly arising from major salivary glands. Histologic grading of adenoid cystic carcinoma of the. A hybrid carcinoma of epithelialmyoepithelial carcinoma and. Clinical features, treatment and outcomes in patients with. The salivary glands are classified as major or minor. Adenoid cystic carcinoma of the salivary gland jama.
Jan 01, 2015 adenoid cystic carcinoma acc of the head and neck is one of the most prevalent malignant salivary gland neoplasms. Current thinking in the management of adenoid cystic carcinoma of. Jan 08, 2017 gland adenoid cystic carcinoma is the most common malignant histology of the submandibular and minor salivary glands squamous cell carcinomas in the parotid region usually represent lymph node metastases from cutaneous squamous cell carcinomas rather than primary gland carcinomas. Surgically resected salivary gland tumors diagnosed as adcc n 219 in 15 reference hospitals in japan were subjected to a retrospective pathological reevaluation. Adenoid cystic carcinoma is a rare tumor, accounting for about 7. Mostly myoepithelial small cells with oval to angulated nuclei, stain with p40 and sma highgrade. A case report294 acc cases occur in the salivary glands, among which acc of the minor salivary gland has the worst prognosis. Salivary gland tumors university of california, davis.
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. Although many contributing factors have been reported, the. It most commonly arises in the major and minor salivary glands of the head and neck. Management of salivary gland adenoid cystic carcinoma. Carcinoma adenoid cystic carcinoma adenocarcinoma, nos intraductal carcinoma carcinoma ex pleomorphic adenoma some tumors have intrinsic grade. A rare case of adenoid cystic carcinoma of ceruminous. Procedure biopsy primary resection specimen with no residual cancer eg, following neoadjuvant therapy cytologic specimens. Adenoid cystic carcinoma occurred more commonly in the minor salivary glands. Adenoid cystic carcinomas are only poorly encapsulated at best. Right medial maxillectomy with resection of the nasal septum was performed, and the pathologic diagnosis was hybrid carcinoma composed of epithelialmyoepithelial carcinoma with an adenoid cystic carcinoma component cribriform pattern fig.
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 acca is an uncommon primary malignancy of salivary glands and rarely nonsalivary tissue. The eurocare data provide a good opportunity to study the survival of this uncommon cancer in a large population. Adenoid cystic carcinoma acc is a rare and slowgrowing epithelial malignant tumor of the salivary glands. Adenoid cystic carcinoma nord national organization for. Conditions better classified as salivary gland neoplasm of uncertain malignant potential sump. Jul 01, 2006 adenoid cystic carcinoma acc is a relatively rare and slowgrowing epithelial tumor of the salivary glands. Protocol for the examination of specimens from patients with carcinomas of the major salivary glands. Adenoid cystic carcinomas of the breast and salivary. A rare case of adenoid cystic carcinoma of ceruminous gland.
Adenoid cystic carcinoma ace is a malignant epithelial salivary gland tumorwith myoepithelial and ductal differentiation. 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. Adenoid cystic carcinoma is a rare malignant parotid tumor. Solitary renal metastasis arising from a pulmonary adenoid. The aims were to evaluate the hisopathological immunopositivity of ki67 in adenoid cystic carcinoma and to compare the results with clinic. Thompson published salivary gland adenoid cystic carcinoma find, read and cite all the research you. The behavior of adenoid cystic carcinoma acc of the salivary glands has been shown to be unpredictable in terms of local and distant spread and mortality. Adenoid cystic carcinoma of parotid salivary glanda case study.
Get a printable copy pdf file of the complete article 2. These are under your tongue and on each side of your jaw below the jawbone. Its innoceous clinical presentation remains a diagnostic challenge. Mucoepidermoid carcinoma is the most frequent primary salivary malignancy, followed by adenoid cystic and acinic cell carcinoma. Risk of nodal metastasis in major salivary gland adenoid cystic. Histogenesis of adenoid cystic carcinoma of the salivary. It commonly occurs in minor salivary glands 30% and nearly half of all oral acc cases occur in the palate, so it comprises 5% to 10% of all salivary gland tumors 1. A denoid cystic carcinoma, a specific variant of adenocarcinoma of the salivary and mucous glands.
Matrix producing carcinomas with basaloid cells falsely interpreted as pleomorphic or basal cell adenoma, ex. Adenoid cystic carcinoma acc is a rare salivary gland malignant neoplasm. The salivary gland oncocytoma is a wellcircumscribed, benign neoplastic growth also called an oxyphilic adenoma. Salivary gland tumors account for only about 5% of all head and neck carcinomas, with acc the fourth most common salivary gland malignancy.
Adenoid cystic carcinoma is a rare kind of cancer that usually starts in the glands that make saliva. They account for up to 30% of minor salivary gland tumors but only for about 6 % of parotid gland tumors. Adenoid cystic carcinoma acc is an uncommon malignancy of secretory glands, which is characterized by slow growth kinetics and perineural invasion, and is among the rarer tumors for which little research has been performed and for which there are few treatment options for patients with advanced disease. Among 117 adenoid cystic carcinomas, 86 were located in minor salivary glands and were selected for a clinicopathological analysis. Adenoid cystic carcinoma of seromucous glands of the head. Among 117 adenoid cystic carcinomas, 86 were located in minor sa. This type of neoplasm was first elaborated by billroth in 1856 as a benign neoplasm and was called cylindroma for cribriform like appearance made by tumor cells with cylindrical pseudo spaces 1.
Histopathological grading of adenoid cystic carcinoma of. Adenoid cystic carcinoma of the parotid metastasizing to. A primary salivary gland or minor salivary gland of upper aerodigestive tract carcinoma characterized by its biphasic ductal and myoepithelial. An external file that holds a picture, illustration, etc.
Pdf adenoid cystic carcinoma acc accounts for approximately 10 % of all salivary gland tumors. More frequent developing in minor salivary gland, this is a slowgrowing tumor with a longlasting natural evolution, quite aggressive locally, but which has a tendency toward local. Adenoid cystic carcinoma, perineural invasion adenoid cystic carcinoma ihc ckit and bcl2 positive epithelial cells. Intracranial acc is even more infrequent and could be primary or secondary occurring either by direct invasion, hematogenous spread, or perineural spread.
Parotid gland is the second most common site to be involved in the head and neck region along with submandibular gland, palate being the most common site involved in the oral cavity. A 50yearold woman with loss of vision in her left eye for 1 week was. Ckit expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous lowgrade adenocarcinoma, and monomorphic adenoma oral surg oral med oral pathol oral radiol endod. The most common histological subtypes were adenoid cystic carcinoma and adenocarcino ma not otherwise. Adenoid cystic carcinoma acc is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. The term adenoid cystic carcinoma carcinoma adenoides cysticum of the salivary glands was first used by ewing 191911 and applied by geschickter in 1945 to tumours of the breast. Alleyne ch, bakay ra, costigan d, thomas b, joseph gj 1996 intracranial adenoid cystic carcinoma. Histological changes during progression of adenoid cystic. Though acc is the most common malignant tumor of the submandibular, sublingual and minor salivary glands, it accounts for only 15% of parotid cancers 3.
A total of 5,400 cgy of postoperative radiation was added as an adjuvant therapy. Sep 03, 2019 salivary glands adenoid cystic carcinoma. 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. It comprises 5% to 10% of all salivary gland tumors, which account for 2% to 4% of all head and neck malignancies. Cap cancer protocol major salivary glands college of american. Independent prognostic predictors include ajcc tnm staging, margin status, high mitotic index defined as.
We describe 15 patients with adenoid cystic carcinoma of the salivary glands treated by radiotherapy from 1960 to 1978. We retrospectively studied 35 operations in 34 patients who had had a pathologic diagnosis of acc of the salivary glands and who had been treated over a 20year period and followed for a. Adenoid cystic carcinomas of the breast and salivary glands. Phase ii study of lapatinib in recurrent or metastatic. Distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during followup. We herein report the case of a 57yearold female patient who was initially diagnosed with primary lung acc and was treated by lobectomy. The prognosis of patients with sacc is strongly associated with the presence of perineural spread pns. Adenoid cystic carcinoma of the head and neck literature. 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. Risk factors and prognosis for salivary gland adenoid cystic. It accounts for 10% to 15% of all salivary neoplasms,2 in contrast to an incidence of only 490 in major salivary gland. Adenoid cystic carcinomas are rare malignant tumors of salivary glands.
Adenoid cystic carcinomas of the salivary glands clinical and morphological features adccs are part of the malignant epithelial tumours of the salivary glands and have been recognised as a speci. Patients with these types of tumours may be asymptomatic. Introduction adenoid cystic carcinoma acc is a rare malignant tumor arising from salivary glands of head and neck region. Adenoid cystic carcinoma acc accounts for about 1% of all head and neck malignancies and 10% of all the salivary gland tumours, and is the. Includes primary salivary gland carcinoma and neuroendocrine carcinoma. Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. Adenoid cystic carcinoma genetic and rare diseases.
They are occurring between the middle and elderly aged patients. Clinical outcomes and prognostic factors of salivary gland adenoid cystic carcinomas. Aug 18, 2020 adenoid cystic carcinoma of the salivary gland sacc is a rare malignant tumors of the head and neck region, but it is one of the most common malignant tumors that are prone to perineural invasion pni of the head and neck. Pdf salivary gland adenoid cystic carcinoma researchgate. The epidemiology of salivary gland tumors sgts is not well documented and. It is characterized by the presence of epithelial and myoepithelial cells forming tubular, cribriform, and solid patterns. Diagnosis and treatment of acc pose numerous challenges, partly due to its biological behavior of slow growth, high tendency of local. Adenoid cystic carcinoma acc is characterized by slow growth, frequent local recurrences, and high. Adenoid cystic carcinoma acc is an infrequent slow growing epithelial tumour constituting for around less than 1% of all the oral and maxillofacial malignancies and almost 10% of all the salivary gland tumors. Jul 30, 2004 adenoid cystic carcinoma acc is a rare malignant neoplasm of the salivary gland. Adenoid cystic carcinoma of seromucous glands of the head and neck.
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