Quick Facts and Statistics for ACC

Quick Facts About ACC

  • ACC accounts for less than 1 out of every 500 new cases of cancer diagnosed each year.
  • The Rare Disease Act of 2002 (HR 4013) defines a rare disease as one that affects less than 200,000 people in the US.
  • Each year, there are approximately 566,000 people diagnosed with cancer in the US, and only about 1224 of them are diagnosed with ACC.
  • Given the current population, approximately 918 people die from ACC each year.
  • There are approximately 14,873 Americans alive today living with this disease.
  • ACC’s progression is typically gradual and relentless. It has a high propensity to recur in the primary area, metastasize to new organs, and spread along neurological pathways.
  • This tendency for slow yet relentless growth has led to survival rates that report approximately 89% of ACC patients will survive at least 5 years, with that being reduced to 40% surviving 15 years. (This statistic is from the 1999 MD Anderson report on ACC Morbidity and Mortality.)
  • ACC primary tumors can occur in 38 different sites of the body, with approximately 58% occurring in the oral cavity and pharynx, followed by 17% in the respiratory system, 12% in the breast and the remaining 13% in other areas of the body.
  • The major salivary glands, including the parotid, sub-mandibular and sub-lingual glands account for 33.7% of all cases.
  • Women comprise almost two-thirds of all ACC cases (62% female vs 38% male).
    • Source: SEER 17 Registries 1985 – 2005, Total count of 3,409 new cases of ACC; 2,123 female and 1,286 male.
    • A contributing factor to the gender difference is the number of cases which are gender specific primary sites, with female breast and genital systems at 14.5% vs male breast and genital system at 0.4% of all cases.
    • When the breast and genital systems are excluded in the count, gender differences between the cases are less significant; with 56% female vs 44% male
  • The median age at diagnosis for ACC patients is 58 years old, which is 10 years younger than that of the average cancer patient. This means that ACC often afflicts more younger and middle-aged patients than other types of cancer. The term "median" does not mean the same thing as the word "average" Median means that there are as many people diagnosed who are less than 58 years old as there are who are over 58 years old.
  • According to the Australian Cancer Database 2010 AIHW - the sites ACC was reported:
 SITE  2006  2007  2008  2009  2010  AVERAGE
 Major salivary glands  33  33  27  28  28  30
  Lip, tongue, gum, floor of mouth, palate, other mouth   20  16 18   13  16  15
 Pharynx, larynx, trachea, bronchus, lung  12  12    4  10  13  10
 Nasal cavity, middle ear, accessory sinuses    8  11  10    9    9    9
 Breast    9    7    9    9    8    8
 Skin    3    4    6    4    4    4
 Other Sites    6    3    4     9    5    5

Statistics for Adenoid Cystic Carcinoma Based on Data from SEER

What is SEER?

The Surveillance, Epidemiology, and End Results (SEER) Program of the NCI is a collection of central cancer registries in the United States that collect and submit cancer incidence, prevalence, mortality, survival, stage at diagnosis data and other statistics to the National Cancer Institute. The National Cancer Act of 1971 mandated the collection, analysis, and dissemination of data useful in the prevention, diagnosis, and treatment of cancer leading to the establishment of the SEER Program. The three primary statistics reported for types of cancer include incidence, mortality and prevalence.


Incidence: In 2008 approximately 1,224 people were diagnosed with Adenoid Cystic Carcinoma in the United States, with 770 being women and 454 being men.

Statistical Basis: This is based on an incidence rate of 0.4 per 100,000 (Source: SEER) and a population of 305.8 million (Source: U.S. Census Bureau).

Definition of Incidence: The number of newly diagnosed cases of cancer during a specific time period.


Mortality: In 2008 approximately 918 people died of adenoid cystic carcinoma in the United States.

Statistical Basis: This is based on a mortality rate of 0.3 per 100,000 (Source: SEER) and a population of 305.8 million (Source: U.S. Census Bureau).

Definition of Mortality: The number of deaths from a cancer during a specific time period.


Prevalence: As of January 1, 2006 approximately 14,873 people were alive in the United States who had been diagnosed with Adenoid Cystic Carcinoma. Statistical Basis: This is based upon the prevalence rate of 1.49 per 100,000 (Source: SEER)

Definition of Prevalence: The number of people alive on a certain date who have been diagnosed with cancer. This number includes newly diagnosed cases, and those with active or stable disease who were diagnosed at some point in their lives. NOTE: The SEER prevalence rate does not include those that are "cured" or no longer have any evidence of ACC in their bodies, since the registry reports no survival rates for ACC.

Explanation of NCI’s SEER Cancer Statistical Analysis

The figures for this report were gathered from four registry groups in 17 geographical regions, which represent a sampling of approximately 26% of the total US population. There were 3,409 histology confirmed cases of ACC reported to these regions in the years 1985 through 2005. There is some variance in the SEER database as to which regions were included in each registry group within a specific range of time, which is noted below.


This report provides analyses for approximately 3,409 histo­logy confirmed cases of adenoid cystic carcinoma obtained from the Surveillance, Epidemiology, and End Results (SEER) Program of the NCI. The most frequently occurring cancers of Adenoid cystic carcinoma origin were in the head and neck group presenting in the oral cavity and pharynx region (58%), followed by the respiratory system including nose, nasal cavity, middle ear, larynx, lung and bronchus (17.42%) The greatest number of cases reported for a single site was in the major salivary glands (33.7%).


Note: SEER Registry classification of salivary gland is specific to the major salivary glands; the parotid, submandibular or submaxillary and sublingual glands. Minor salivary glands are located in many oral cavity structures, including the oral mucosa, palate, uvula, posterior tongue, retromolar trigone, paranasal sinuses, pharynx, larynx, peritonsillar area, and floor of mouth. All minor salivary glands are referred to by their anatomical site.

Materials and Methods

The NCI SEER Program contracts individually with central cancer registries based in organizations such as universities and state health departments, to obtain data on all cancers diagnosed in residents of the registry’s catchment area. SEER collects data on all invasive and in situ cancers except basal cell and squamous cell carcinomas of the skin and in situ carcinomas of the uterine cervix.

SEER cancer registries are selected on the basis of two criteria: the registry’s ability to operate and maintain a population-based cancer reporting system and the epide­miologic significance of their population subgroups. While some cancer registries have remained in the SEER Pro­gram since it began, others have left; additional registries have joined at a later date or left for a period of time and rejoined the Program later. This analysis is based on data from 17 geographic areas, (SEER 17 Registries) which represent approximately 26% of the total US population. SEER 9 Registries were used to calculated incidence-based mortality, as it was the only database available for mortality rates of adenoid cystic carcinoma. The ICD-0-3 code 8200/3, malignant adenoid cystic carcinoma, was used for the years 1985-2005.

SEER 9 Registries The SEER 9 registries are Atlanta, Connecticut, Detroit, Hawaii, Iowa, New Mexico, San Francisco-Oakland, Seattle-Puget Sound, and Utah. Data are available for cases diagnosed from 1973 and later for these registries with the exception of Seattle-Puget Sound and Atlanta. The Seattle-Puget Sound and Atlanta registries joined the SEER program in 1974 and 1975, respectively.

SEER 11 Registries The SEER 11 registries consist of the SEER 9, as described above, plus Los Angeles and San Jose-Monterey. Los Angeles and San Jose-Monterey joined the SEER program in 1992. Therefore, data for the SEER 11 are available from 1992 on.

SEER 13 Registries The SEER 13 registries consist of the SEER 11, as described above, plus Rural Georgia and the Alaska Native Tumor Registry. Data are available from all cases diagnosed from 1992 and later for these registries. These registries report on expanded race.

SEER 17 Registries The SEER 17 registries consist of the SEER 13, as described above, plus Greater California, Kentucky, Louisiana, and New Jersey. Data are available from all cases diagnosed from 2000 and later for these registries. Louisiana cases diagnosed from July - December 2005 are excluded from statistical analysis.