Adenoid Cystic Carcinoma

oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

Adenoid cystic carcinoma (ACC) is an uncommon and unusually indolent cancer arising within glands and occurring mainly in the head and neck but also in the breast, trachea, lacrimal glands, skin and vulva.

ACC arising in the head and neck often has a relentless progress and poor prognosis but tumours arising in some sites (notably in the breast) may have a better prognosis. ACCs are often slow-growing but locally aggressive and particularly prone to recurrence.

  • Adenoid cystic carcinoma (ACC) accounts for about 1% of all head and neck malignancies.[1]
  • The age range is unusually wide and it can occur in children. It is most common between the ages of 40 and 70 years. It occurs more often in females in a 3:2 ratio.
  • The p53 tumour suppressor gene may be inactivated in advanced disease but otherwise no particular or consistent genetic or environmental factors have been identified.[2]
  • However, a recent study identifies loss of 1p32-p36 as the most frequent genetic change which was also a marker for poor prognosis in ACC of salivary glands.[3]

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It has a distinct histopathological appearance. There are three main growth patterns at histology:

  • Cribiform.
  • Tubular.
  • Solid.This follows a more aggressive course usually.

It may, however, be confused with basaloid squamous carcinoma.

Presentation depends on site, but it can present as:

  • A painless slow-growing mass in the face or mouth. In one study using endoscopic resection of tumours in the paranasal sinuses and anterior skull base, adenoid cystic carcinoma (ACC) represented 15% of the tumours found.[4]
  • More advanced tumours may invade nerves, causing paralysis and pain. ACC is of interest to neurosurgeons and neurologists because of a tendency to infiltrate neural structures and spread perineurally. Intracranial involvement can occur but is rare.[5][6]
  • Tumours of the lacrimal gland may affect vision and also may cause proptosis. ACC is the most common primary malignant epithelial neoplasm affecting lacrimal glands.[7]
  • In the lung, ACC may present with respiratory symptoms. ACC is the second most common primary tumour of the trachea and carries a poor prognosis (38.5% 5-year survival). Relative to squamous carcinomas it is found in younger patients and is relatively resistant to treatment.[8]
  • In the larynx it may cause voice changes and presents with hoarseness and difficulty breathing.[9]
  • ACC may present as a breast tumour and can present in men.[10] It is a rare breast carcinoma, representing less than 1% of breast carcinomas. Generally it has a favourable prognosis.[11][12]
  • ACC is unusual in not routinely metastasising to regional lymph nodes.
  • ACC may present late or after treatment failure, with distant metastases (most commonly lung, followed by liver).[13] Bone secondaries may also occur.
  • Salivary gland tumours:
    • Benign mixed salivary gland tumour.
    • Mucoepidermoid carcinoma.
    • Polymorphous low-grade adenocarcinoma (PLGA) shares features with adenoid cystic carcinoma (ACC).
  • Squamous cell carcinoma.
  • Tumour (new or recurrent) may be identified by CT scan or at MRI.
  • There are no serological markers, although a recent report using CD43 (a sialoglycoprotein) may be useful as an adjunct to histological examination for distinguishing adenoid cystic carcinoma (ACC) from PLGA and monomorphic adenoma.[14]
  • Diagnosis is by biopsy or resection followed by histological assessment.
  • Investigation of immunoreactivity to identify markers has not so far proved very helpful.[2][15]

Staging to plan treatment and assess prognosis should identify:

  • Site. Some sites have a worse prognosis, often a function of the difficulty of complete surgical resection.
  • Clearance of surgical margins.
  • Histological subtype.
  • Tumour, node and metastasis (TNM) stage.
  • Aggressive surgical resection is the mainstay of treatment.[7]
  • Postoperative radiotherapy is used to try to reduce local recurrence.[16] Some consider this should be standard therapy for adenoid cystic carcinoma (ACC) of the head and neck.[17] However, there is also a suggestion from one study that it is unnecessary where surgical margins are negative.[18]
  • Neutron beam therapy may be more effective. It should be considered as initial primary treatment for locally advanced, unresectable ACC of minor salivary glands and in patients where surgery carries unacceptable morbidity.[19]
  • Chemotherapy has not produced good results. Clinical trials of combination chemotherapy are underway for metastatic and locally recurrent disease.[20]

A wide range of complications can occur from local spread, metastasis and treatment. These are determined by site and extent of local invasion.

  • Adenoid cystic carcinoma (ACC) is a slow-growing, indolent tumour but runs a relentless course. This gives good survival figures at 5 years but poor figures at 15 years. The 5-year survival is 89%, 10-year survival is 65% and the 15-year survival is 40%.[21]
  • Poor prognostic features include neural spread and positive surgical margins.[22] Clinical stage, primary site and histological subtype also obviously affect prognosis.[23][24][25]
  • Although salivary gland lesions have a relatively high tendency to metastasise, ACC of the breast tends to have a much better prognosis.[26]
  • Recurrence rates are mainly influenced by tumour site, but also by clinical factors (age, sex, site and stage), postoperative treatment (radiotherapy or not) and pathological variables (grade, margins of resection and perineural invasion).[27]
  • There is a recent suggestion that Ki-67 markers and other markers may correlate with prognosis.[3][16]

Further reading & references

  1. Dodd RL, Slevin NJ; Salivary gland adenoid cystic carcinoma: a review of chemotherapy and molecular Oral Oncol. 2006 Sep;42(8):759-69. Epub 2006 Jun 6.
  2. Edwards PC, Bhuiya T, Kelsch RD; Assessment of p63 expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and basal cell and canalicular adenomas.; Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 May;97(5):613-9.
  3. Rao PH, Roberts D, Zhao YJ, et al; Deletion of 1p32-p36 Is the Most Frequent Genetic Change and Poor Prognostic Marker in Adenoid Cystic Carcinoma of the Salivary Glands. Clin Cancer Res. 2008 Aug 15;14(16):5181-7.
  4. Buchmann L, Larsen C, Pollack A, et al; Endoscopic Techniques in Resection of Anterior Skull Base/Paranasal Sinus Malignancies.; Laryngoscope. 2006 Sep 25;.
  5. Alleyne CH, Bakay RA, Costigan D, et al; Intracranial adenoid cystic carcinoma: case report and review of the literature.; Surg Neurol. 1996 Mar;45(3):265-71.
  6. Lee AG, Phillips PH, Newman NJ, et al; Neuro-ophthalmologic manifestations of adenoid cystic carcinoma.; J Neuroophthalmol. 1997 Sep;17(3):183-8.
  7. Terasaki M, Tokutomi T, Maruiwa H, et al; High-grade adenoid cystic carcinoma originating from the lacrimal gland.; Brain Tumor Pathol. 2000;17(3):159-63.
  8. Clough A, Clarke P; Adenoid cystic carcinoma of the trachea: a long-term problem.; ANZ J Surg. 2006 Aug;76(8):751-3.
  9. Khan AR, Jan A, Nawaz G, et al; Adenoid cystic carcinoma of larynx.; J Coll Physicians Surg Pak. 2006 Oct;16(10):669-70.
  10. Kshirsagar AY, Wader JV, Langade YB, et al; Adenoid cystic carcinoma of the male breast.; Int Surg. 2006 Jul-Aug;91(4):234-6.
  11. Shin SJ, Rosen PP; Solid variant of mammary adenoid cystic carcinoma with basaloid features: a study of nine cases.; Am J Surg Pathol. 2002 Apr;26(4):413-20.
  12. Arpino G, Clark GM, Mohsin S, et al; Adenoid cystic carcinoma of the breast: molecular markers, treatment, and clinical outcome.; Cancer. 2002 Apr 15;94(8):2119-27.
  13. Zeidan BA, Abu Hilal M, Al-Gholmy M, et al; Adenoid cystic carcinoma of the lacrimal gland metastasising to the liver: Report of a case.; World J Surg Oncol. 2006 Sep 20;4(1):66.
  14. Woo VL, Bhuiya T, Kelsch R; Assessment of CD43 expression in adenoid cystic carcinomas, polymorphous low-grade adenocarcinomas, and monomorphic adenomas.; Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Oct;102(4):495-500.
  15. Ferrazzo KL, Alves SM Jr, Santos E, et al; Galectin-3 immunoprofile in adenoid cystic carcinoma and polymorphous low-grade adenocarcinoma of salivary glands.; Oral Oncol. 2006 Sep 21;.
  16. Triantafillidou K, Dimitrakopoulos J, Iordanidis F, et al; Management of adenoid cystic carcinoma of minor salivary glands.; J Oral Maxillofac Surg. 2006 Jul;64(7):1114-20.
  17. Chen AM, Bucci MK, Weinberg V, et al; Adenoid cystic carcinoma of the head and neck treated by surgery with or without postoperative radiation therapy: prognostic features of recurrence.; Int J Radiat Oncol Biol Phys. 2006 Sep 1;66(1):152-9.
  18. Silverman DA, Carlson TP, Khuntia D, et al; Role for postoperative radiation therapy in adenoid cystic carcinoma of the head and neck.; Laryngoscope. 2004 Jul;114(7):1194-9.
  19. Douglas JG, Laramore GE, Austin-Seymour M, et al; Neutron radiotherapy for adenoid cystic carcinoma of minor salivary glands.; Int J Radiat Oncol Biol Phys. 1996 Aug 1;36(1):87-93.
  20. Terashima K, Shioyama Y, Nakamura K, et al; Long-term local control of recurrent adenoid cystic carcinoma in the parotid gland with radiotherapy and intraarterial infusion chemotherapy.; Radiat Med. 2006 May;24(4):287-91.
  21. Garden AS, Weber RS, Ang KK, et al; Postoperative radiation therapy for malignant tumors of minor salivary glands. Outcome and patterns of failure.; Cancer. 1994 May 15;73(10):2563-9.
  22. Garden AS, Weber RS, Morrison WH, et al; The influence of positive margins and nerve invasion in adenoid cystic carcinoma of the head and neck treated with surgery and radiation.; Int J Radiat Oncol Biol Phys. 1995 Jun 15;32(3):619-26.
  23. Santucci M, Bondi R; Histologic-prognostic correlations in adenoid cystic carcinoma of major and minor salivary glands of the oral cavity.; Tumori. 1986 Jun 30;72(3):293-300.
  24. Qing J, Zhang Q, Wei MW, et al; [Prognostic analysis of adenoid cystic carcinoma of major salivary glands of 64 cases.]; Ai Zheng. 2006 Sep;25(9):1138-43.
  25. Huang M, Ma D, Sun K, et al; Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands.; Int J Oral Maxillofac Surg. 1997 Dec;26(6):435-9.
  26. Marchio C, Weigelt B, Reis-Filho JS; Adenoid cystic carcinomas of the breast and salivary glands (or 'The strange case J Clin Pathol. 2010 Mar;63(3):220-8.
  27. Prokopakis EP, Snyderman CH, Hanna EY, et al; Risk factors for local recurrence of adenoid cystic carcinoma: the role of postoperative radiation therapy.; Am J Otolaryngol. 1999 Sep-Oct;20(5):281-6.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Richard Draper
Current Version:
Last Checked:
18/02/2011
Document ID:
1044 (v22)
© EMIS