Larynx and hypopharynx
Salivary carcinoma
Adenoid cystic carcinoma

Senior Author: Tony Ng, M.D., Ph.D.
Editorial Board Member: Kelly Magliocca, D.D.S., M.P.H.
Editor-in-Chief: Debra Zynger, M.D.
Nathan Lee, D.M.D.
Tony Ng, M.D., Ph.D.

Topic Completed: 18 June 2019

Revised: 3 July 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Adenoid cystic carcinoma [title] larynx

Nathan Lee, D.M.D.
Tony Ng, M.D., Ph.D.
Page views in 2018: 359
Page views in 2019 to date: 309
Cite this page: Lee N, Ng T. Adenoid cystic carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/larynxacc.html. Accessed July 23rd, 2019.
Definition / general
Essential features
  • Slow growing salivary gland tumor that can also be found in the larynx
  • Presents at late stage; difficult to achieve clear surgical margins
  • Various histologic patterns including tubular, cribriform, solid (similar to the oral cavity)
ICD coding
  • ICD-O: 8200/3 - adenoid cystic carcinoma
Epidemiology
Sites
Pathophysiology
  • Arises from subepithelial glands (Otolaryngol Head Neck Surg 2016;154:433)
  • Associated with t(6;9)(q22-23;p23-24) translocation creating a fusion gene between MYB proto-oncogene and NFIB transcription factor gene (Mod Pathol 2011;24:1169)
  • Overall, 56/68 (82%) of adenoid cystic carcinomas expressed MYB-NFIB fusion in a study by Brill et al.; of those 2/5 (40%) were from the larynx (Mod Pathol 2011;24:1169)
  • This chromosomal rearrangement juxtaposes super enhancers to the MYB locus to create a positive feedback loop elicited when the MYB protein activates these enhancers (Oncotarget 2016;7:66239)
  • Fusion between MYBL1 and NFIB genes without MYB aberration have been identified, demonstrating that tumor development may also be driven by genetic alterations in different members of the same transcription factor family (Oncotarget 2016;7:66239)
Etiology
Clinical features
Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:

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Bulging vocal cord mass

Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Vancouver General Hospital slide collection
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Characteristic histologic features

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Involvement of the trachea

Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Larynx, resection:
    • Adenoid cystic carcinoma, differentiated tubular and cribriform growth patterns (see synoptic report)
    • Maximum tumor size: at least 9 cm
    • Tumor extensively involves lateral paratracheal and paralaryngeal margins
    • Vocal cords and pyriform sinus negative for malignancy
    • Perineural invasion present
Differential diagnosis
Board review question #1

    Which of the following is true about this adenoid cystic carcinoma of the larynx?

  1. Immunohistochemical staining for CD117 will be negative in tumor cells
  2. Perineural invasion is extremely rare in this type of tumor
  3. The cystic spaces contain mucoid or hyalinized material
  4. The primary site of laryngeal adenoid cystic carcinoma is the vocal cords themselves
  5. This tumor is clinically aggressive, reflected by the high mitotic rate and basaloid cells
Board review answer #1
C. The cystic spaces contain mucoid or hyalinized material. These spaces often contain a mildly basophilic mucoid material, a hyalinized eosinophilic product or a combined mucoid hyalinized appearance.

Reference: Adenoid cystic carcinoma

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Board review question #2
    What is the most common malignant minor salivary gland tumor of the larynx?

  1. Acinic cell carcinoma
  2. Adenoid cystic carcinoma
  3. Mucoepidermoid carcinoma
  4. Pleomorphic adenoma
  5. Polymorphous adenocarcinoma
Board review answer #2
B. Adenoid cystic carcinoma. While minor salivary gland tumors of the larynx are rare overall, adenoid cystic carcinoma is the most common malignant entity in this location.

Reference: Adenoid cystic carcinoma

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