Salivary glands
Primary salivary gland neoplasms - malignant
Adenoid cystic carcinoma

Topic Completed: 1 September 2012

Revised: 13 May 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Adenoid cystic carcinoma[TI] salivary glands[TI]

See also: Dedifferentiated tumors

Adriana Handra-Luca, M.D.
Page views in 2018: 29,256
Page views in 2019 to date: 18,902
Cite this page: Handra-Luca A. Adenoid cystic carcinoma. website. Accessed July 16th, 2019.
Definition / general
  • Deceptively benign histologic appearance and cribriform, solid or tubular pattern similar to cylindroma of skin (Arch Pathol Lab Med 2011;135:511)
  • Formerly called cylindroma
  • Most common in submandibular, sublingual or minor salivary glands
    • Also seen in nose, sinus, upper airway
  • 22% of salivary gland malignancies
Clinical features
  • Occurs in fifth to sixth decades
  • Male predominance
  • Slow growing, indolent but aggressive
  • Recurrences are frequent
    • Often late and difficult to predict
  • Rarely lymph node metastases
  • 5 year survival is 60%, 10 year is 30%, 15 year is 15%
  • Recurrence rates by pattern: solid (100%), cribriform (89%), tubular (59%)
  • 15 year survival rates by pattern: solid (5%), cribriform (26%), tubular (39%)
  • Better prognosis for tumors of palate or parotid gland; poorer prognosis for dedifferentiated, p53+ tumors
  • Radical surgery regardless of tumor differentiation, radiotherapy
Gross description
  • Small, poorly circumscribed or encapsulated and infiltrative, 1 - 8 cm
Microscopic (histologic) description
  • Cribriform, solid or tubular pattern similar to cylindroma of skin
  • Small bland myoepithelial cells with scant cytoplasm and dark compact angular nuclei surround pseudoglandular spaces with PAS+ excess basement membrane material and mucin
  • Peripheral perineurial invasion and small true glandular lumina
  • No squamous differentiation, no extensive necrosis
  • Note: presence of pseudoglandular lumina, true glandular lumina and perineurial invasion is usually required for diagnosis
  • Dedifferentiated tumors have irregular tumor islands composed of anaplastic cells with abundant cytoplasm and desmoplastic stroma

    Adenoid cystic carcinoma grading systems, prepared by Adriana Handra-Luca, M.D.
     Perzin 1978 and Szanto 1984  Spiro 1992  Van Weert 2015
    Grade 1: Predominantly tubular and cribriform tumor component / pattern, no solid component Grade 1: Mostly tubular or cribriform components, occasional solid component Solid component absent
    Grade 2: Predominantly cribriform component or mixed, < 30% solid component Grade 2: Mixture of components with a solid component of > 50% Solid component present
    Grade 3: Marked predominance of the solid component (> 30%) Grade 3: Mostly / only solid component

  • References:
Cytology images

Images hosted on other servers:
Missing Image Missing Image

Small cells, with bland nuclear features

Positive stains
Negative stains
Electron microscopy description
  • Pseudoglandular spaces, intercellular spaces, abundant basal lamina, true glandular lumina
  • Cells are intercalated ducts, myoepithelial, secretory and reserve cells
Molecular / cytogenetics description
Differential diagnosis
Back to top