Lung
Salivary gland type tumors
Adenoid cystic carcinoma


Topic Completed: 1 February 2017

Minor changes: 28 September 2020

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PubMed Search: adenoid cystic carcinoma lung

Roseann Wu, M.D., M.P.H.
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Cite this page: Wu R. Adenoid cystic carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lungtumorACC.html. Accessed October 20th, 2020.
Definition / general
  • #2 most common salivary gland-type carcinoma of lung
  • Usually in large bronchi, may involve the trachea
  • Prolonged course, but overall prognosis is poor
Essential features
  • See also Salivary glands - Adenoid cystic carcinoma
  • Primary pulmonary adenoid cystic carcinoma is rare, and metastasis from salivary glands must be excluded
  • Morphology is similar to adenoid cystic carcinomas in other sites, with cribriform, tubular and solid patterns
  • These tumors tend to arise in association with central airways and spread along neurovascular bundles
Terminology
  • Formally called bronchial adenoma, but now considered malignant
ICD coding
  • Use code specific for location of tumor
  • C34.90 Malignant neoplasm of unspecified part of unspecified bronchus or lung
Epidemiology
  • Primary pulmonary adenoid cystic carcinoma is very rare, < 0.2% of lung cancers
  • Typically adults
Sites
  • Usually central / endobronchial but may be peripheral
Pathophysiology
  • Slow growing but persistent, with recurrences over years, potentially with metastasis to lymph nodes and distant sites
Etiology
  • Unclear, probably arise from submucosal bronchial glands
Clinical features
  • Obstructive symptoms, i.e., pneumonia, dyspnea, cough, wheeze, hemoptysis
  • Peripheral lesions asymptomatic
Diagnosis
  • Exclude metastasis from salivary glands
Radiology images

Images hosted on other servers:
Adenoid cystic carcinoma<br>obstructing right upper<br>lobar bronchus

Adenoid cystic carcinoma
obstructing right upper
lobar bronchus

Prognostic factors
  • Variable by tumor stage
Case reports
Treatment
Clinical images

Images hosted on other servers:
Various images

Various images

Gross description
  • Large, centrally located, polypoid, intrabronchial mass
  • May grow along bronchi (subepithelial) causing thickened bronchial wall
  • Circumscribed, soft, yellowish white
Gross images

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75 year old woman with 1 cm lung lesion

75 year old woman with 1 cm lung lesion

Microscopic (histologic) description
  • Propensity for tracking along nerves and cartilaginous plates → bronchial margins more often positive than in other lung cancers
  • Infiltrative growth, cribriform / cylindromatous (islands and nests, with luminal matrix), tubular (gland-like spaces) or solid (insular, with scant matrix) - usually a mix of patterns are seen
  • Defining features are pseudocysts (rounded extracellular space containing basal lamina), intercellular spaces, basal lamina and true glandular lumens (Hum Pathol 1982;13:916)
  • Monotonous, polygonal, basaloid cells
  • Absence of mitoses, nuclear pleomorphism, necrosis and hemorrhage in most cases; solid type may show more mitoses
Microscopic (histologic) images

Contributed by Yale Rosen, M.D.
Adenoid cystic carcinoma Adenoid cystic carcinoma Adenoid cystic carcinoma Adenoid cystic carcinoma

Adenoid cystic carcinoma



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Cribriform-like structures with mucus

Cribriform-like structures with mucus

Focal tubular structure

Focal tubular structure

Various images

Various images

14 year old girl with 11 cm lung mass

14 year old girl with 11 cm lung mass


75 year old woman with 1 cm lung lesion (H&E, TTF1) 75 year old woman with 1 cm lung lesion (H&E, TTF1) 75 year old woman with 1 cm lung lesion (H&E, TTF1)

75 year old woman with 1 cm lung lesion (H&E, TTF1)

Cytology description
  • Cylinders or spheres of myxochondroid matrix within epithelial groups
  • Diagnosis more difficult if matrix is scarce, as in solid type
  • Cellular uniformity, distinct nucleolus, granular cytoplasm, distinct cell border, organoid cluster, hyaline globule and hyaline basement membrane material (J Pathol Transl Med 2015;49:511)
Cytology images

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Cytomorphology of pulmonary ACC

Cytomorphology of pulmonary adenoid cystic carcinoma

Immunohistochemistry
  • Usually not necessary for diagnosis
  • Luminal cells: c-kit/CD117+, p63-, actin-
  • Basal (myoepithelial) cells: c-kit/CD117-, p63+, actin+
  • Tumor cells are usually positive for keratin and S100, and negative for neuroendocrine markers
  • Basement membrane material is positive for collagen type IV or laminin
Electron microscopy description
  • May show evidence of partial myoepithelial differentiation
Molecular / cytogenetics description
Differential diagnosis
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