Salivary glands
Primary salivary gland neoplasms
Malignant
Cystadenocarcinoma


Topic Completed: 1 September 2012

Minor changes: 7 April 2020

Copyright: 2003-2020, PathologyOutlines.com, Inc.

PubMed Search: Cystadenocarcinoma salivary glands

Adriana Handra-Luca, M.D.
Nella Cristina Fernandez, M.D.
Page views in 2019: 1,567
Page views in 2020 to date: 1,503
Cite this page: Handra-Luca A, Fernandez N. Cystadenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/salivaryglandscystadenocarcinoma.html. Accessed August 15th, 2020.
Definition / general
Terminology
  • Also called papillary cystadenocarcinoma, mucus producing adenopapillary (nonepidermoid) carcinoma, malignant papillary cystadenoma and low grade papillary adenocarcinoma of palate
  • 2005 WHO classification considered low grade salivary duct carcinoma to be a variant of cystadenocarcinoma, designated as low grade cribriform cystadenocarcinoma (LGCCC)
Epidemiology
  • < 1% of all salivary gland tumors
  • No sex predilection
  • Mean age 59 years
Sites
  • 65% occur in major salivary glands (mainly parotid)
  • Minor salivary gland sites: buccal mucosa, lips, palate are most frequent
Clinical features
  • Mean age 59 years, range 20 - 86 years
  • Male gender predominance
  • Slow growing, compressible, asymptomatic mass
  • Often major salivary glands but also lips, buccal mucosa, palate, tongue, retromolar area, floor of mouth, ectopic salivary glands
  • Tumors of palate may erode bone
  • Usually indolent behavior but occasionally recurs locally or metastasizes to lymph nodes (Ear Nose Throat J 2011;90:E6)
Prognostic factors
Case reports
Treatment
  • Superficial parotidectomy, glandectomy of submandibular and sublingual tumors and wide excision of minor gland tumors
  • Bone resection performed only when it is directly involved by tumor
  • Radiotherapy for high grade tumors
Gross description
  • Multiple cystic spaces, 0.4 to 6 cm, that are variable in size and often filled with mucin
  • At least partially circumscribed
Microscopic (histologic) description
  • Well circumscribed but not encapsulated
  • Invasive, cystic growth pattern, 75% with conspicuous papillary component
  • Irregularly arranged cysts filled with mucin
  • Ruptured cysts with hemorrhage and granulation tissue are common
  • Intervening fibrous stroma
  • Small solid neoplastic islands or duct-like structures may be seen
  • Well or poorly differentiated, composed of small cuboidal cells, large cuboidal cells, tall columnar cells or mixture (Am J Surg Pathol 1996;20:1440)
  • Cell types of lining epithelium include, most often, small and large cuboidal and columnar cells, occasionally mucous, clear and oncocytic cells
  • Typically bland nuclei with conspicuous nucleoli and rare mitoses
  • Does not involve the native duct system
  • Diagnostic criteria: cysts and smaller duct-like structures at least focally infiltrate the salivary parenchyma and surrounding connective tissue
  • Variants include mucinous (aggressive, Auris Nasus Larynx 2005;32:301, Acta Cytol 2001;45:605), oncocytic (children), papillary (low grade, often in minor salivary glands, Int J Surg Pathol 2008;16:341, Auris Nasus Larynx 2007;34:259)
Microscopic (histologic) images

Images hosted on other servers:

Infiltrative growth pattern

Cystic lesion with papillary projections

Cyst with papillary projections

Cystadeno-carcinoma

Mucin spillage

Cyst lining

Various images

Papillary cystadeno-carcinoma

Cytology description
Cytology images

Images hosted on other servers:

Papillary cystadenocarcinoma

Positive stains
Molecular / cytogenetics description
Differential diagnosis
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