Salivary glands
Primary salivary gland neoplasms
Malignant
Intraductal carcinoma

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Minor changes: 7 April 2021

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PubMed Search: Intraductal carcinoma salivary glands[TIAB]

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Cite this page: Ely KA. Intraductal carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/salivaryglandsintraductalsalivary.html. Accessed June 23rd, 2021.
Definition / general
  • Rare tumor usually involving the parotid, which is characterized by intracystic / intraductal proliferations of neoplastic epithelial cells
  • Exists in at least 3 variations, each with unique histologic, immunophenotypic and molecular features
  • Relationship to salivary duct carcinoma is unclear
Essential features
  • Resembles ductal carcinoma in situ of the breast, with cribriform, micropapillary, solid, comedo or clinging patterns (Cancer 1996;78:958)
  • At least 3 variants
  • Diagnosis requires thorough sampling to rule out invasion or presence of a myoepithelial layer by immunohistochemistry
  • No purely intraductal carcinomas have definitively recurred or distantly metastasized
Terminology
  • Low grade cribriform cystadenocarcinoma
  • Low grade intraductal carcinoma
  • Low grade salivary duct carcinoma
  • Salivary duct carcinoma in situ
ICD coding
  • ICD-9: 142.9 - malignant neoplasm of salivary gland, unspecified
  • ICD-10: C08.9 - malignant neoplasm of salivary gland, unspecified
Epidemiology
Sites
  • Parotid (84.6%), intraparotid lymph nodes (5.1%), accessory parotid gland (2.6%), submandibular gland (2.6%) and minor salivary glands (5.1%) (Head Neck Pathol 2013;7:S59)
Clinical features
Diagnosis
Prognostic factors
Case reports
Treatment
Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Kim A. Ely, M.D.
Well circumscribed

Well circumscribed

Cystic foci

Cystic foci

Cribriform pattern

Cribriform pattern

Intercalated duct-like subtype

Intercalated duct-like subtype

Cytology description
  • Overlapping sheets and groups of cells with tight intercellular connections (Diagn Cytopathol 2011;39:218)
  • Cribriform / microcystic, solid, pseudopapillary configurations (Korean J Pathol 2013;47:592)
  • Round to oval and slightly irregular nuclei
  • Nucleoli may be evident
  • Cytoplasm can be abundant, well delimited and apocrine
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Parotid, right, complete excision:
    • Intraductal carcinoma (see comment)
    • Comment: The neoplasm is well circumscribed and consists of a complex intraductal proliferation of epithelial cells arranged in cribriform and papillary patterns. A p63 immunostain highlights the myoepithelial layer surrounding these intraductal nests. Comprising cells contain abundant granular eosinophilic cytoplasm and nuclei with prominent nucleoli. Mitoses are inconspicuous and necrosis absent. Additional immunohistochemical stains were performed and are positive for androgen receptor (AR), while negative for S100 and SOX10.
Differential diagnosis
Board review style question #1

Which of the following is true regarding this variant of intraductal carcinoma with the following immunophenotype: S100 and SOX10+; AR-?

  1. It is associated with HRAS and PIK3CA hotspot mutations
  2. It is the most common variant
  3. It is the type most likely to exhibit high grade nuclear features
  4. It is usually GCDFP-15+
Board review style answer #1
B. It is the most common variant

The photomicrograph shows an intraductal proliferation of bland appearing cuboidal cells arranged in cribriform and clinging patterns. Nuclei are small and oval with a dispersed chromatin distribution. Immunohistochemical studies were positive for S100 and SOX10, while negative for AR (androgen receptor) and GCDFP-15. This constellation of findings is characteristic of the intercalated duct-like variant, which is the most common of the variants. It is frequently associated with RET fusions, commonly NCOA4-RET. HRAS and PIK3CA hotspot mutations are usually seen in the apocrine variant, which is the form more likely to exhibit higher grade atypia. It has the following immunophenotype: S100-, SOX10-, AR+ and GCDFP-15+. This staining pattern is similar to that of salivary duct carcinoma, leading to some to believe that apocrine intraductal carcinoma may be a precursor to conventional salivary duct carcinoma.

Comment Here

Reference: Intraductal carcinoma
Board review style question #2
Which of the following is true regarding intraductal carcinoma?

  1. A diagnosis can be made on core biopsy
  2. Has a marked predilection for women
  3. It is most common in the submandibular gland followed by the parotid
  4. No purely intraductal carcinomas have definitively recurred or distantly metastasized
Board review style answer #2
D. No purely intraductal carcinomas have definitively recurred or distantly metastasized.

Intraductal carcinoma involves the parotid (84.6%), intraparotid lymph nodes (5.1%), accessory parotid gland (2.6%), submandibular gland (2.6%) and minor salivary glands (5.1%). Women and men are almost equally affected, with a female to male ratio of 1.5:1. According to the diagnostic criteria outlined by Cheuk et al., a diagnosis of intraductal carcinoma can be made once an invasive component is excluded. This requires thorough sampling or the demonstration of a myoepithelial layer around the epithelial nest by immunohistochemistry. This is more confidently accomplished on an excisional specimen. When confined by a myoepithelial cell layer, no intraductal (or in situ) carcinomas have definitively recurred or distantly metastasized.

Comment Here

Reference: Intraductal carcinoma
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