Salivary glands
Epithelial / myoepithelial tumors
Mammary analogue secretory carcinoma

Author: Julie Guilmette, M.D. (see Authors page)
Editors: Jeffrey Krane, M.D., Ph.D. and Olguta Gologan, M.D.

Revised: 29 March 2016, last major update September 2015

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Mammary analogue secretory carcinoma [title] salivary glands
Cite this page: Mammary analogue secretory carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/salivaryglandsmammary.html. Accessed January 21st, 2017.
Definition / General
  • Recently described, rare salivary gland tumor that relates the morphology and genetics of an equally rare malignancy of the breast, secretory carcinoma (SC)
  • Previously classified as acinic cell carcinoma ("zymogen poor", intercalated cell predominant variant), mucoepidermoid carcinoma, and adenocarcinoma NOS
  • Harbors specific cytogenetic characteristic: t(12; 15)(q13;q25): ETV6-NTRK3 translocation, demonstrated by either FISH or PCR (Am J Surg Pathol 2010;34:599)
  • Epidemiology
  • Typically it is a disease of young male patients, but occurs in a wide age range (21 - 75), with a mean of 46 years (Laryngoscope 2014;124:188)
  • Pediatric cases have been reported (Otolaryngol Head Neck Surg 2012;146:514, Med Mol Morphol 2014;47:57)
  • Male predominance
  • Sites
  • Parotid gland (up to 70%); lips, hard palate, submandibular glands
  • More frequent in non-parotid sites compared to acinic cell carcinoma (AciCC) (Am J Surg Pathol 2013;37:1053)
  • Clinical Features
  • Overall slowly growing, painless tumor
  • Occasional extracapsular extension and perineural invasion (Am J Surg Pathol 2012;36:27)
  • Infrequent local recurrences
  • Rare metastatic dissemination to cervical lymph nodes, pleura, pericardium and lungs (Am J Surg Pathol 2010;34:599)
  • Prognostic Factors
  • Broad range of clinical behaviors, from indolent to aggressive
  • Currently there is no way to predict which tumors will behave aggressively
  • Higher incidence of regional lymph node involvement than acinic cell carcinomas (Histopathology 2012;61:387)
  • Case Reports
  • 13 year old boy with a rare malignancy of the parotid gland (Med Mol Morphol 2014;47:57)
  • 14 year old girl with mammary analogue secretory carcinoma of the parotid gland (Otolaryngol Head Neck Surg 2012;146:514)
  • 35 year old man with mammary analogue secretory carcinoma (Head Neck Pathol 2012;6:135)
  • 39 year old woman with cystic 4.7 cm submandibular gland mass (Case of the Week #382)
  • 58 year old woman with mammary analogue secretory carcinoma (Laryngoscope 2014;124:188)
  • Labial mammary analog secretory carcinoma (Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:630)
  • Treatment
  • Local excision
  • Radiation therapy in select cases
  • Molecular targeted gene therapy currently investigated (Biochem Biophys Res Commun 2012;429:87)
  • Gross Description
  • Most frequently solitary, well-circumscribed, non encapsulated or multinodular mass
  • Brown or gray in color and rubbery in texture
  • Variable sizes, from 0.2 cm to 5.5 cm (Virchows Arch 2015;466:245)
  • Micro Description
  • Prominent "bubbly" low power aspect
  • Cystic, tubular, solid or papillary architecture
  • Intermediate size cells with eosinophilic / amphophilic vacuolated cytoplasm; absence of zymogen granules
  • Low grade, bland and pale nuclei, some with prominent nucleoli
  • Intraluminal or intracellular colloid-like material with a "bubbly" appearance
  • May have mucinous differentiation (Am J Surg Pathol 2012;36:27)
  • May have perineural invasion
  • No extensive necrosis and very low mitotic activity
  • Unusual feature: foci of high grade/dedifferentiation with large nests and comedonecrosis (Virchows Arch 2015;466:245)
  • Micro Images

    Case of the Week #382:

    Mammaglobin



    Images courtesy of Olguta Gologan, M.D. and Jeffrey Krane, M.D., Ph.D.:

    Missing Image Missing Image Missing Image

    Prominent "bubbly" colloid-like material on low power

    Missing Image Missing Image Missing Image

    Various histological patterns

    Missing Image Missing Image Missing Image

    Intermediate sized cells

    Missing Image

    S100+

    Missing Image

    Mammaglobin+

    Missing Image

    CK7+

    Missing Image

    PASD

    Missing Image

    DOG1-

    Missing Image

    p63-

    Cytology Description
  • Cellular smears; background of eosinophilic, filamentous matrix and mucin
  • Sheets and clusters of bland polygonal epithelial cells with patterns ranging from acinar-like structures, papillae and tubuloglandular (Head Neck Pathol 2013;7:S30)
  • Most tumor cells have abundant small cytoplasmic vacuoles, some containing mucin; others will have a finely eosinophilic granular cytoplasm
  • Dispersed, isolated cells with a "histiocyte-like" appearance
  • Nuclei are uniform, small, round, eccentrically located, with small nucleoli
  • Cytology Images

    Images courtesy of Jeffrey Krane, M.D., Ph.D.:

    Missing Image

    Sheets and clusters cells

    Missing Image

    Nuclei are uniform

    Positive Stains
  • Cytokeratins: CK7, CK8, CK18, CK19, CKAE1/AE3, CAM 5.2
  • S100, STAT5a (strong, diffuse), GCDFP-15 (most cases) (Head Neck Pathol 2013;7:S30)
  • Tumor cells and eosinophilic material will stain for mammaglobin
  • With appropriate morphology, the positivity of both S100 and mammaglobin are strongly indicative of MASC (Head Neck Pathol 2015;9:85)
  • Eosinophilic secretory material stains for PAS / PASD and Alcian blue
  • Negative Stains
  • DOG1; p63
  • Myoepithelial markers: p63, calponin, CK14, SMA and CK5/6
  • ER, PR
  • Molecular / Cytogenetics Description
  • t(12;15)(q13;q25) translocation, a fusion of the ETV6 and NTRK3 gene (also identified in secretory breast carcinoma, infantile fibrosarcoma and acute myelogenous leukaemias)
  • Other ETV6 translocation partner (ETV6-X fusion) (Am J Surg Pathol 2015;39:602)
  • Additional References