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Mammary analogue secretory carcinoma of salivary glands, containing the ETV6-NTRK3 fusion gene: a hitherto undescribed salivary gland tumor entity
Alena Skálová, Tomas Vanecek, Radek Sima, Jan Laco, Ilan Weinreb, Bayardo Perez-Ordonez, Ivo Starek, Marie Geierova, Roderrick HW. Simpson, Fabricio Passador-Santos, Ales Ryska, Ilmo Leivo, Zdenek Kinkor, Michal Michal
Language English Country United States
Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't
Grant support
NS9725
MZ0
CEP Register
Digital library NLK
Full text - Article
Source
NLK
Journals@Ovid Ovid Full Text
from 2000-01-01 to 2010-02-01
- MeSH
- Cystadenocarcinoma genetics metabolism pathology MeSH
- Adult MeSH
- Oncogene Proteins, Fusion genetics metabolism MeSH
- Gene Rearrangement MeSH
- In Situ Hybridization, Fluorescence MeSH
- Immunohistochemistry MeSH
- Combined Modality Therapy MeSH
- Middle Aged MeSH
- Humans MeSH
- Chromosomes, Human, Pair 12 MeSH
- Chromosomes, Human, Pair 15 MeSH
- Young Adult MeSH
- Neoplasms, Multiple Primary MeSH
- Biomarkers, Tumor metabolism MeSH
- Salivary Gland Neoplasms genetics metabolism pathology MeSH
- Reverse Transcriptase Polymerase Chain Reaction MeSH
- RNA, Neoplasm analysis MeSH
- Aged MeSH
- Salivary Glands surgery MeSH
- Translocation, Genetic MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Young Adult MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
We present a series of 16 salivary gland tumors with histomorphologic and immunohistochemical features reminiscent of secretory carcinoma of the breast. This is a hitherto undescribed and distinctive salivary gland neoplasm, with features resembling both salivary acinic cell carcinoma (AciCC) and low-grade cystadenocarcinoma, and displaying strong similarities to breast secretory carcinoma. Microscopically, the tumors have a lobulated growth pattern and are composed of microcystic and glandular spaces with abundant eosinophilic homogenous or bubbly secretory material positive for periodic acid-Schiff, mucicarmine, MUC1, MUC4, and mammaglobin. The neoplasms also show strong vimentin, S-100 protein, and STAT5a positivity. For this tumor, we propose a designation mammary analogue secretory carcinoma of salivary glands (MASC). The 16 patients comprised 9 men and 7 women, with a mean age of 46 years (range 21 to 75). Thirteen cases occurred in the parotid gland, and one each in the minor salivary glands of the buccal mucosa, upper lip, and palate. The mean size of the tumors was 2.1 cm (range 0.7 to 5.5 cm). The duration of symptoms was recorded in 11 cases and ranged from 2 months to 30 years. Clinical follow-up was available in 13 cases, and ranged from 3 months to 10 years. Four patients suffered local recurrences. Two patients died, 1 of them owing to multiple local recurrences with extension to the temporal bone, and another owing to metastatic dissemination to cervical lymph nodes, pleura, pericardium, and lungs. We have shown a t(12;15) (p13;q25) ETV6-NTRK3 translocation in all but one case of MASC suitable for analysis. One case was not analyzable and another was not available for testing. This translocation was not found in any conventional salivary AciCC (12 cases), nor in other tumor types including pleomorphic adenoma (1 case) and low-grade cribriform cystadenocarcinoma (1 case), whereas ETV6-NTRK3 gene rearrangements were proven in all 3 tested cases of mammary secretory carcinoma. Thus, our results strongly support the concept that MASC and AciCC are different entities.
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