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Head and neck cancer, excluding thyroid

2425 - Mammary Analogue Secretory Carcinoma (MASC): clinical characteristics in 28 ETV6-NTRK3 fusion gene confirmed patients.

Date

09 Sep 2017

Session

Head and neck cancer, excluding thyroid

Presenters

Eline Boon

Citation

Annals of Oncology (2017) 28 (suppl_5): v372-v394. 10.1093/annonc/mdx374

Authors

E. Boon1, M.H. Valstar2, W.T.A. van der Graaf1, E. Bloemena3, S.M. Willems4, C.A. Meeuwis5, L.A. Smit6, M.A.W. Merkx7, R.P. Takes8, U.E. Flucke9, C.M.L. Herpen10

Author affiliations

  • 1 Department Of Medical Oncology, Radboud university medical centre, 6525AG - Nijmegen/NL
  • 2 Department Of Head And Neck Oncology And Surgery, Antoni van Leeuwenhoek / Netherlands Cancer Institute, Amsterdam/NL
  • 3 Department Of Pathology, VU University Medical centre, Amsterdam/NL
  • 4 Department Of Pathology, University Medical Centre Utrecht, 3584 CX - Utrecht/NL
  • 5 Department Of Otorhinolaryngology / Head & Neck Surgery, Erasmus University Medical Centre, Rotterdam/NL
  • 6 Department Of Pathology, Antoni van Leeuwenhoek / Netherlands Cancer Institute, Amsterdam/NL
  • 7 Department Of Oral And Maxillofacial Surgery, Radboud university medical centre, 6525AG - Nijmegen/NL
  • 8 Department Of Otorhinolaryngology, Radboud University Medical Centre, 6525AG - Nijmegen/NL
  • 9 Department Of Pathology, Radboud University Medical Centre, 6525AG - Nijmegen/NL
  • 10 Department Of Medical Oncology, Radboud University Medical Centre, 6525AG - Nijmegen/NL
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Resources

Abstract 2425

Background

Recently, a new subtype of salivary gland cancer (SGC), mammary analogue secretory carcinoma (MASC), has been defined, which is characterized by the presence of ETV6-NTRK3 fusion gene. Previously, MASC was mixed up with acinic cell carcinoma (AciCC), polymorphous low grade adenocarcinoma and (cyst)adenocarcinoma. At present the clinical features and outcome of MASC patients are not well known. We aimed to describe the clinical presentation and outcome of MASC.

Methods

Firstly, we re-evaluated the pathological diagnosis of salivary gland cancers with a morphological resemblance to MASC, diagnosed in 4 of the 8 head and neck centres in the Netherlands, for their presence of ETV6-NTRK3 and also included genetically confirmed prospectively diagnosed cases. The ETV6-NTRK3 fusion gene was analyzed using RT-PCR. Secondly, the clinical characteristics were retrieved from the patient files.

Results

Twenty-eight patients with ETV6-NTRK3 fusion gene positive MASC were included (10 prospectively and 18 retrospectively). Of these 18 retrospective patients 13 patients were previously diagnosed as AciCC, the other 5 patients as (low-grade) adenocarcinoma. The median age at diagnosis was 49 years (range 19 – 83 years), 15 patients (54%) were male. The duration of symptoms varied from 6 weeks until 20 years with a median of 14 months. In 18 patients (64%) the tumour was located in the parotid gland; the other patients had tumours of the minor salivary glands (2), submandibular gland (1), oral mucosa/lip (5) or palate (2). All patients had a T1-2 tumour. One patient had lymph node metastasis at diagnosis. All patients underwent surgery of which 4 patients needed re-resection and 12 patients (43%) underwent postoperative radiotherapy. One patient had a local recurrence 50 months after primary surgery, but was cured after second resection. None of the patients had regional recurrences or distant metastases. The median follow-up was 49 months and both the 5- and 10-year overall survival rate were 94%.

Conclusions

MASC is a recently acknowledged new entity of SGC characterized by the ETV6-NTRK3 fusion gene. The clinical course seems to be favourable with a very low rate of recurrences and an excellent survival.

Clinical trial identification

Clinical trial identification not applicable

Legal entity responsible for the study

Radboudumc

Funding

None

Disclosure

All authors have declared no conflicts of interest.

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