Objectives: In 2010, a new subtype of salivary gland cancer (SGC), (mammary analogue) secretory carcinoma (SC), was defined, characterized by the ETV6-NTRK3 fusion gene. As clinical behavior and outcome data of this histological subtype tumor are still sparse, we aimed to describe the clinicopathological course and outcome of a series of translocation positive SC patients. Patient and methods: We re-evaluated the pathological diagnosis of a subset of SGCs, diagnosed in 4 of 8 Dutch head and neck centers. Subsequently, tumors with a morphological resemblance to SC were tested for the ETV6-NTRK3 fusion gene using RT-PCR. Furthermore, patients prospectively diagnosed with SC were included. The clinical characteristics and outcomes were retrieved from the patient files. Results: Thirty-one patients with ETV6-NTRK3 fusion gene positive SC were included. The median age was 49 years, 17 patients (55%) were male. Eighteen tumors (58%) arose in the parotid gland. One patient presented with lymph node metastasis. All patients underwent tumor resection and 4 patients had a neck dissection. Four patients had re-resection and 15 patients (48%) received postoperative radiotherapy. One patient developed a local recurrence, no regional recurrences or distant metastases were observed. After a median follow-up of 49 months the 5- and 10-year overall survival were 95%, the 5- and 10-year disease free survival were 89%. Conclusion: The clinical course of SC is favorable with a low rate of locoregional recurrence and excellent survival. Given the low incidence of nodal metastases, elective neck treatment, i.e. surgery and/or radiotherapy, does not seem to be indicated.

Additional Metadata
Keywords ETV6-NTRK3, Gene Fusion, Head and Neck Neoplasms, Mammary analogue secretory carcinoma, MASC, Prognosis, Salivary gland neoplasms, Secretory carcinoma
Persistent URL dx.doi.org/10.1016/j.oraloncology.2018.04.022, hdl.handle.net/1765/106412
Journal Oral Oncology
Citation
Boon, E. (E.), Valstar, M.H. (M. H.), van der Graaf, W.T.A, Bloemena, E, Willems, S.M, Meeuwis, C.A, … Herpen, C.M.L. (2018). Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands. Oral Oncology, 82, 29–33. doi:10.1016/j.oraloncology.2018.04.022