Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands.
Loading...
Embargo End Date
ICR Authors
Authors
Boon, E
Valstar, MH
van der Graaf, WTA
Bloemena, E
Willems, SM
Meeuwis, CA
Slootweg, PJ
Smit, LA
Merkx, MAW
Takes, RP
Kaanders, JHAM
Groenen, PJTA
Flucke, UE
van Herpen, CML
Valstar, MH
van der Graaf, WTA
Bloemena, E
Willems, SM
Meeuwis, CA
Slootweg, PJ
Smit, LA
Merkx, MAW
Takes, RP
Kaanders, JHAM
Groenen, PJTA
Flucke, UE
van Herpen, CML
Document Type
Journal Article
Date
2018-07
Date Accepted
2018-04-26
Abstract
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.
Citation
Oral oncology, 2018, 82 pp. 29 - 33
Rights
Source Title
Publisher
ISSN
1368-8375
eISSN
1879-0593
Collections
Research Team
Clinical and Translational Sarcoma