Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands.
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Date
2018-07ICR Author
Author
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
Type
Journal Article
Metadata
Show full item recordAbstract
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.
Collections
Subject
Humans
Salivary Gland Neoplasms
Neoplasm Recurrence, Local
Oncogene Proteins, Fusion
Survival Analysis
Adult
Aged
Aged, 80 and over
Middle Aged
Female
Male
Young Adult
Research team
Clinical and Translational Sarcoma
Language
eng
Date accepted
2018-04-26
License start date
2018-07
Citation
Oral oncology, 2018, 82 pp. 29 - 33