Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands.

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  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: England NLM ID: 9709118 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1879-0593 (Electronic) Linking ISSN: 13688375 NLM ISO Abbreviation: Oral Oncol Subsets: MEDLINE
    • Publication Information:
      Publication: Amsterdam : Elsevier
      Original Publication: Oxford ; New York : Pergamon, c1997-
    • Subject Terms:
      Oncogene Proteins, Fusion/*genetics ; Salivary Gland Neoplasms/*pathology; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Salivary Gland Neoplasms/genetics ; Survival Analysis ; Young Adult
    • 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.
      (Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.)
    • Contributed Indexing:
      Keywords: ETV6-NTRK3; Gene Fusion; Head and Neck Neoplasms; MASC; Mammary analogue secretory carcinoma; Prognosis; Salivary gland neoplasms; Secretory carcinoma
    • Accession Number:
      0 (ETV6-NTRK3 fusion protein, human)
      0 (Oncogene Proteins, Fusion)
    • Publication Date:
      Date Created: 20180619 Date Completed: 20191213 Latest Revision: 20191217
    • Publication Date:
      20221213
    • Accession Number:
      10.1016/j.oraloncology.2018.04.022
    • Accession Number:
      29909898