Mammary analogue secretory carcinoma of the salivary gland with NTRK fusions: New approaches for diagnostics and targeted therapy (review)

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a rare salivary cancer, histologically resembling to secretory carcinoma of the breast. In 2017 World Health Organization reported MASC is a new salivary cancer subtype. The aim of this article is to collect and analyze data about MASC, particularly clinical, histological and molecular profile, to evaluate targeted therapy effects. We discuss a case report of dramatic and durable response with entrectinib and the development of acquired resistance in an NTRK3.fusion positive salivary cancer, detected by next-generation sequencing. Next-generation sequencing as a comprehensive molecular profiling, that helps to investigate molecular profile of rare tumors and gives an opportunity to use an effective therapeutic options. Identifying ETV6.NTRK3 positive MASC provides a better prognosis for metastatic disease by using a novel effective targeted therapy with tyrosine kinase inhibitors (entrectinib, larotrectinib). Despite a durable and dramatic response, we showed an interesting case of the development of acquired resistance to tyrosine kinase inhibitors mediated by the appearance of a novel NTRK3 G623R mutation. Finally, we believe in great perspectives of comprehensive molecular profiling and targeted therapy for rare malignancies with NTRK gene fusions, including second-generation tyrosine kinase inhibitors. © 2020 Academic Press. All rights reserved.

Ignatova A.V. 1, 2 , Mudunov A.M. 1, 3 , Podvyaznikov S.O. 1 , Alymov Yu.V.3
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  • 1 Russian Medical Academy of Continuing Professional Education, Ministry of Health of Russia, Bld. 1, 2/1 Barrikadnaya St., Moscow, 125993, Russian Federation
  • 2 RUDN University, 6 Miklukho-Maklaya St., Moscow, 117198, Russian Federation
  • 3 N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia, 24 Kashirskoe Shosse, Moscow, 115478, Russian Federation
Ключевые слова
Entrectinib; ETV6-NTRK3; Fluorescent in situ hybridization; Immunohistochemistry; Larotrectinib; Mammary analogue secretory carcinoma; Next-generation sequencing; Polymerase chain reaction; Tyrosine kinase inhibitors
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