Diagnostic challenges and successful organ-preserving therapy in a case of secretory carcinoma of minor salivary glands.


Journal

Cancer reports (Hoboken, N.J.)
ISSN: 2573-8348
Titre abrégé: Cancer Rep (Hoboken)
Pays: United States
ID NLM: 101747728

Informations de publication

Date de publication:
03 2022
Historique:
revised: 14 06 2021
received: 04 05 2021
accepted: 22 06 2021
pubmed: 8 7 2021
medline: 26 4 2022
entrez: 7 7 2021
Statut: ppublish

Résumé

Secretory carcinoma is a more recently described subtype of salivary gland carcinoma that may pose diagnostic challenges and frequently harbors NTRK fusions that may successfully be targeted by TRK inhibitors in advanced disease. We present the case of a female patient with secretory carcinoma arising in the base of tongue with persistent disease after debulking surgery and definitive chemoradiation. As an alternative to salvage surgery, which would have resulted in significant impairment of swallowing and speech function, a targeted therapy with the TRK-inhibitor larotrectinib against an identified ETV6-NTRK3 fusion product was initiated. Larotrectinib treatment has been well tolerated, resulted in durable complete response and the patient maintains good swallowing and speech function. The presented case underscores the importance of the accurate diagnosis of secretory carcinoma. It further highlights the impact of molecular testing as targeted therapies may play an important role in the management of advanced salivary gland cancers.

Sections du résumé

BACKGROUND
Secretory carcinoma is a more recently described subtype of salivary gland carcinoma that may pose diagnostic challenges and frequently harbors NTRK fusions that may successfully be targeted by TRK inhibitors in advanced disease.
CASE
We present the case of a female patient with secretory carcinoma arising in the base of tongue with persistent disease after debulking surgery and definitive chemoradiation. As an alternative to salvage surgery, which would have resulted in significant impairment of swallowing and speech function, a targeted therapy with the TRK-inhibitor larotrectinib against an identified ETV6-NTRK3 fusion product was initiated. Larotrectinib treatment has been well tolerated, resulted in durable complete response and the patient maintains good swallowing and speech function.
CONCLUSION
The presented case underscores the importance of the accurate diagnosis of secretory carcinoma. It further highlights the impact of molecular testing as targeted therapies may play an important role in the management of advanced salivary gland cancers.

Identifiants

pubmed: 34231337
doi: 10.1002/cnr2.1491
pmc: PMC8955062
doi:

Substances chimiques

Oncogene Proteins, Fusion 0

Types de publication

Case Reports Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e1491

Subventions

Organisme : NCI NIH HHS
ID : P01 CA240239
Pays : United States
Organisme : postdoc.mobility fellowship of the Swiss National Science Foundation
ID : P400PM_183852

Informations de copyright

© 2021 The Authors. Cancer Reports published by Wiley Periodicals LLC.

Références

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Auteurs

Ruben Bill (R)

Center for Systems Biology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Daniel G Deschler (DG)

Department of Otolaryngology - Head and Neck Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts, USA.

Mikael J Pittet (MJ)

Center for Systems Biology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
Department of Pathology and Immunology, University of Geneva, Geneva, Switzerland.
Department of Oncology, Geneva University Hospitals, Geneva, Switzerland.

Sara I Pai (SI)

Department of Otolaryngology - Head and Neck Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts, USA.
Department of Surgery, Division of Oncologic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Peter M Sadow (PM)

Departments of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Jong Chul Park (JC)

Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

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