Primary squamous cell carcinoma of thyroid with a novel BRAF mutation and High PDL-1 expression: A case report with treatment implications and review of literature.


Journal

Pathology, research and practice
ISSN: 1618-0631
Titre abrégé: Pathol Res Pract
Pays: Germany
ID NLM: 7806109

Informations de publication

Date de publication:
Oct 2020
Historique:
received: 18 05 2020
revised: 07 07 2020
accepted: 24 07 2020
pubmed: 28 8 2020
medline: 17 8 2021
entrez: 28 8 2020
Statut: ppublish

Résumé

Primary squamous cell carcinoma of thyroid (SCC-T) is an extremely rare, aggressive neoplasm with median survival of 9 months. Pure squamous morphology with absence of other cell types is required for diagnosis of SCC-T. Clinically, SCC-T behaves like anaplastic thyroid carcinoma (ATC) showing rapid growth, and extra thyroidal extension. We report a 91-year-old woman presenting with an enlarging thyroid mass and accompanying dysphagia and hoarseness. Fine needle aspiration revealed hypercellular specimen with large, pleomorphic, malignant cells. Intraoperative assessment revealed an inoperable tumor involving both thyroid lobes and extensively infiltrating surrounding soft tissues. A subtotal thyroidectomy was performed. Histology revealed squamous cell carcinoma replacing native thyroid tissue and infiltrating adjacent skeletal muscle. Lymphovascular and perineural invasion were present. Immunohistochemistry showed tumor cells positive for CK5-p40, Pax-8, TTF-1 and negative for thyroglobulin. P53 expression by IHC was high and Ki-67 proliferation index was > 90 %. (Next generation sequencing revealed a novel BRAF mutation (BRAF c.1799 T > A; 1801_1812del) along with TP53 and TERT mutations. PDL-1 immunohistochemistry showed positive expression in tumor cells (>80%), making patient also amenable to anti-PDL-1 immunotherapy. Patient was treated with BRAF inhibitor therapy with initial relief but eventually was put on hospice care due to increasing intolerance to therapy. This case represents a rare thyroid malignancy with a unique molecular signature consisting of a novel BRAF mutation [previously not described in SCC-T or ATC], associated with TERT-TP53 mutations. Further, importance of PDL-1 testing as a prognostic marker and as a guide to immunotherapy in refractory tumors is discussed.

Identifiants

pubmed: 32853962
pii: S0344-0338(20)32001-X
doi: 10.1016/j.prp.2020.153146
pii:
doi:

Substances chimiques

B7-H1 Antigen 0
Biomarkers, Tumor 0
CD274 protein, human 0
BRAF protein, human EC 2.7.11.1
Proto-Oncogene Proteins B-raf EC 2.7.11.1

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

153146

Informations de copyright

Copyright © 2020 Elsevier GmbH. All rights reserved.

Auteurs

Mary Torrez (M)

Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States.

Ryan C Braunberger (RC)

Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States.

Emrullah Yilmaz (E)

Department of Oncology/ Hematology, University of New Mexico Comprehensive Cancer Center, Albuquerque, New Mexico, United States.

Shweta Agarwal (S)

Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States. Electronic address: shagarwal@salud.unm.edu.

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Classifications MeSH