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Review
. 2023 Jan-Feb;26(1):59-64.
doi: 10.1177/10935266221138706. Epub 2022 Nov 30.

A Pediatric Case of High-Grade Secretory Carcinoma of the Maxillary Sinus With ETV6::NTRK3 Gene Fusion, Therapeutic Implications, and Review of the Literature

Antonello Cardoni  1 , Rita De Vito  1 , Giuseppe Maria Milano  2 , Maria Debora De Pasquale  2 , Rita Alaggio  1
Affiliations
Review

A Pediatric Case of High-Grade Secretory Carcinoma of the Maxillary Sinus With ETV6::NTRK3 Gene Fusion, Therapeutic Implications, and Review of the Literature

Antonello Cardoni et al. Pediatr Dev Pathol. 2023 Jan-Feb.

Abstract

Secretory carcinoma (SC) is a salivary gland tumor with a generally low grade microscopic appearance, a characteristic immunophenotype, and a recurrent translocation leading to ETV6::NTRK3 fusion gene. Rare cases are reported in children. The maxillary sinus is an unusual localization. SC have an overall favorable prognosis, but cases with high grade morphology have been described in adult population and are related to a more aggressive clinical course. We present a pediatric case of secretory carcinoma involving the maxillary sinus with high grade morphology, with a review of the literature of secretory carcinomas with high grade component.

Keywords: pediatric secretory carcinoma high grade.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
(A) Low-grade component with papillary/tubular architecture. (B) Higher magnification of the low-grade component, with cells with dense chromatin and inconspicuous nucleoli; (C) High grade component with solid pattern. Note the transition from the low-grade (blue arrow) to the high-grade (yellow arrow) component and the microscopic focus of necrosis (white star); (D) Higher magnification of the high-grade component, with cells with vesicular chromatin, evident nucleoli, and higher mitotic index; (E) PAS-D positive luminal secretion; (F) S100; (G) GATA3; (H) pan-TRK; (I) MRI, neoplasm before treatment; (J) MRI, after 3 courses of chemotherapy.
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