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Case Reports
. 2025 Jan 7;19(1):7.
doi: 10.1007/s12105-024-01746-y.

EWSR1::BEND2 Adenocarcinoma with Hyaline Pseudorosettes of the Trachea

Juan J Sanchez-Ramirez  1 , Matthew R Hoffman  1 , John C Keech  2 , Anthony N Snow  1 , Andrew M Bellizzi  1 , Anand Rajan  3   4   5
Affiliations
Case Reports

EWSR1::BEND2 Adenocarcinoma with Hyaline Pseudorosettes of the Trachea

Juan J Sanchez-Ramirez et al. Head Neck Pathol. .

Abstract

We describe the novel occurrence of a adenocarcinoma involving the trachea, with distinct solid and glandular components, in a 34-year-old patient. We illustrate its morphological and immunophenotypic features and describe the molecular finding of an EWSR1::BEND2 gene fusion detected by next-generation sequencing (NGS). We discuss the findings in comparison to BEND2-fusion associated neoplasms reported in the head and neck region in the literature to date.

Keywords: BEN domain proteins; BEND2; ESWR1::BEND2 Fusion; Gene fusion; Salivary gland carcinoma.

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Conflict of interest statement

Declarations. Ethics Approval \ Patient Consent: This report does not contain any personal identifying information. The University of Iowa Human Subjects Office determined that the study did not meet the regulatory definition of human subjects’ research as it is a report on a single case and exempted review by the IRB. Conflict of Interest: The following authors have no financial disclosures: J.J.S.R, M.R.H, J.C.K, A.N.S, A.M.B, A.R.

Figures

Fig. 1
Fig. 1
Resection - histologic features. (A) Panoramic view of tumor from the tracheal resection (2.5x), with transmural extension of tumor across the tracheal wall (asterisk). (B) Higher magnification view showing condensed cellular tumor around branched leaf-like vascular stroma (arrows), and areas with clear cells (arrowheads) (10x). (C) Numerous pseudorosettes with hyalinized stromal cores (arrowheads), adjacent to vascular stromal cores (arrows) (20x). (D) Submucosal area with prominent hyalinized stromal whorls (arrowheads) (28x). (E) Solid sheets of tumor, with nests of clear cells (arrowheads) and small hyaline stromal cores of pseudorosettes (arrows) (24x). (F) mucosal aspect showing a thin and flattened squamous epithelial lining (arrows) (40x). (G) Deeper area with distinct glandular differentiation (28.6x)
Fig. 2
Fig. 2
Immunohistochemical and molecular findings. Panels (A) p40 nuclear positivity in tumor cells, while being negative in the glandular component (28x). (B) Smooth muscle actin is negative in tumor cells and highlights myoepithelial cells in entrapped benign glands and stromal elements (20x). (C) S100 protein is negative in tumor cells while highlighting nerve twigs (25x). (D) p63 expression parallels p40 expression and is negative in glandular elements and stroma (20x). (E) cytoplasmic expression of pan-keratin AE1/AE3, accentuated in the central rim of the pseudorosette cells (arrows) while diminished in tumor cells immediately outward (asterisk) (20x). (F) Diffuse nuclear BEND2 positivity in tumor nuclei (40x). (G) Archer Analysis software visualization showing fusion between EWSR1 exon 7 and BEND2 exon 2
See this image and copyright information in PMC

References

    1. Abhiman S, Iyer LM, Aravind L (2008) BEN: a novel domain in chromatin factors and DNA viral proteins. Bioinformatics 24(4):458–461. 10.1093/bioinformatics/btn007 - DOI - PMC - PubMed
    1. Todorovic E, Dickson BC, Weinreb I (2020) Salivary gland Cancer in the era of routine next-generation sequencing. Head Neck Pathol 14:311–320 - DOI - PMC - PubMed
    1. Palsgrove DN, Manucha V, Park JY, Bishop JA (2023) A low-grade Sinonasal Sarcoma harboring EWSR1::BEND2: expanding the Differential diagnosis of Sinonasal Spindle Cell Neoplasms. Head Neck Pathol 17(2):571–575. 10.1007/s12105-023-01527-z - DOI - PMC - PubMed
    1. Weinreb I, Rooper L, Bishop JA, Bell D, Dogan S, Xu B et al (eds) (2024) 3/28/2024 EWSR1::BEND2 Fusion Defines a Novel Salivary Gland Adenocarcinoma with a Predilection for the Pharynx: Description of an Emerging Entity and Validation of a Specific BEND2 Antibody. United States and Canadian Academy of Pathology 113th Annual Meeting;; Baltimore: Nature; 2024
    1. Zhang YD, Sun JJ, Xi SY, Jiang ZM, Xie DR, Yang Q, Zhang XC (2024) Malignant salivary gland neoplasm of the Tongue Base with EWSR1::BEND2 Fusion: an unusual case with Literature Review. Head Neck Pathol 18(1):118. 10.1007/s12105-024-01726-2 - DOI - PMC - PubMed

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