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Case Reports
. 2023 Sep;17(3):803-807.
doi: 10.1007/s12105-023-01554-w. Epub 2023 Apr 28.

Differentiated Papillary NUT Carcinoma: An Unexpected, Deceptively Bland Presentation of a Sinonasal Carcinoma

Martin Wartenberg #  1 , Sara-Lynn Hool #  2 , Andrea Marrazzini  3 , Roland Giger #  2 , Niels J Rupp #  4   5
Affiliations
Case Reports

Differentiated Papillary NUT Carcinoma: An Unexpected, Deceptively Bland Presentation of a Sinonasal Carcinoma

Martin Wartenberg et al. Head Neck Pathol. 2023 Sep.

Abstract

Background: In recent years, the list of tumor entities in the sinonasal tract has significantly expanded, requiring advanced diagnostic testing. We report the case of a 32-year-old patient with an unusual NUT carcinoma originating in the maxillary sinus, which showed extensive well-differentiated, papillary squamous morphology, similar to the spectrum of the recently described DEK::AFF2 fusion-associated carcinoma.

Methods: We performed immunohistochemical and molecular studies including EBV- and HPV-testing, as well as DNA/RNA next generation sequencing.

Results: The tumor showed predominantly exophytic papillary growth with mature squamous differentiation. An additional component harbored atypical, less differentiated basaloid tumor cells with infiltration of the adjacent stroma. Conspicuous inflammation was evident. There was no evidence of HPV DNA or EBV RNA. Next-generation sequencing revealed a NUT::NSD3 gene fusion corresponding to ("speckled-type") immunopositivity of NUT in the tumor cells.

Conclusions: We describe a NUT::NSD3 gene fusion-associated NUT carcinoma of the sinonasal tract with a deceptively well-differentiated papillary growth pattern, thus expanding the morphological spectrum of this typically poorly differentiated neoplasm.

Keywords: DEK::AFF2 carcinoma; EBV- and HPV-associated carcinomas; NUT carcinoma; Undifferentiated as well as SWI/SNF complex deficient sinonasal carcinomas.

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Conflict of interest statement

The authors declare that they have no conflict of interest. The authors have no financial or non-financial interests that are directly or indirectly related to the work submitted for publication to disclose.

Figures

Fig. 1
Fig. 1
Oro-antral fistula on the alveolar ridge of the left upper jaw after tooth extraction 25
Fig. 2
Fig. 2
A Coronal CT scan of the paranasal sinuses showing a large bone defect and opacification of the left maxillary sinus. B FDG-PET/CT showing an intensely metabolically active tumor with osseous destruction of the left maxillary sinus
Fig. 3
Fig. 3
A–D Histopathology of the tumor: A Microscopically, the tumor shows papillomatous-exophytic well-differentiated squamous differentiation (10x), B–C infiltration of basaloid-appearing, less differentiated cells into the adjacent stroma (20x and 40x), D accompanied by prominent granulocytic infiltration (20x). E Immunohistochemistry for NUT (C52B1, Rabbit mAb, Cell Signaling Technology) (40x, magnified inset)
See this image and copyright information in PMC

References

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