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Teratocarcinosarcoma-Like and Adamantinoma-Like Head and Neck Neoplasms Harboring NAB2::STAT6: Unusual Variants of Solitary Fibrous Tumor or Novel Tumor Entities?

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Abstract

The archetypal solitary fibrous tumor (SFT) features fibroblastic cells with varying cellularity without any particular architectural arrangement in a collagenous matrix, with staghorn vessels, CD34 and STAT6 expression, and NAB2::STAT6. To date, this fusion is thought to be specific for SFT. With more routine use of fusion gene panels, the histologic diversity of NAB2::STAT6-positive tumors is increasingly appreciated. Here we present four head and neck tumors harboring NAB2::STAT6 but exhibiting remarkably unusual morphologic features for SFT. All cases were pulled from the authors’ consultation files. Immunohistochemistry was performed, along with targeted RNA sequencing in all cases, plus DNA next-generation sequencing on two. The cases arose in the nasal cavity (n = 2), retromolar trigone (n = 1) and parapharynx (n = 1), in patients ranging from 39 to 54 (mean, 44). Both nasal cases were biphasic, with a variably cellular collagenized stroma that resembled SFT but also interspersed malignant epithelial and neuroepithelial nests. One of the nasal cases also exhibited overt rhabdomyoblastic differentiation within both components. The two non-nasal cases were comprised of plump, epithelioid cells that were diffusely positive for pan-cytokeratin. One of these cases had prominent cystic change lined by overtly squamous epithelium. STAT6 immunostaining was positive in all cases, although the epithelial/neuroepithelial nests in the sinonasal cases were negative. All cases were confirmed to harbor NAB2::STAT6 by RNA sequencing. The two sinonasal cases were also found to harbor oncogenic mutations. The presented cases highlight a much broader histologic diversity than previously known for neoplasms with NAB2::STAT6. The biphasic nasal cases closely resemble teratocarcinosarcoma, while the epithelioid, cytokeratin-positive cases could be conceptualized as “adamantinoma-like,” to borrow terminology already in use for Ewing sarcomas with complex epithelial differentiation. To identify similar cases, pathologists should have a low threshold for using STAT6 immunohistochemistry on any difficult-to-characterize head and neck tumor.

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Possible upon reasonable request, deidentified for maintenance of anonymity and compliance with IRB approval.

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Not applicable.

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Funding

This study was funded by the Jane B. and Edwin P. Jenevein M.D Endowment for Pathology at UT Southwestern Medical Center. No external funding was obtained for this study.

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Authors and Affiliations

  1. Department of Pathology, University of Alabama at Birmingham, Birmingham, AL, USA

    Todd M. Stevens

  2. Department of Pathology & Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA

    Todd M. Stevens

  3. Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Lisa M. Rooper

  4. Laboratorio Bacchi, São Paulo, Brazil

    Carlos E. Bacchi & Igor Lima Fernandes

  5. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Cristina R. Antonescu

  6. Department of Pathology, University of Texas Southwestern Medical Center, 6201 Harry Hines Blvd, Dallas, TX, 75390, USA

    Jeffrey Gagan & Justin A. Bishop

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  1. Todd M. Stevens
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  2. Lisa M. Rooper
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  3. Carlos E. Bacchi
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  6. Jeffrey Gagan
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  7. Justin A. Bishop
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All authors confirm they have meaningfully contributed to the research and read and approved the final manuscript.

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Correspondence to Justin A. Bishop.

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All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

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All procedures performed in this retrospective data analysis involving human participants were in accordance with the ethical standards of the institutional review board (IRB 112017-073), which did not require informed consent.

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Stevens, T.M., Rooper, L.M., Bacchi, C.E. et al. Teratocarcinosarcoma-Like and Adamantinoma-Like Head and Neck Neoplasms Harboring NAB2::STAT6: Unusual Variants of Solitary Fibrous Tumor or Novel Tumor Entities?. Head and Neck Pathol 16, 746–754 (2022). https://doi.org/10.1007/s12105-022-01444-7

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  • DOI: https://doi.org/10.1007/s12105-022-01444-7

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