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Epstein-Barr Virus-Associated Smooth Muscle Tumors of Larynx: A Clinicopathologic Study and Comprehensive Literature Review of 12 Cases

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Abstract

Laryngeal mesenchymal neoplasms are rare, with smooth muscle tumors comprising a small subset. Specifically, Epstein-Barr virus (EBV)-associated smooth muscle tumors are exceptionally rare, lacking a comprehensive evaluation of their clinical and histologic features. Two patients (a 59 year old male and 51 year old female) had received renal transplants 156 and 240 months, respectively prior to onset of laryngeal symptoms. Supraglottic polypoid masses were identified and removed conservatively. Histologically, the tumors were hypercellular, showing alternating light and dark areas, the latter composed of primitive appearing round cells, while a more characteristic spindled tumor cell population was noted in the remaining areas. Cytoplasmic vacuoles were noted adjacent to the nucleus. There was no tumor necrosis or pleomorphism, but increased mitotic figures (11–12/2 mm2) were seen, without atypical forms. The tumor cells were strongly immunoreactive with smooth muscle actin and smooth muscle myosin heavy chain and with Epstein-Barr virus encoded RNA (EBER) by in situ hybridization. These patients were reviewed in the context of a thorough English literature review, which demonstrates a wide age range at presentation without a sex predilection, but with most patients from specific ethnic groups (Chinese, Thai, Pilipino). Three-quarters of patients are part of multifocal disease and the majority are post-renal transplantation patients. Conservative management seems to yield the best overall outcome for these indolent tumors. In conclusion, EBV-associated smooth muscle tumors should be considered in any immunocompromised patient with a head and neck smooth muscle tumor, especially when EBER is documented by in situ hybridization. Conservative management may be employed, even when multifocal tumors are documented.

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Authors and Affiliations

  1. Cleveland Clinic Foundation, Cleveland, OH, USA

    Rumeal D. Whaley

  2. Department of Pathology, Southern California Permanente Medical Group, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA

    Lester D. R. Thompson

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  1. Rumeal D. Whaley
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Correspondence to Lester D. R. Thompson.

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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 #5968), which did not require informed consent. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of Southern California Permanente Medical Group.

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Whaley, R.D., Thompson, L.D.R. Epstein-Barr Virus-Associated Smooth Muscle Tumors of Larynx: A Clinicopathologic Study and Comprehensive Literature Review of 12 Cases. Head and Neck Pathol 15, 1162–1171 (2021). https://doi.org/10.1007/s12105-021-01328-2

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