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Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

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Abstract

Kaposi sarcoma (KS) of the head and neck area is uncommon with limited published case series. Our routine and consultation files were reviewed for histologically and immunohistochemically proven KS affecting any cutaneous or mucosal head and neck site. Ten males and one female aged 42–78 years (median, 51 years; mean, 52 years) were retrieved. Eight patients were HIV-positive and three were HIV-negative. The affected sites were skin (n = 5), oral/oropharyngeal mucosa (n = 5), and lymph nodes (n = 3) in variable combination. The ear (pinna and external auditory canal) was affected in two cases; both were HIV-negative. Multifocal non-head and neck KS was reported in 50% of patients. At last follow-up (12–94 months; median, 46 months), most of patients were either KS-free (n = 8) or had ongoing remission under systemic maintenance therapy (n = 2). One patient was alive with KS (poor compliance). Histopathological evaluation showed classical features of KS. One case was predominantly sarcomatoid with prominent inflammation mimicking undifferentiated sarcoma. Immunohistochemistry showed consistent expression of CD31, CD34, ERG, D2-40 and HHV8 in all cases. This is one of the few series devoted to head and neck KS showing high prevalence of HIV-positivity, but also unusual presentations in HIV-negative patients with primary origin in the skin of the ear and the auditory canal. KS should be included in the differential diagnosis of difficult-to-classify spindle cell lesions at this uncommon location.

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Authors and Affiliations

  1. Institute of Pathology, University Hospital, Krankenhausstrasse 8-10, 91054, Erlangen, Germany

    Abbas Agaimy

  2. Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital, 91054, Erlangen, Germany

    Sarina K. Mueller

  3. Department of Internal Medicine-3, University Hospital, 91054, Erlangen, Germany

    Thomas Harrer

  4. Sarcoma Center, Western German Cancer Center, University of Duisburg-Essen Medical School, 45122, Essen, Germany

    Sebastian Bauer

  5. Department of Pathology, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA, 91367, USA

    Lester D. R. Thompson

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  1. Abbas Agaimy
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  2. Sarina K. Mueller
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  3. Thomas Harrer
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  4. Sebastian Bauer
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  5. Lester D. R. Thompson
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Correspondence to Abbas Agaimy.

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All authors declare that they have no conflict of interest as it relates to this research project.

Ethical Approval

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

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Agaimy, A., Mueller, S.K., Harrer, T. et al. Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases. Head and Neck Pathol 12, 511–516 (2018). https://doi.org/10.1007/s12105-018-0902-x

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  • DOI: https://doi.org/10.1007/s12105-018-0902-x

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