Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases
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ORIGINAL PAPER
Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases Abbas Agaimy1 · Sarina K. Mueller2 · Thomas Harrer3 · Sebastian Bauer4 · Lester D. R. Thompson5 Received: 24 January 2018 / Accepted: 26 February 2018 © Springer Science+Business Media, LLC, part of Springer Nature 2018
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. Keywords Kaposi sarcoma · Ear canal · Differential diagnosis · Oral cavity · Head and neck · HIV · HHV8 · Immunohistochemistry
Introduction Kaposi sarcoma (KS) is an uncommon angioproliferative endothelial neoplasm with distinctive clinicopathological, epidemiological and immunophenotypic characteristics. Diagnosis relies on a combination of morphological and immunohistochemical findings in the appropriate * Abbas Agaimy abbas.agaimy@uk‑erlangen.de 1
Institute of Pathology, University Hospital, Krankenhausstrasse 8‑10, 91054 Erlangen, Germany
2
Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital, 91054 Erlangen, Germany
3
Department of Internal Medicine‑3, University Hospital, 91054 Erlangen, Germany
4
Sarcoma Center, Western German Cancer Center, University of Duisburg-Essen Medical School, 45122 Essen, Germany
5
Department of Pathology, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA 91367, USA
clinicopathological settings [1]. Since its original description by the Hungarian dermatologist Moritz Kaposi in 1872 as “idiopathic multiple pigmented sarcoma,” [2], KS has been classified into five distinct clinical settings: (1) classical (idiopathic)
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