Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

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.

Intraoral granulocytic sarcoma as a manifestation of myelofibrosis: A case report and review of the literature

“…Of these, only the AIDS‐related (acquired immunodeficiency syndrome) type is associated with oral manifestations. Oral KS most frequently occurs on the hard palate, followed by the gingiva and the tongue, as purplish, reddish blue, or dark brown macules, plaques, and nodules that may ulcerate . The second most frequent site, gingiva, and the clinical presentation of oral KS are consistent with the present case but there was no previous diagnosis of AIDS.…”

Atypical Manifestations in Classic Kaposi Sarcoma: Case Series of Two Patients HIV - Negative

“…Head and neck involvement in HIV-negative patients is uncommon, reported in less than 5% of classical forms. Classical skin lesions generally consist of angioedematous plaques and nodules located on the limbs, on the contrary, HIV-related form is characterised by disseminated cutaneous manifestations, with oral and craniofacial involvement in about 95% of patients [7], [8]. However, in our cases, chemotherapy with vinblastine seems to be effective, with control of atypical manifestations without evidence of skin recurrence or visceral involvement.…”

Recurrent Kaposi Sarcoma of the Ear in an HIV-negative patient: A Case report with review of the literature. Is ear a predilection site for Kaposi sarcoma in HIV-negatives?

“…While oral (59.1%) and craniofacial (43.9%) involvement is common in HIV-positives(1), Kaposi sarcoma of the head and neck is rare (approximately <5 % of the KS cases) in the HIV-negative individuals. 6 The most common presentation of KS in HIV-negatives is multiple bilateral lesions of the lower extremities. 7 Among the head and neck KS, the incidence of auricular involvement is much lower, so it should be considered a distinct manifestation.…”