Discussion
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. The presence of a recurrent, auricular KS with
an atypical presentation in a young immunocompetent individual is a very
rare finding.
In this article, we presented a case of recurrent KS on the ear with a
literature review on ear KS cases (Table 1). 5, 6,
8-27 The literature review disclosed 24 cases since the year 1941 until
2020, highlighting the rarity of this presentation. Sixteen males and
seven females aged 3-85 years (median, 62 years; mean, 57.4 years) were
retrieved. Of these 24 cases of ear skin KS, 2 cases were
HIV-positive24, 27, 13 cases were
HIV-negative6, 16-23, 25, 26 and others were unknown.
Among these, 4 cases had visceral involvement including lymph node,
bone, urethra, stomach, colon, liver and spleen and the rest were
limited to the skin including Just limited to the auricle (n=11), ear
and mucosal sites (n=4), ear and extremities (n=9), ear and other sites
in the head and neck region (chin, eyelid) (n=3). Among the 13
HIV-negatives, 5 cases had some degrees of immunosuppression (1 case
kidney transplantation25, 1 case Congenital
immunodeficiency25, 1 case receiving systemic
corticosteroid23, 1 case non-Hodgkin
lymphoma21 and the last a case of severe
lymphocytopenia17. While excision was the most common
treatment option, other modalities were antiretroviral medications for
HIV-positives, radiotherapy and chemotherapy with liposomal doxorubicin,
bleomycin, vincristine and IFN-alpha for more widespread disease. Among
17 cases that their follow up were available, ranging from 15 months to
17 years, the majority of them were free of disease after the initial
treatment (n=12), 3 cases had recurrent lesions, 1 case was alive with
disease and 1 died with disease because of uremia. While KS in
HIV-negative patients has an indolent coarse, our case was highly
recurrent, despite total excision with free margins, it has recurred
twice in five years and after that, a new lesion on the foot appeared.
So the recurrence rate of the KS in the ear needs to be further studied.
While KS pathogenesis is multifactorial and both genetic and environment
are responsible, Human Herpes Virus 8 (HHV8) is the main causal factor
in the development of KS in all variants irrespective of the
clinicopathological setting of the disease. 4, 28 HHV8
contributes to cell growth, signaling apoptosis, angiogenesis, and
immunomodulation. It produces some proteins that inhibit host adaptive
and innate immunity. 1, 4 While the increased risk of
KS in HIV-positives and iatrogenically immunosuppressed cases is well
understood, the occurrence in immunologically competent individuals
remains largely unelucidated. 7 Agaimy A et al
hypothesized that maybe impaired local immunosurveillance and
pro-inflammatory cytokines release is the causative factor.6 Although the exact reason why the ear is a
predilection site in HIV negative patients who develop KS in head and
neck region is not clear Francés RL et al proposed that in addition to
some factors such as trauma and infection in acral sites, insufficient
vascularization makes it difficult for immune system to access.22
Due to the rarity of head and neck, KS, especially in HIV- negative
patients, unusual presentations of KS may be challenging if not
considered in the differential diagnosis. The occurrence of KS in
atypical sites like ear leads to unrecognition and misdiagnosis. The
possibility of occult HIV infection should be considered beside. They
may be misdiagnosed as other spindle cell tumors pathologically or other
vascular lesions such as ALHE clinically. HHV8 immunohistochemistry was
positive in 95 % of KS lesions irrespective of HIV
positivity4, so it is a good marker to detect KS.
In summary, we presented a case of recurrent ear KS in a young
HIV-negative and otherwise healthy individual with a review of the
literature on 24 cases of ear KS from 1941 to 2020 implicating ear as a
predilection site for head and neck KS in HIV-negative patients,
therefore, we highly suggest to consider KS as a differential diagnosis
for lesions on ears.
Author 1: has made substantial contributions to conception and design,
acquisition of data, analysis and interpretation of data, drafting the
manuscript and revising it critically, given final approval of the
version to be published. Author 2: has made substantial contributions to
conception and design, acquisition of data, analysis and interpretation
of data, drafting the manuscript and revising it critically. Author 3:
has made substantial contributions to conception and design, drafting
the manuscript and revising it critically, Author 4: has made
substantial contributions to conception and design, acquisition of data,
analysis and interpretation of data, drafting the manuscript and
revising it critically.