Ⅰ.INTRODUCTION

Kaposi sarcoma (KS) is a malignant mesenchymal neoplasm arising from endothelial cells that could involve skin, mucous membrane, or solid organ.(1) KS has four main epidemiologic variants according to the inciting factor of disease.

1.Classic, or sporadic

2.Endemic (African)

3.Epidemic (AIDS-related)

4.Iatrogenic (post-transplant).(2)

Lesions can arise anywhere along the GI tract from the oral cavity to the perianal region. It has been clearly shown that Human herpesvirus 8 (HHV-8) is a crucial factor in disease pathogenesis because HHV-8 DNA is found in almost all cases of KS and in all of the clinical forms.(3)

KS was a rare skin cancer affecting elderly, some Endemic origin before 1979(pre-AIDS era) and became the first indicator of a devastating pandemic caused by soon to be discovered HIV 1980-1995(pre-HAART era) (4, 5). The incidence of KS started to drop significantly after the mid-1990s (HAART era). Introduction of Highly Active Antiretroviral Therapy (HAART) partially restores immune system function(6). Presently, HIV infection is considered as the greatest risk factor for KS. Oral-KS is one of the first recognized opportunistic diseases in human immunodeficiency virus (HIV) infection.(7) About 22% of HIV patients with KS have oral involvement as the initial manifestation. In up to 71% of HIV patients, there are oral KS along with dermal and visceral lesions. In a South African epidemiological study, Khammissa et al. showed that the hard palate and gingival area is the most common site of oral involvement (30%), with the dorsum of the tongue being the least common (5%).(7)

Here, we present a case of KS who was unaware about his HIV infection and was referred from local clinic due to long standing oral discomfort. The patient was tested and later was confirmed for HIV infection after the histopathological diagnosis of KS. Patient did not have any other sign or symptom of HIV infection/AIDS besides oral swelling. So, oral examination plays a very important role for early detection of KS as a first sign of HIV infection to reduce disease burden.

Ⅱ.CASE REPORT

2017. March, a 48-year-old male patient with a chief complain of pain during eating and bleeding on tooth brushing was referred from local dental clinic to the oral and maxillofacial surgery at Seoul National University Dental Hospital for the evaluation of the swelling on both posterior maxillary area and left mandibular angle.

He reported that the symptoms started 6 months ago. He was unaware of viral infection at the time of reporting. Intraoral examination revealed diffuse, red, blue enlargement with widespread necrosis in the posterior maxilla and mandible on both sides Fig.1.

An incisional biopsy was performed on #38 operculum area for the pathological diagnosis. The Histopathological examination with routine H&E revealed microanatomical features of a spindle cell tumor with mixed blood vessels and hemorrhage consistent with KS. Pathological findings revealed that consistent with KS. Fig.2. Immunohistochemically staining showed that the tumor spindle cells express vascular markers Factor VIII, CD31, CD34, and they were positive for HHV8(LANA). Fig.3. Fig.4, confirming the diagnosis of KS. The patient was confirmed for HIV (Ag, Ab) using serum analysis.

The patient was referred to the ‘Division of Hematologic Cancer department’ for further treatment. On physical exam, a nodular erythematous and friable lesion approximately 3 by 4 centimeters was present on the right cheek and biopsy was done. Division of Hematologic Cancer department made treatment plan and explained the patient about HIV and favorable prognosis followed by HAART but he failed to keep his appointment and failed to follow up.

2017 July, patient was admitted to the emergency room for malaise and lethargy. He reported that he only took small rice porridge for 2 weeks due to pain in mouth and dysphagia. He denied fever but had chilly feeling. At that time, his viral load was over 57,781 copies per milliliter (ml) and absolute CD4 T helper cell lymphocyte (CD4) count of 31 cells per microliter. From the blood work, the patient was found to be positive for P. carnii. Later with chest X-ray, the patient confirmed Pneumocystis pneumonia.

Ⅲ.DISCUSSION

KS is an important opportunistic disease, which frequently occurs in HIV+ pt.(8) The most frequent site of involvement is the skin followed by the gastrointestinal tract. The gastrointestinal system can be affected by multicentric lesions observed from oral cavity, oropharynx and esophagus to the perianal area.(9)

In a study of 138 patients with HIV-1-associated Kaposi’s Sarcoma in 2000, oral mucosa involvements are very critical because it had a higher death rate than those having exclusively cutaneous manifestations of the disease.(10)

Head and neck involvement in AIDS-KS is very high as 40-67%, in which the cutaneous lesions predominate. Also, oral lesions represent the first sign of KS in 22% of HIV-positive individuals, and ultimately, 71% of these patients will develop AIDS-associated oral KS (AIDS-OKS) (11, 12). The most frequent intraoral site are the hard palate, soft palate, gingival and dorsal tongue involved in this order of frequency.(13) The color of the lesions can range from dark pink, red, purple or brown.

There is a unique relationship between neoplastic transformation of KS and immune function. Epidemiologic studies suggested an association between all types of KS and the infectious etiology by the oncogenic human herpes virus type 8 (HHV-8), also known as Kaposi’s sarcoma herpes virus (KSHV) and a high concentration of KSHV laboratory markers KS patients [9].

Kaposi’s sarcoma has been reported in HIV-negative people also. A retrospective study in 2008 showed a group of 28 men who were all HIV-negative, which shows that KS can rarely present in the absence of significant immunosuppression.(14)

The focus of treatment among HIV patients with KS should be initiation and maintenance of HAART therapy. Proper compliance of HAART therapy may result in a less severe form of the disease or even possibly regression. It is likely that initiation of HAART therapy can both improve immune system function and reduce HIV-1 viral load. (15) Treatment goals include pain control, and reestablishment of oral function which ultimately results in improvement of quality of life for these patients. The burden of this tumor can be significant for many patients as it may cause a reduction in quality of life due to features like tumor- associated edema, dysphagia.

As a clinician, questions about associated risks and evaluation of possible oral lesion findings, may allow in making a diagnosis sooner and reduce overall disease burden.

Ⅳ.SUMMARY

The Kaposi’s sarcoma involving oral mucosa in HIV positive patient has a greater mortality risk compared with only cutaneous involvement of Kaposi’s sarcoma. Oral Kaposi’s sarcoma can be an important prognostic factor for patients free from other opportunistic infections. So, early detection of KS oral involvement could be a crucial role to reduce disease burden.