Disseminated Oral Kaposi Sarcoma Lesion with Extensive Visceral Involvement

View metadata, citation and similar papers at core.ac.uk brought to you by CORE

provided by Elsevier - Publisher Connector Oral Oncology EXTRA (2006) 42, 18–21

http://intl.elsevierhealth.com/journal/ooex

CASE REPORT Disseminated oral Kaposi sarcoma lesion with extensive visceral involvement

Kishore Shetty *

University of Texas Health Sciences Center, Medically Complex Patient Clinic, 6516 M.D. Anderson Blvd., Suite 475, Houston, TX 77030, United States

Received 7 August 2005; accepted 9 August 2005

KEYWORDS Summary Kaposi’s sarcoma (KS) has been considered the most common malig- Kaposi sarcoma; nancy associated with human immunodeﬁciency virus (HIV) infection because of Disseminated; its propensity to develop in such individuals.This article, reports an unusual presen- HIV; tation of a disseminated oral KS lesion with extensive visceral involvement in an AIDS otherwise healthy young African-American male leading to a positive diagnosis of HIV. c 2005 Elsevier Ltd. All rights reserved.

Case report The patient’s extra oral exam revealed a normo- cephalic facial symmetry with no signs or symp- A 26-year-old African-American male presented at toms of acute infection, swelling or tenderness. the Oral Surgery Outpatient Clinic for evaluation There was a raised well defined reddish nodular of an oral lesion. He first noticed a ‘‘gum boil’’ four mass measuring 2.0 · 1.8 cm on the posterior side months ago which has been steadily increasing in of his head (Fig. 1). Palpable lymph nodes were size since its appearance. He failed to seek medical appreciated on his left submandibular and poster- attention for this lesion since it was not painful or ior cervical chains; the nodes were firm, mobile has not bled in the past. He also reported unex- and painless. Poor oral hygiene was noted with plained weight loss in the last six months and feb- moderate dental plaque and calculus accumulation rile episodes. His previous medical history was and generalized gingival edema. Bleeding on peri- unremarkable. He reported no known drug allergy. odontal probing was generalized. There was no At the time of our examination he was taking amox- radiographic evidence of bone loss and the deepest icillin. He denied tobacco use, alcohol use, illicit probing was around 4 mm. Additionally, there was drugs use or intravenous drug use. a2· 1.8 cm raised, dark red, soft, exophytic enlargement of the mandibular attached gingiva * Tel.: +1 713 500 4210. of lower right canine on the facial and lingual as- E-mail address: [email protected] pect, with a smooth surface, ill defined margins

Figure 1 A raised well deﬁned reddish nodular mass Figure 3 A2· 1.8 cm raised, dark red, soft, exophytic measuring 2.0 · 1.8 cm on the posterior side of the head. enlargement of the mandibular attached gingiva of lower right canine on the lingual aspect.

Figure 2 A raised, dark red, soft, exophytic enlargement of the mandibular attached gingiva of lower right Figure 4 An ill-defined raised, diffuse, reddish, soft, canine on the facial aspect, with a smooth surface, and nodular enlargement on the posterior right hard palate ill defined margins. focal areas of ulceration involving the soft palate and the uvula and progressing into the oropharynx. and negative blanching (Figs. 2, 3). The lesion was not tender to palpation and showed no erosions or tached gingiva. A local anesthesia infiltration of ulcerations. Another 5 · 4 cm ill-defined raised, the right mental nerve was obtained using 1.8 mL diffuse, reddish-blue, soft, nodular enlargement of lidocaine 2% with epinephrine 1:100,000. The on the posterior right hard palate was observed, tumor was excised and placed in 10% formalin involving the soft palate and the uvula and pro- and submitted for histopathological diagnosis. gressing into the oropharynx (Fig. 4). This lesion The histologic examination revealed a cellular showed an ulcerated surface and there was no ten- spindle tumor within the connective tissue, numer- derness. The patient repeatedly denied any history ous extravasated erythrocytes and poorly defined of dysphasia or dyspnea or difficulty to chew asso- vascular slits. These microscopic findings plus the ciated with this lesion. There were no other oral le- clinical presentation and the medical history were sions, tumors or masses noted intraorally. consistent with a diagnosis of Kaposi’s sarcoma. Two other dark-red nodules were observed on Additionally, head and neck magnetic resonance his left arm and forehead with the same character- imaging (MRI) with contrast solution were ordered istics described for the oral lesions (Figs. 5, 6). The for further assessment of respiratory and/or esoph- patient agreed to undergo an incisional biopsy of ageal tract obstruction and did not show any major the exophytic tumor on the facial aspect of at- obstruction of upper airways. 20 K. Shetty

course. The disease, first described by the Hungar- ian dermatologist Moritz Kaposi in 1872, was rare and found classically in elderly men of Italian, Jew- ish, or Slavic ancestry.1 With the advent of the AIDS epidemic, KS has become one of the most common malignancies associated with HIV infection and was one of the first diseases to define AIDS in 1981.2 KS is traditionally separated into four different types: classic, which primarily affects elderly men of Mediterranean and eastern European origin; endemic, which is common in parts of Africa; epidemic or AIDS-associated; and transplantation- associated. The human herpes virus-8 infection and its pathogenic process characterize all four types. Although most Americans remain at low risk for infection of AIDS-HHV-8 KS, homosexual and Figure 5 A dark-red macule less than 0.5 · 0.5 cm in diameter on the left forehead. bisexual men are at strikingly high risk as a result of sexual transmission in some form.3 KS sometimes begins as a single lesion of the oral mucosa but more frequently begins as multiple lesions. Fifty percent of the affected patients show oral lesions and 20–25% of these lesions may be the initial site of involvement.4 KS can invade bone and create tooth mobility when involves the palate or gingiva. The lesion initially is a flat brown or reddish purple macule that does not blanch with pres- sure. The neoplasm may develop into plaques or nodules, and this progressive malignancy may disseminate to lymph nodes and organ systems. Morbidity of KS oral lesions may be associated with pain, bleeding, and functional interferences caused by the tumor. Months before manifestation of the tumors, HHV-8 viremia leads to development of specific Figure 6 A dark-red nodule on his left arm. antibodies. In most cases, epidemic KS causes widespread lesions that erupt in many places soon We discussed the results with patients and the after AIDS develops. Lesions of epidemic KS may risk factors for HIV were elucidated. He agreed to arise on the skin and the mouth and may affect take the enzyme-linked immunosorbent assay the lymph nodes and other organs, usually the gas- (ELISA) test. The test was positive and his status trointestinal tract, lung, liver, and spleen. In contrast, classic KS usually involves only one or a few as HIV seropositive was confirmed by the western 5 blot procedure. Subsequent biopsies of the cutane- areas of skin, most often the lower legs. At the ous and pharyngeal lesions were consistent with a time of diagnosis of epidemic KS some people similar diagnosis of AIDS-related Kaposi’s sarcoma. experience no symptoms, especially if their only le- At the time of his hospital admission his CD4+ sions are on the skin. However, many of those with lymphocytes count was 4.8 cells/cc3 and his CD8+ epidemic KS – even those with no skin lesions, will lymphocytes count was 57.3 cells/cc3 (CD4/CD8 have swollen lymph nodes, unexplained fever, or ratio = 0.1) with a viral load of 35000. weight loss. Eventually, in almost all cases, epidemic KS spreads throughout the body. Extensive KS lung involvement can be fatal. More often, however, patients die of other AIDS-related complica- Discussion tions, such as infections.6 KS afflicts of 10–20% of HIV-positive patients. Kaposi’s sarcoma (KS) is a malignant, multifocal Some studies suggest that HIV-positive patients systemic disease that originates in the vascular with Kaposi’s sarcoma have a shorter survival than endothelium and has a very variable clinical might be predicted from their CD4 concentrations.7 Disseminated oral Kaposi sarcoma lesion with extensive visceral involvement 21

The authors reported that patients with lesions of prevention. 4th ed. Lippincott-Raven: Philadelphia; 1997. the oral mucosa had a higher death rate than those p. 295–317. with exclusively cutaneous manifestations of the 3. Brodt HR, Karnps BS, Helm EB, Schofer H, Mitrou P. Kaposi’s sarcoma in HIV-infection: impact on opportunistic infection disease. and survival. AIDS 1998;12:1475–81. KS treatment has been rapidly improving with 4. Tambussi G, Repetto L, Torri V, et al. Epidemic HIV-related many new options currently under study or recently Kaposi’s sarcoma: a retrospective analysis and validation of proven to be efficacious and licensed for use in pa- TIS staging. GICAT. Gruppo Italiano Collaborativo AIDS e tients with AIDS.8 Various local therapies aim to Tumori. Ann Oncol 1995;6:383–7. 5. Pauk J, Huang M-L, Brodie SJ, et al. Mucosal shedding of eradicate small lesions; however, they do not af- human herpes virus 8 in men. New Engl J Med 2000;343: fect the KS in general nor the likelihood of its 1369–77. recurrence. Systemic chemotherapy treats exten- 6. Levine AM, Tulpule A. Clinical aspects and management of sive visceral involvement, which no other form of AIDS-related Kaposi’s sarcoma. Eur J Cancer 2001;37: treatment may affect.9,10 Newer treatment ap- 1288–95. 7. Rohrmus Bettina, Thoma-Greber Eva M, Bogner R Johannes, proaches are aimed at HHV-8 or at the inflamma- Rocken M. Outlook in oral and cutaneous Kaposi’s sarcoma. tory cytokine or angiogenic milieu necessary for Lancet 2000;356(9248):2160. KS growth. 8. Grulich AE, Li Y, McDonald AM, Correll PK, Law MG, Kaldor JM. Decreasing rates of Kaposi’s sarcoma and non-Hodgkin’s lymphoma in the era of potent combination antiretroviral therapy. AIDS 2001;15:629–33. References 9. Dupont C, Vasseur E, Beauchet A, Aegerter P, Berthe H, de Truchis P, et al. Long-term efficacy on Kaposi’s sarcoma of 1. Moore P. The emergence of Kaposi’s sarcoma-associated highly active antiretroviral therapy in a cohort of HIV- Herpes virus (Human Herpes virus 8). New Engl J Med positive patients. AIDS 2000;14:987–93. 2000;343(19):1411–3. 10. Portsmouth S, Stebbing J, Gill J, Mandalia S, Bower M, 2. Safai B. Kaposi’s sarcoma and acquired immunodefi- Nelson M, et al. A comparison of regimens based on non- ciency syndrome. In: DeVita VT, Hellman JS, Rosenberg nucleoside reverse transcriptase inhibitors in preventing SA, editors. AIDS: etiology, diagnosis, treatment and Kaposi’s sarcoma. AIDS 2003;17:F17–22.