Abstract. In 90% of cases of AIDS-associated Kaposi's sarcoma (KS), the lesion is observed in the oral cavity. Oral KS usually reveals distinct clinical features.
Non-pigmented oral Kaposi's sarcoma (AIDS) Report of two cases
P e t e r A. R e i c h a r t 1 a n d M o r t e n Schiedt = 1Abteilung f/Jr Zahn&rztliche Chirurgie/ Oralchirurgie, Freie Universit&t Berlin, W. Germany, 2Institute of Oral Pathology and Medicine, Royal Dental College, and Department of Oral Medicine & Oral Surgery, University Hospital, Copenhagen, Denmark
P. A. Reichart and M. Schiodt: Non-pigmented oral Kaposi's sarcoma (AIDS). Report o f two cases. Int. J. Oral Maxillofac. Surg. 1989; 18." 19~199. Abstract. In 90% of cases of AIDS-associated Kaposi's sarcoma (KS), the lesion is observed in the oral cavity. Oral KS usually reveals distinct clinical features characterized by a brown-bluish or otherwise pigmented appearance. The histological features are identical to classical KS. The occurrence of a non-pigmented oral KS in 2 male homosexual patients has p r o m p t e d the present case reports. Clinicians should be aware that not all cases of AIDS-associated oral KS appear as brown or purplish tumors but may present without any discoloration.
AIDS-associated Kaposi's sarcoma (KS) was first described in 1981 in young male homosexual men 3. While the classical f o r m of KS is rare, about 30% of all A I D S patients, primarily homosexual men, develop this lesion 5. Declines in the proportion o f KS among cases of A I D S in multiple risk groups have been observed 4. Although all organs may be affected by KS, skin, and particularly oral mucosa, may show early involvement; 50 to 90% of A I D S patients with KS may develop oral manifestations 1°,12. These are often located on the hard palate, particularly in the area where the p a l a t i n e vessels leave the palatal bone 6'13. Initially, oral KS appears as a flat brownish or purplish macule, located in the palatal mucosa or gingiva. In later stages, a nodular appearance is characteristic. Longstanding gingival KS may lead to widening of the periodontal space and alveolar bone destruction 9. M o r p h o l o gically, oral KS is identical to the classical form of K S and characterized by atypical vascular channels, prominent spindle cells, extravasated erythrocytes, hemosiderin and the presence of eosinophilic bodies, chronic inflammatory cells and a few mitotic figures in tumor stage lesions 6'13. Ultrastructurally, tumor cells of one kind reveal characteristics of normal endothelial cells such as Weibel-Palade bodies, multivesicular bodies, tight intercellular junctions and basement membranesS.~L A second type of cell is represented by intervascular spindle-shaped stromal cells with blunt-
Key words: AIDS; Kaposi's sarcoma. Accepted for publication 10 April 1989
ended enlarged nuclei. Basement membrane and connective tissue proteins 11 as well as other collagen types have been studied in oral KS lesions ~. The purpose of the present paper was to describe a clinical variety of A I D S associated KS. In addition to oral KS lesions with typical pigmented appearance, neoplasms without any discoloration may appear and give rise to diagnostic problems.
lesion on the right side of the palate had regressed. In August 1986 oral examination revealed sublingual ecchymosis which was due to an immune thrombocytopenia. On the dorsum of the tongue a 2 x 2 cm firm, welldefined lesion of normal red color was seen. The lesion was 5 mm in height. A tentative diagnosis of Iymphoma or atypical KS was made (Fig. 1). Biopsy revealed the characteristic features of KS. In October 1986 the patient's condition rapidly worsened and he died in December 1986 from Pneumocystis carinii pneumonia.
Case reports Case 1
Case 2
A 30-year-old homosexual male was first seen in the Dept. of Oral Medicine and Oral Surgery in June 1985 (University Hospital, Copenhagen). Previous history revealed a hepatitis B infection in 1985 and syphilis in 1981. In November 1984, pneumonia had developed. T4/T8 ratio was 0.3 and the absolute numbers of T-helper cells was 0.2 x 109/1. Cervical lymph nodes were enlarged, HIV-seropositivity was confirmed. There were no further symptoms until June 1985 when the patient developed multifocal AIDS-associated KS. Oral examination (June 1985) revealed a bluish palatal lesion of the right side with some ulceration. Another blue-brown lesion w a s noted on the alveolar process in the area of the 2nd right mandibular molar. Biopsies from the palatal lesions were not taken because KS was clinically obvious and biopsies from skin lesions had confirmed the diagnosis. In July 1985, the patient was treated with alpha-interferon. By this time, the T4/ T8 ratio was 0.1, 80 million units of alphainterferon were administered over 5 days. Treatments were repeated 4 times with intervals and no new lesions occurred. The KS
A 42-year-old male, homosexual patient was first seen in May 1986 (Dept. of Oral Surgery, West Berlin). HIV-seropositivity was known since September 1985. When first examined the patient had multifocal extraoral and in-
Fig. 1. Tumorous lesion on the dorshm of the tongue with normal appearing color of the oral mucosa (Case 1).
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Reichart & Schiodt Discussion
Fig. 2. Nodular to granular appearance of the hard and part of the soft palate, representing non-pigmented oral KS. The color of the covering oral mucosa is normal (Case 2).
traoral AIDS-associated KS lesions. Extraorally, the lesions were located on the face, particularly the tip of the nose, which was severely affected. Intraorally, a non-pigmented lesion was observed in the soft and hard palate on the right and left sides (Fig. 2). The surface of the oral mucosa was irregular, appearing almost like a lymphangioma or lymphoma. A biopsy was taken from the right and left regions close to the 3rd upper
molars. Histologically, AIDS-associated KS was diagnosed (Fig. 3). The 1st and 2nd molars in the right maxilla were sligthly mobile. Radiological examination showed destruction of alveolar bone. Radiation therapy (20 Gy) was administered to both the intraoral lesion and the nasal KS. However, the facial and intraoral lesions regressed, and the patient died in August 1986 from an opportunistic infection.
AIDS-associated Kaposi's sarcoma is frequently observed in homosexual men. Due to discoloration, the diagnosis of KS is easily established, particularly in those cases where there is known seropositivity for H I V antibodies. Gingival lesions, such as peripheral giant cell granulomas or pyogenie granulomas, however, may be confused with gingival KS lesions 2. In cases where oral KS does not appear as a blue, purplish or brownish macule or nodule, even if other typical lesions occur simultaneously, the clinical diagnosis may be difficult. Since other neoplasms, particularly l y m p h o m a s may occur in HIV-infected individuals 13'14, biopsies are mandatory. In the cases presented oral KS occurred as lesions which, clinically, could not be diagnosed as KS. The typical pigmentation was missing and only the biopsy revealed the nature o f the lesion. Histological features in both eases were characteristic for nodular KS, with vascular channels, spindle-shaped cells, hemosiderin, extravasated erythrocytes and eosinophilic granules. The a m o u n t of extravasated erythrocytes was comparable to that observed in pigmented lesions, but in one case there were fewer vascular channels than in pigmented KS lesions. The mucosa overlying the nodules was not markedly thickened. The spectrum of oral manifestations in H I V infections is immense. Their appearance and behavior, however, does not always follow patterns k n o w n from non-HIV-infected patients. The observation of non-pigmented oral KS is another demonstration of the variety of lesions seen in the oral cavity in H I V infections. The color which usually is so characteristic for intraoral K S may not always be present. Biopsies are mandatory to reveal the true nature o f such lesions.
References
Fig. 3. Higher magnification of non-pigmented KS. Vascular spaces are lined by swollen endothelial cells. The spindle-cell component is seen between vascular lumina (HE x 250).
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