Large mass arising from the tongue as an initially and sole manifestation of Kaposi sarcoma.

We report a 30- year-old Iranian woman presenting with a red to yellowish, well demarcated, painless exophytic and lobulated mass originating from the right hand side of the tongue. An excisional biopsy was obtained and it was diagnosed histopathologically as Kaposi's sarcoma by detecting atypical spindle cells with rare mitoses delineating blood-filled vascular slits.

Four types of Kaposi's sarcoma (KS) as a vascular neoplasm with mucocutaneus involvement has been described: classic, endemic, HIV-related and iatrogenic KS that occurs most commonly in patients who receive immunosuppressive therapy 1.

We report a 30- year-old Iranian woman presenting with a red to yellowish, well demarcated, painless exophytic and lobulated mass originating from the right hand side of the tongue (Figure 1). The lesion had started as a small tumor 4 months before. It later became extensive and friable and transformed into a rapid growing flesh to yellowish colored mass that began interfering with her speaking and eating. Additionally since almost 5 years ago she has suffered from intractable pemphigus vulgaris with oral erosions and cutaneous bullous lesions which had been treated with prednisolone and azothioprin. Otherwise physical examination and routine laboratory and HIV tests were normal. An excisional biopsy was obtained and it was diagnosed histopathologically as Kaposi's sarcoma by detecting atypical spindle cells with rare mitoses delineating blood-filled vascular slits.

Figure 1

Red to yellowish, well demarcated, painless exophytic and lobulated mass originating from right hand side of the tongue.

Traditionally, Kaposi sarcoma has a general distribution, often appearing first on the limbs 2. Kaposi's sarcoma is a frequently seen as an AIDS-related malignant neoplasm in the head and neck region, especially in the oral cavity, but is rarely described in HIV-negative or non-immunosuppressed individuals 3. Oral involvement occurs in approximately 11% of all cases described 4. Initial oral involvement is an even rarer occurrence, with only fourteen cases previously documented in literature for non-HIV related KS 5. A statistically increased incidence of malignancy has been observed in patients with pemphigus 6 but notably iatrogenic KS occurs infrequently in the setting of pemphigus vulgaris 7. Interestingly, while immunosuppression is highly associated with KS, it cannot be considered an etiology 5. Basically it is believed that immunosuppression creates an environment that allows an opportunistic factor to cause KS 5, 8. HHV8 is considered to be the etiological agent of all forms of Kaposi's sarcoma 9. In Iran a high prevalence of HHV8 infection has been observed in several risk groups such as haemodialysis, renal transplant and HIV-positive patients 10 but unfortunately we did not test the patient for HHV8 DNA. A number of dermatologic diseases can be present as an oral mass such as bacillary hemangioma, lymphoma and squamous cell carcinoma 11. Accordingly, Kaposi's sarcoma should be considered as a differential diagnosis of oral masses in patients especially if they are treated with immunosuppressive agents. The patient successfully underwent surgery (Figure 2) and there was no recurrence after more than 1 year of follow up.

Figure 2

Immediately after surgery