Primary nasal Kaposi Sarcoma in well-controlled HIV-positive patient with normal CD4 count associated with long-term nasal steroid use

Introduction

Kaposi sarcoma (KS) is a systemic, multifocal angioproliferative disease caused by human herpesvirus 8 (HHV-8). There are four categories of KS: classic, endemic, epidemic or acquired immunodeficiency syndrome (AIDS)-related, and iatrogenic. While prior studies have shown an association between extensive use of systemic corticosteroids and the development or worsening of KS, ¹ to our knowledge, this may be the first case of KS arising in the setting of intranasal steroid use in a patient with well-controlled HIV.

Case report

A 63-year-old man of Ashkenazi descent with a 19-year history of HIV, as well as chronic severe allergic rhinitis, presented with a nasal polyp. Five years prior, his allergic rhinitis was treated with prednisone (initial dose 40 mg daily with taper over 2 weeks until discontinuation). Since that time, he had been effectively managed with inhaled nasal fluticasone 50 mg per spray 1–2 puffs once to twice daily in addition to azelastine spray. Current antiviral treatment constituted nevirapine plus emtricitabine/tenofovir alafenamide. Since initiation of antiretroviral therapy (ART) 15 years ago, HIV viral load had always been undetectable (<40 copies/mL) and the patient did not have a history of opportunistic infections. The recent screenings of rapid plasma reaign (syphilis), hepatitis C, and hepatitis B surface antigen were all negative (normal).

The nasal lesion was initially concerning for a pyogenic granuloma. However, excisional biopsy revealed KS stemming from the squamo-epithelial junction of the anterior lateral nares (Figure 1). Histopathologic examination revealed fascicles of monomorphic spindle cells, hyaline globules, and abundant extravasated red blood cells; HHV-8 immunostaining confirmed the diagnosis of KS (Figure 2). At the time of KS diagnosis, HIV viral load was <20 copies/mL and CD4 count was 1408 cells/µL (normal CD4 count ranges from 500 to 1200 cells/µL). Intranasal fluticasone was discontinued and the patient was instructed to use this medication judiciously in the future. A dermatologic full body skin examination showed no additional KS lesions, and a repeat nasal examination five months later showed no recurrence of disease.OPEN IN VIEWER

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Figure 2.

Histopathologic images. While there are four clinically distinct categories of Kaposi sarcoma (KS), all share the same histologic features, which are demonstrated well in this case. (a) and (b) Just below the nasal mucosa, the mass is readily apparent, demonstrating ill-defined fascicles of monomorphic spindle cells, with slit-like vascular spaces, and abundant extravasated red blood cells. (c) Hyaline globules, a frequent finding in KS, are easily identified. (d) Immunostaining for HHV-8 supports the morphologic impression of KS.

Discussion

In HIV-positive individuals with CD4 counts less than 200 cells/mm, AIDS-related KS is the second most common tumor. ² Since the introduction of ART, there has been a decline in the incidence and severity of AIDS-related KS. ³ However, there have been reports of atypical KS occurring in well-controlled HIV patients with normal CD4 count as in this patient. ⁴

KS can present as solidary or diffuse pink to violaceous patches, plaques, or nodules on mucocutaneous surfaces. Visceral involvement may also occur, most often in the gastrointestinal and respiratory tracts. Primary nasal KS is extremely rare and there are only a few cases reported in literature. ⁵

A major KS cytokine, oncostatin M, has been shown to contribute to the pathogenesis and proliferation of AIDS-related KS cells. ⁶ Simultaneous exposure to corticosteroid and oncostatin M generated a substantial synergistic effect on the proliferation of AIDS-related KS cells, suggesting an interaction between corticosteroid and growth factor intracellular pathways in KS cells. ⁷ As such, both topical ⁸ and systematic ¹ corticosteroid therapy have been associated with the development of KS. However, this may be the first report of KS arising from intranasal corticosteroid administration. In our case, we postulate that the inhaled steroid led to localized immunosuppression within the nasal mucosa, allowing for the development of KS. Therefore, KS should be considered in the differential diagnosis in HIV-positive patients presenting with nasal polyps while on intranasal steroids, despite undetectable viral load and normal CD4 count.