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AIDS-related Kaposi sarcoma of the oral cavity

A 25-year-old man with recently diagnosed acquired immunodeficiency syndrome (AIDS) was referred for consultation because of multiple intraoral lesions that had developed within past 2 months. Physical examination revealed diffuse multinodular purplish-red lesions with spontaneous bleeding affecting both the maxillary and mandibular gingiva (Figure 1). Histopathology of an incisional biopsy revealed a fascicular arrangement of spindle cells with congested blood vessels and hemorrhage in a telangiectatic pattern (Figure 2A). Immunohistochemistry for human herpesvirus 8 (HHV-8) was diffusely positive (Figure 2B), confirming the diagnosis of Kaposi sarcoma (KS). Complete remission of the oral lesions was achieved after 16 months of antiretroviral therapy (ART) and liposomal doxorubicin chemotherapy. No signs of relapse were observed at 1 year post-treatment follow-up (Figure 3).

FIGURE 1:
Initial clinical presentation showing multinodular lesions of the maxillary and mandibular gingiva.

FIGURE 2:
Histopathology of an incisional biopsy of an oral lesion. A, Photomicrography showing a fascicular arrangement of spindle cells with many congested blood vessels and hemorrhage in a telangiectatic pattern (hematoxylin and eosin stain; original magnification ×100) B, Higher magnification (hematoxylin and eosin stain; original magnification ×400). C, Photomicrography showing diffuse nuclear immunoreactivity for human herpesvirus 8 (HHV-8) in neoplastic spindle cells (DAB stain; original magnification ×100). D, Higher magnification of nuclear immunoreactivity for HHV-8 (DAB stain; original magnification ×400).

FIGURE 3:
The gingiva 1 year after completing treatment.

KS is a multifocal angioproliferative disorder associated with HHV-811. Bunn BK, Carvalho MV, Louw M, Vargas PA, van Heerden WF. Microscopic diversity in oral Kaposi sarcoma. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;115(2):241-8. Available from: https://doi.org/10.1016/j.oooo.2012.11.009
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. AIDS-related KS, the most common and aggressive variant, is the most frequent malignancy affecting people living with HIV22. Guedes PTL, Pontes FSC, Prado-Ribeiro AC, Rodrigues-Oliveira L, Brandão TB, Souza LL, et al. HIV-positive patients with oral Kaposi's sarcoma: An overall survival analysis of 31 patients. Oral Surg Oral Med Oral Pathol Oral Radiol . 2021;131(6):702-10. Available from: https://doi.org/10.1016/j.oooo.2020.11.007
https://doi.org/10.1016/j.oooo.2020.11.0...
, in whom it often presents with mucocutaneous and/or visceral involvement. The oral cavity is affected in up to 70% of cases, and oral KS is sometimes the first manifestation of undiagnosed HIV infection11. Bunn BK, Carvalho MV, Louw M, Vargas PA, van Heerden WF. Microscopic diversity in oral Kaposi sarcoma. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;115(2):241-8. Available from: https://doi.org/10.1016/j.oooo.2012.11.009
https://doi.org/10.1016/j.oooo.2012.11.0...
. According to the AIDS Clinical Trials Group, extensive oral involvement is a sign of advanced KS that worsens survival33. Freeman EE, McCann NC, Semeere A, Reddy KP, Laker-Oketta M, Byakwaga H, et al. Evaluation of four chemotherapy regimens for treatment of advanced AIDS-associated Kaposi sarcoma in Kenya: a cost-effectiveness analysis. Lancet Glob Health. 2022;10(8):e1179-e1188. Available from: https://doi.org/10.1016/S2214-109X(22)00242-X
https://doi.org/10.1016/S2214-109X(22)00...
. Although ART plays an important role in KS prevention and improves survival, factors such as delayed diagnosis, suboptimal linkage to care, and limited access to effective chemotherapy regimens hinder the management of advanced KS, especially in low- and middle-income countries22. Guedes PTL, Pontes FSC, Prado-Ribeiro AC, Rodrigues-Oliveira L, Brandão TB, Souza LL, et al. HIV-positive patients with oral Kaposi's sarcoma: An overall survival analysis of 31 patients. Oral Surg Oral Med Oral Pathol Oral Radiol . 2021;131(6):702-10. Available from: https://doi.org/10.1016/j.oooo.2020.11.007
https://doi.org/10.1016/j.oooo.2020.11.0...
,33. Freeman EE, McCann NC, Semeere A, Reddy KP, Laker-Oketta M, Byakwaga H, et al. Evaluation of four chemotherapy regimens for treatment of advanced AIDS-associated Kaposi sarcoma in Kenya: a cost-effectiveness analysis. Lancet Glob Health. 2022;10(8):e1179-e1188. Available from: https://doi.org/10.1016/S2214-109X(22)00242-X
https://doi.org/10.1016/S2214-109X(22)00...
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ETHICS

All procedures involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

REFERENCES

  • 1
    Bunn BK, Carvalho MV, Louw M, Vargas PA, van Heerden WF. Microscopic diversity in oral Kaposi sarcoma. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;115(2):241-8. Available from: https://doi.org/10.1016/j.oooo.2012.11.009
    » https://doi.org/10.1016/j.oooo.2012.11.009
  • 2
    Guedes PTL, Pontes FSC, Prado-Ribeiro AC, Rodrigues-Oliveira L, Brandão TB, Souza LL, et al. HIV-positive patients with oral Kaposi's sarcoma: An overall survival analysis of 31 patients. Oral Surg Oral Med Oral Pathol Oral Radiol . 2021;131(6):702-10. Available from: https://doi.org/10.1016/j.oooo.2020.11.007
    » https://doi.org/10.1016/j.oooo.2020.11.007
  • 3
    Freeman EE, McCann NC, Semeere A, Reddy KP, Laker-Oketta M, Byakwaga H, et al. Evaluation of four chemotherapy regimens for treatment of advanced AIDS-associated Kaposi sarcoma in Kenya: a cost-effectiveness analysis. Lancet Glob Health. 2022;10(8):e1179-e1188. Available from: https://doi.org/10.1016/S2214-109X(22)00242-X
    » https://doi.org/10.1016/S2214-109X(22)00242-X
  • Financial Support: None.

Publication Dates

  • Publication in this collection
    02 June 2023
  • Date of issue
    2023

History

  • Received
    31 Mar 2023
  • Accepted
    12 Apr 2023
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