Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis

M R Openshaw; E Gervasi; C A M Fulgenzi; D J Pinato; A Dalla Pria; M Bower

doi:10.1186/s12967-023-04130-6

Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis

J Transl Med. 2023 Apr 27;21(1):283. doi: 10.1186/s12967-023-04130-6.

Authors

M R Openshaw^{1

2}, E Gervasi³, C A M Fulgenzi^{4

5}, D J Pinato^{4

6}, A Dalla Pria⁷, M Bower⁷

Affiliations

¹ Institute of Cancer and Genomics Sciences, University of Birmingham, Birmingham, UK. mropenshaw@doctors.org.uk.
² UK National Centre for HIV Oncology, Chelsea Westminster Hospital, London, UK. mropenshaw@doctors.org.uk.
³ Infectious Diseases Unit, Papa Giovanni XXIII Hospital, Bergamo, Italy.
⁴ Department of Surgery and Cancer, Faculty of Medicine, Hammersmith Hospital, Imperial College London, London, UK.
⁵ Department of Medical Oncology, University Campus Bio-Medico of Rome, Rome, Italy.
⁶ Division of Oncology, Department of Translational Medicine, University of Piemonte Orientale, Novara, Italy.
⁷ UK National Centre for HIV Oncology, Chelsea Westminster Hospital, London, UK.

Abstract

Background: The taxonomy of Kaposi Sarcoma (KS) is based on a classification system focused on the description of clinicopathological features of KS in geographically and clinically diverse populations. The classification includes classic, endemic, epidemic/HIV associated and iatrogenic KS, and KS in men who have sex with men (MSM). We assessed the medical relevance of the current classification of KS and sought clinically useful improvements in KS taxonomy.

Methods: We reviewed the demographic and clinicopathological features of 676 patients with KS, who were referred to the national centre for HIV oncology at Chelsea Westminster hospital between 2000 and 2021.

Results: Demographic differences between the different subtypes of KS exist as tautological findings of the current classification system. However, no definitive differences in clinicopathological, virological or immunological parameters at presentation could be demonstrated between the classic, endemic or MSM KS patients. Reclassifying patients as either immunosuppressed or non-immunosuppressed, showed that the immunosuppressed group had a significantly higher proportion of adverse disease features at presentation including visceral disease and extensive oral involvement, classified together as advanced disease (chi² P = 0.0012*) and disseminated skin involvement (chi² P < 0.0001*). Immunosuppressed patients had lower CD4 counts, higher CD8 counts and a trend towards higher HHV8 levels compared to non-immunosuppressed patients, however overall survival and disease specific (KS) survival was similar across groups.

Conclusion: The current system of KS classification does not reflect meaningful differences in clinicopathological presentation or disease pathogenesis. Reclassification of patients based on the presence or absence of immunosuppression is a more clinically meaningful system that may influence therapeutic approaches to KS.

Keywords: Acquired immune deficiency syndrome; Disease classification; Human herpes virus 8; Human immunodeficiency virus; Kaposi Sarcoma.

Publication types

Review

MeSH terms

CD4 Lymphocyte Count
HIV Infections* / complications
Homosexuality, Male
Humans
Male
Sarcoma, Kaposi* / epidemiology
Sarcoma, Kaposi* / etiology
Sarcoma, Kaposi* / pathology
Sexual and Gender Minorities*