mTOR, VEGF, PDGFR, and c-kit signaling pathway activation in Kaposi sarcoma

Hum Pathol. 2017 Jul:65:157-165. doi: 10.1016/j.humpath.2017.05.002. Epub 2017 May 12.

Abstract

Kaposi sarcoma (KS) is a locally progressive, intermediate-grade vascular neoplasm with no known cure, high recurrence rates, and potential for wide dissemination. Low efficacy and high toxicity limit current therapeutic options for advanced disease. Activation of mammalian target of rapamycin (mTOR), platelet-derived growth factor (PDGF), vascular endothelial growth factor (VEGF), and c-kit signaling pathways has been implicated in KS pathogenesis and may suggest a role for targeted inhibitors. KS cases were retrospectively retrieved (N=274), most (90%) associated with human immunodeficiency virus. Tissue microarray slides were stained with human herpes virus-8, Friend leukemia integration 1 transcription factor, CD117 (c-kit), phospho-S6 (pS6), PDGF receptor-β, VEGF, and phospho-mTOR. Both intensity and extent of staining were scored. Multiplying these scores for each core yielded total staining H-scores. Human herpes virus-8 was positive in 87% and Friend leukemia integration 1 transcription factor in 95.7% of cases. Most were also VEGF+ (97.6%), pS6+ (95.7%), CD117+ (92.5%), and PDGFRB+ (87.4%). Approximately half (55.6%) were phospho-mTOR+. There was no significant difference in staining among patients with low (<500 cells/mm3) or preserved CD4 T-cell counts. Immunohistochemistry confirms upregulation of the mTOR, PDGF, VEGF, and c-kit pathways in a large cohort of KS samples. Of proteins tested, pS6, downstream of mTOR, demonstrated the highest proportion of strong positivity (67.1%). These results support the possibility of using targeted inhibitors in KS. Overexpression was independent of CD4 count, suggesting that even patients with low counts may be targeted therapy candidates.

Keywords: C-kit; Immunohistochemistry; Kaposi sarcoma; PDGFR; VEGF; mTOR.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / therapeutic use
  • Biomarkers, Tumor / analysis*
  • CD4 Lymphocyte Count
  • Child
  • Child, Preschool
  • Female
  • HIV Infections / immunology
  • HIV Infections / virology
  • Herpesvirus 8, Human / isolation & purification
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Molecular Targeted Therapy
  • Phosphorylation
  • Predictive Value of Tests
  • Proto-Oncogene Proteins c-kit / analysis*
  • Proto-Oncogene Proteins c-kit / antagonists & inhibitors
  • Receptors, Platelet-Derived Growth Factor / analysis*
  • Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors
  • Retrospective Studies
  • Ribosomal Protein S6 / analysis
  • Sarcoma, Kaposi / chemistry*
  • Sarcoma, Kaposi / drug therapy
  • Sarcoma, Kaposi / pathology
  • Sarcoma, Kaposi / virology
  • Signal Transduction* / drug effects
  • TOR Serine-Threonine Kinases / analysis*
  • TOR Serine-Threonine Kinases / antagonists & inhibitors
  • Tissue Array Analysis
  • Vascular Endothelial Growth Factor A / analysis*
  • Vascular Endothelial Growth Factor A / antagonists & inhibitors
  • Young Adult

Substances

  • Antineoplastic Agents
  • Biomarkers, Tumor
  • Ribosomal Protein S6
  • VEGFA protein, human
  • Vascular Endothelial Growth Factor A
  • MTOR protein, human
  • Proto-Oncogene Proteins c-kit
  • Receptors, Platelet-Derived Growth Factor
  • TOR Serine-Threonine Kinases