AtlasMalignantKaposi Sarcoma

Malignant Tumors

Kaposi Sarcoma

aka KS

Low-grade vascular malignancy caused by HHV-8 (KSHV); oral involvement is a defining lesion of AIDS, typically as purple palatal macules or nodules.

Virus
HHV-8
Site
Hard palate
Rx
ART + local therapy

§ overviewOverview

HHV-8-driven mesenchymal malignancy of endothelial origin producing multifocal vascular lesions of skin and mucosa.

§ icdICD Classification

C46

§ etiologyEtiology

  • 01HHV-8 (KSHV) infection
  • 02Cofactor: immunosuppression (HIV, transplant)

§ epidemiologyEpidemiology

AIDS-KS: >20 000× risk vs general population before ART; commonest HIV-associated malignancy.

§ clinicalClinical Features

  • 01Flat red-purple macule → plaque → nodule
  • 02Hard palate most common oral site, then gingiva, tongue
  • 03Multifocal, painless initially → ulceration and bleeding
  • 04Cutaneous, GI, pulmonary involvement in advanced disease

§ differentialDifferential Diagnosis

  • 01Haemangioma
  • 02Pyogenic granuloma
  • 03Melanoma
  • 04Bacillary angiomatosis
  • 05Purpura

§ histopathHistopathology

  • 01Proliferating spindle cells forming slit-like vascular spaces with extravasated RBCs
  • 02Hyaline globules
  • 03Plasma cell infiltrate

§ investigationsInvestigations

  • 01Biopsy with HHV-8 IHC
  • 02HIV testing and CD4 count
  • 03CT chest/abdomen for staging
  • 04Endoscopy if GI symptoms

§ ihcIHC / Special Stains

  • 01HHV-8 LANA-1 positive (diagnostic)
  • 02CD31, CD34, D2-40 positive

§ classificationClassification

  • 01Classic (elderly Mediterranean)
  • 02Endemic (African)
  • 03Iatrogenic (post-transplant)
  • 04AIDS-associated (epidemic)

§ treatmentTreatment

  • 01Optimise ART (mainstay for AIDS-KS)
  • 02Local: intralesional vinblastine, radiotherapy, cryotherapy, sclerotherapy
  • 03Systemic: liposomal doxorubicin, paclitaxel for advanced disease
  • 04Reduce immunosuppression in transplant-related KS

§ complicationsComplications

  • 01Airway obstruction (laryngeal)
  • 02Bleeding, disfigurement
  • 03Systemic dissemination

§ prognosisPrognosis

AIDS-KS: dramatically improved with ART. Classic KS: indolent, elderly-appropriate care.

§ examKey Examination Points

  • 01Purple palatal lesion → biopsy and HIV test
  • 02Assess extent (skin, GI, pulmonary)

§ revisionQuick Revision Summary

  • 01HHV-8 · palate · LANA-1+ · ART + local/systemic therapy

§ vivaBDS Viva Questions

  • 01Aetiological agent of KS?
  • 02Four clinical variants?
  • 03Treatment of AIDS-KS?

§ mcqsMCQs — Assessment (3)

Question 1

Causative virus:

Question 2

Most common oral site:

Question 3

Diagnostic IHC marker:

References

  1. Cesarman E. Nat Rev Dis Primers 2019

Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.