Kaposi's Sarcoma

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Clinical Description

Kaposi sarcoma (KS) is an indolent angio-proliferative spindle-cell tumour derived from endothelial and immune cells infected with human herpes virus type 8 (HHV-8); also known as Kaposi sarcoma herpes virus (KSHV)). KS is currently the most common cancer in Malawi.

Clinical Features 

SIGNS AND SYMPTOMS

  • KS presents as mucocutaneous and visceral lesions.
  • Mucocutaneous lesions usually manifest as dark, red, purple, or brown papules, plaques, nodules, cauliflower lesions, with or without edema on the skin or mucous membranes. The lesions may ulcerate and form a whitish creamy layer of necrotic tissue.
  • Visceral disease occurs in organs such as lungs or GIT and may cause effusions in serous body cavities.
  • KS can co-exist with other Human Herpes Virus 8 associated conditions such as Castleman’s disease and Primary Effusion Lymphomas.

INVESTIGATIONS

  • HIV test if unknown. If positive and on treatment, do CD4, Viral Load to rule out HAART failure.
  • CXR to rule out lung involvement, Gastroscopy and Colonoscopy if GIT related symptoms.
  • Clinical diagnosis in HIV positive patients only without histology.
  • Do a biopsy for histology if clinical picture not typical of KS at presentation or in HIV negative patients.
  • HHV8 immunohistochemistry may be a useful addition

Treatment 

  • Treatment depends on the extent of disease; early stage can be controlled with ART alone.
  • All patients with HIV associated KS should receive ART.
  • Visceral and T1 disease should be considered for rapid initiation of treatment.
  • Local therapies: intralesional treatment, radiation and surgery are local therapies applied to KS lesions in general. Radiation therapy is reserved for disease that is limited but causing severe pain, bleeding, distress or is chemo-refractory. Surgery is reserved for aggressive local KS which is causing severe disfigurement, organ malfunction and overwhelming sepsis. In this case organ amputation is necessary, followed by systemic chemotherapy.
  • Systemic therapy: several cytotoxic chemotherapy agents are effective in providing rapid improvement in the majority of patients with locally aggressive and disseminated KS.

PHARMACOLOGICAL THERAPY 

First line treatment:  

  • Paclitaxel 100 mg/m2 2 weekly IV 6 - 8 cycles.
  • Always premedicate with Dexamethasone 8-16 mg IV/PO, Cimetidine 400 mg PO, Ondansetron 8 mg IV/PO and Promethazine5 mg IV 30 minutes before to reduce the risk of hypersensitivity reactions.
  • If not effective after 4 - 6 cycles, refer to a specialist for further evaluation.

Note: The initial assessment and first line therapy should be done at a health care facility with competent staff in handling cytotoxic drugs and safe drug handling equipment/tools to protect staff have been introduced. The FBC machine should have proper QC/QA to ensure correct counts are used.