Guidelines

Subtypes Kaposi´s Sarcoma (KS)

1. Classic KS
   

2. Endemic KS
   

3. Epidemic KS in HIV positive patients
   

4. Iatrogenic KS

Staging workup

CT, computerised tomography; KS, Kaposi’s sarcoma; HIV, human immunodeficiency virus; US, ultrasound; GI, endoscopy; HHV-8, human herpesvirus-8.
- : usually not  +: usually yes   ++: mandatory    +/- : according to symptoms

Treatment

1. Radiotherapy is most efficient treatment for localised KS.  30-36 Gy in 2- or 3-Gy daily fractions using low-energy photons and/or electrons. Information about possible risks of out-of-field recurrence and of radiotherapy-induced skin toxicity like telangiectasia, hyperpigmentation, skin atrophy and fibrosis.
   

2. Surgical Excision in few well-defined lesions. Caution: high recurrence rate and repeated excisions can cause functional impairment. 
   

3. Cryosurgery and laser in superficial lesions, response rate 80-90%, information about risk of sequelar hypopigmentation to patients.
   

4. Topical treatments/ local or intralesional chemical or immunemodifying agents:

   Intralesional Vincristin or Vinblastin

   Electrochemotherapy with Bleomycin

   Imiquimod

   Topical 9-cis-retinoid acid (Alitretinoin gel 0.1%) – currently in EU not available
   

5. Medical treatment in aggressive forms characterised by lymph node and/or visceral involvement, severe oedema, local complications or rapid extension: liposomal anthracyclines and, less frequently, weekly taxanes. Or low dose interferon alpha in younger, healthy patients (<70 years old and normal cardiac function).  
   

6. Iatrogenic KS: Tapering down immunosuppressive therapy to the lowest possible level and switching to mammalian target of rapamycin (m-TOR) inhibitors such as sirolimus.
   

7. HIV-related KS regresses with combination antiretroviral therapy. Chemotherapy is recommended for patients with poor prognosis or rapidly progressive disease.

8.  Follow Up

Further information

Lebbe C. et al. Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC) EJC 2019 DOI: 10.1016/j.ejca.2018.12.036

1. Surgical treatment/safety margin (SM)

Dermal LMS: Primarily microscopically controlled R0, extension of the SM to 1 cm

Subcutaneous LMS: Primarily microscopically controlled R0, if possible extension of the SM to 2 cm, or rather to the fascia (or according to an interdisciplinary tumor conference)

2. Diagnostics (imaging)

None in standard cases.

In case of suspicion or evidence of locoregional metastasis: ultrasound of regional lymph nodes

In the case of non-displaceable tumours/ suspected deep infiltration: locoregional CT or MRI

3. Adjuvant radiation therapy

LMS R0 and small tumours: none

LMS R1 or R-2 resection, small safety margin or large tumours

(LMS > 5cm): recommended

1. Radiotherapy:

In palliative, inoperable situations: therapeutic radiotherapy

2. Medical treatment:

Individual therapy decision

E.g. doxorubicin or anthracycline-based combination therapy (in case of rapid progression)

6. Follow-up care:

7. further information:

Reference: Helbig et al. S1-guideline cutaneous and subcutaneous leiomyosarcoma https://doi.org/10.1111/ddg.14989