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NCCN Flash Updates: NCCN Guidelines Updated for Kaposi Sarcoma

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines^®), the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™) for Kaposi Sarcoma. These NCCN Guidelines^® are currently available as Version 1.2025.

Link directly to the Updates section of the NCCN Guidelines: Kaposi Sarcoma

Global

• References were updated throughout the Guidelines.
• "AIDS" has been replaced by "HIV" where appropriate.

KS-1

• Diagnosis
  • Essential, bullet 1 revised: Review of all adequate slides with at least one from paraffin block representative of the tumor by a pathologist with expertise in the diagnosis of Kaposi sarcoma.
• Workup
  • Essential
    • Bullet 1, History and physical examination
      • Sub-bullet 1 revised: Including history of additional immunosuppression immunosuppressive disease or therapy, such as transplant/local or systemic glucocorticoids.
      • Sub-bullet 2 revised: Including complete skin, oral, and lymph node examinations, and evaluation documentationof edema.
  • Essential Useful in selected cases
    • Bullet 1 moved from Essential: In patients of childbearing potential if chemotherapy or radiation therapy (RT) planned: pregnancy testing.
    • Bullet 9 added: For PWH, begin discussions regarding the possible need to modify ART due to DDIs and the need to involve an HIV specialist in care decisions. See Principles of Systemic Therapy and Drug-Drug Interactions (HIV-B) in the NCCN Guidelines for Patients with HIV.
• Footnote a, second sentence modified: Involvement of an infectious disease (ID) HIV specialist to evaluate for coexisting OI is appropriate, especially with advanced immunosuppression.

KS-2

• First line therapy if symptomatic and/or cosmetically bothersome, options revised: Topicals or Intralesional chemotherapy or Local therapy or RT Local exicision or Cryotherapy or Systemic therapy or Clinical trial.
• Footnote h revised: All PWH who have limited cutaneous disease that is symptomatic (eg, edema, oral KS, or other manifestation that interfere with normal functions or activities) and/or cosmetically bothersome should receive ART with or without another first-line therapy. Initiation of ART may result in immune reconstitution inflammatory syndrome (IRIS) within 3–6 months; IRIS is characterized by marked lesional swelling, increased tenderness, and peripheral edema. Therefore, systemic KS-directed therapy should be initiated as soon as possible in all PWH who have symptomatic KS (eg, edema, oral KS, or other manifestation that interfere with normal functions or activities). In patients with limited cutaneous disease, KS-associated IRIS is an indication for urgent initiation of systemic KS therapy; signs of past IRIS may also be an indication for earlier initiation of systemic KS therapy. ART should not be delayed or discontinued unless life-threatening IRIS develops. Reconstitution of immune function is important for obtaining and maintaining control or remission of KS. See Principles of Immune Reconstitution Inflammatory Syndrome (IRIS) (KS-C) (Also for KS-3)
• Footnote p, second sentence revised: If KS, evaluate for inadequate HIV control/ART failure as a contributing factor to inadequate KS control and address possible change in ART in conjunction with an HIV specialist. (Also for KS-3)

KS-3

• Advanced cutaneous, oral, visceral, or nodal disease, bottom pathway revised: not immediately eligible for systemic therapy.
  • First-line therapy, bottom pathway revised: Medical optimization and Consider RT and Continue or initiate ART for PWH.
  • Not eligible for systemic therapy, pathway revised: Consider RT or Best supportive care.
  • Footnote r modified: Systemic therapy is preferred over RT as first-line therapy and relapsed/refractory therapy for disseminated disease whenever systemic therapy is feasible. Although uncommon, considering performance status and medical comorbidities may preclude the use of upfront chemotherapy in some patients. Reversible medical issues should be addressed (eg, active infection requiring antibiotic or antiviral treatment; high-level HIV viremia requiring initiation/modification of ART) so that systemic therapy can be delivered as quickly as possible. If poor performance status is because of KS, systemic therapy should be considered because it may lead to an improvement in performance status.

KS-4

• Surveillance
  • Bullet 1, quaternary bullet 2 revised: Including complete skin and oral examinations, and documentation evaluation and monitoring of edema
  • Bullet 2 modified: Photography of oral, conjunctival, and cutaneous lesions (with reference unit of measure in the picture) for documentation evaluation of extent of disease if change in disease is noted.

KS-D

• Local Therapy
  • Topical, bullet 1, sub-bullet revised: Apply 3–4 2 times daily to affected skin sites; increase to 3–4 times daily if tolerated.
  • Cryotherapy, bullet added: Cryotherapy should be done by clinicians with expertise in cutaneous cancer cryotherapy.
  • Last header revised: For local control of small, symptomatic lesions (ie, ≤1 cm), the following may be considered: for local control of symptomatic lesions
    • Bullet 1 revised: Marginal excision
    • Bullet 2 added: Electrodesiccation and curettage
    • Bullet 3, sub-bullet revised and moved above intralesional chemotherapy options: Treatment schemas are listed below as a guide. Other treatment schemas have been studied, with a variety of vinblastine chemotherapy concentrations, doses, administration volumes, frequencies of administration, and total doses/volumes administered. See Discussion for additional references and information.
    • Bullet 3, sub-bullet 2 added: Bleomycin as an option for intralesional chemotherapy
    • Bullet 3, sub-bullet 2, sub-bullet added: 1.5 to 3 units/mL solution with a dose of 0.5 unit per 0.5 cm² of lesion (volume given depends on the size of the lesion) every 3 to 4 weeks until response
    • Footnote a added: For larger lesions when systemic therapy is not feasible or effective, radiotherapy is preferred over excision.
• Footnote b added: Wide excision is not indicated.
• Reference 4 added: Poignonec S, Lachiver LD, Lamas G, et al. Intralesional bleomycin for acquired immunodeficiency syndrome-associated cutaneous Kaposi's sarcoma. Arch Dermatol 1995;131:228.

KS-E

• Principles of Radiation Therapy
  • General Treatment Information
  • Tertiary bullet revised: Various dosing schemas may be used. Lower total doses are preferred for smaller and more superficial lesions and palliative situations. Higher doses may be preferred for more extensive, deeply invasive lesions, or when a more durable local response is desired.
• Footnote a added: BED is a measure of the radiosensitivity of the tissue, with BED10 denoting the estimated effective dose to rapidly growing tumor tissue and BED3 denoting the estimated dose to normal tissue (organs at risk).

• Table: row 1, column 2 revised: 10.8 9.6–14.4 Gy

KS-F (1 of 4)

• Systemic Therapy
  • Subsequent systemic therapy options revised: (also for table on KS-F [2 of 4])
    • Headers removed:
      • Preferred Regimen
      • Other Recommended Regimens
      • Preference stratification for subsequent therapy options was changed.
    • Useful in certain circumstances, regimens revised:
    • Nivolumab ± ipilimumab + nivolumab (for classic KS)
    • Pembrolizumab (for endemic and classic KS)
• Footnote g, second sentence added: If the patient has a history of KSHV-associated diseases, ICIs should be used with caution and with consideration of more frequent monitoring for signs and symptoms of KICS or MCD. See NCCN Guidelines for Cancer in People with HIV, Principles of Systemic Therapy and Drug-Drug Interactions. (Also for KS-F [3 of 4])
• Footnote removed: Pomalidomide has been U.S. Food and Drug Administration (FDA) approved for the treatment of adult patients with AIDS-related KS after failure of highly active ART or in patients with KS with no HIV.

KS-F (2 of 4)

• Useful in certain circumstances, regimen and dosing schedule added: Nivolumab, 480 mg IV every 4 weeks.

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Biomarkers Compendium^®, the NCCN Chemotherapy Order Templates (NCCN Templates^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

To view the NCCN Guidelines for Patients^®, please visit NCCN.org/patientguidelines.

Free NCCN Guidelines apps for iPhone, iPad, and Android devices are now available! Visit NCCN.org/apps.

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