Classic Kaposi sarcoma: Clinical features, staging, diagnosis, and treatment
- Susan E Krown, MD
Susan E Krown, MD
- Member Emerita
- Memorial Sloan-Kettering Cancer Center
- Vice-Chair for International Activities
- The AIDS Malignancy Consortium
- Jasmeet Chadha Singh, MD
Jasmeet Chadha Singh, MD
- Attending Physician
- Memorial Sloan Kettering Cancer Center
- Commack, NY
- Section Editors
- Robert Maki, MD, PhD
Robert Maki, MD, PhD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Medicine
- Monter Cancer Center
- Hofstra-Northwell School of Medicine
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
- June K Robinson, MD
June K Robinson, MD
- Section Editor — Nonmelanoma Skin Cancer
- Professor of Clinical Dermatology
- Northwestern University Feinberg School of Medicine
- Raphael E Pollock, MD
Raphael E Pollock, MD
- Editor-in-Chief — General Surgery
- Section Editor — Soft Tissue Sarcoma
- Professor of Surgery
- The Ohio State University
Kaposi sarcoma (KS) is an angioproliferative disorder that requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated Herpes virus (KSHV), for its development [1-3]. KS is classified into four types based upon the clinical circumstances in which it develops: classic (the type originally described by Kaposi, which typically presents in middle or old age), endemic (several forms described in sub-Saharan indigenous Africans prior to the AIDS epidemic), iatrogenic (a type associated with immunosuppressive drug therapy, typically seen in renal allograft recipients) and AIDS-associated (epidemic KS). The epidemiologic and clinical aspects of these four types of KS are compared in the table (table 1).
This review will focus on the clinical presentation, staging, diagnosis, and treatment of classic KS (CKS). The epidemiology, risk factors, pathology, and molecular pathogenesis of CKS are discussed elsewhere, as are AIDS-related KS and iatrogenic KS developing in the setting of immunosuppressive therapy. (See "Classic Kaposi sarcoma: Epidemiology, risk factors, pathology, and molecular pathogenesis" and "AIDS-related Kaposi sarcoma: Clinical manifestations and diagnosis" and "AIDS-related Kaposi sarcoma: Staging and treatment" and "Development of malignancy following solid organ transplantation", section on 'Kaposi sarcoma'.)
Classic Kaposi sarcoma (CKS) occurs most often in older men of Mediterranean or Central/Eastern European ancestry, in whom the lesions usually occur on the distal extremities, particularly the lower legs and feet. (See "Classic Kaposi sarcoma: Epidemiology, risk factors, pathology, and molecular pathogenesis".)
Skin lesions — CKS is characterized by the appearance of purplish, reddish blue, or dark brown/black macules, plaques, and nodules on the skin (picture 1A-C). Nodular lesions may ulcerate and bleed easily. The skin lesions range in size from very small to several centimeters in diameter, and they can remain unchanged for months to years, or grow rapidly within a few weeks and disseminate. (See 'Natural history' below.)
The dermatology literature contains reference to at least 10 different morphologic variants of the cutaneous lesions of KS, which are referred to as patch, plaque, nodular, lymphadenopathic (usually in African children), exophytic, infiltrative (the latter two in African adults with endemic KS), ecchymotic, telangiectatic, keloidal, and cavernous or lymphangioma-like variants . The cavernous- or lymphangioma-like variants are common in CKS, particularly in the setting of chronic lymphedema. In this variant, lesions develop on the lower extremities that consist of compressible nodules that appear to be fluid-filled cysts. Histologically, lymphangioma-like KS consists of anastomosing networks of smaller, irregular and compressible dilated lymphatics lined by flat and cytologically banal endothelial cells [5-7].
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- CLINICAL FEATURES
- Skin lesions
- Extracutaneous involvement
- Natural history
- DIAGNOSIS AND STAGING
- Differential diagnosis
- Radiographic evaluation
- Local treatments
- - Surgery
- - Radiation therapy
- - Cryotherapy and laser therapy
- - Intralesional therapy
- - Topical therapy
- Systemic treatments
- - Chemotherapy
- - Immunomodulators
- Investigational therapies
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS