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September 15, 2008

Cutaneous Tumors, Massive Lymphadenopathy, and Secondary Lymphedema in a 16-Year-Old Boy—Diagnosis

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Arch Dermatol. 2008;144(9):1217-1222. doi:10.1001/archderm.144.9.1218-d

Hematoxylin-eosin staining of the biopsy specimen showed dilated irregular vascular channels surrounding preexisting vessels. The promontory sign and characteristic eosinophilic globules were evident. Also, the stroma was infiltrated with lymphocytes and plasma cells and showed multiple slitlike spaces with extravasated red blood cells.

Kaposi sarcoma is a vascular proliferation that was first reported in 1872.1 It is classified into 4 clinical variants: (1) classic, (2) African/endemic, (3) AIDS-associated/epidemic, and (4) immunosuppression-associated/transplant-associated/acquired/iatrogenic. African/endemic KS was first described in the 1950s2 and is now the most common tumor in human immunodeficiency virus (HIV)-negative and HIV-positive individuals in Central Africa.3 African/endemic KS is predominant in men (male to female ratio, approximately 3:1). The peak age at onset ranges from 35 to 39 years in males and 25 to 39 years in females. Clinically, there are 4 types of African/endemic KS: (1) nodular, (2) florid, (3) infiltrative, and (4) lymphadenopathic. The lymphadenopathic type runs a fulminant course in the pediatric population and can aggressively progress to fatality within 1 year of onset. It has a peak rate of incidence in children younger than 10 years.4 In Southern and Eastern Africa, KS is reported to account for 2% to 10% of all cancers in the pediatric population, and it is responsible for 25% to 50% of all soft tissue sarcomas in these children.5 The lymphadenopathic form clinically presents with extreme lymphadenopathy of the inguinal and/or axillary regions.6

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