Kaposiʼs Sarcoma in the Pediatric Population: The Critical Need for a Tissue Diagnosis

Kaposiʼs sarcoma (KS) is a low-grade vascular neoplasm mediated by the human herpesvirus-8. Only 1 clinical subtype, the endemic/African subtype, commonly affects the pediatric population. Although adults with KS often present with cutaneous findings and generalized lymphadenopathy, African children are more likely to present without classic skin findings. Definitive diagnosis requires histologic examination from tissue biopsy; however, as pathology resources are scarce in many developing African countries where KS is prominent, appropriate diagnosis and treatment of the condition are challenging. We report the case of a Malawian child who presented with generalized lymphadenopathy and was presumptively treated for lymphoma, with clinical worsening of his lesions. A diagnosis of KS was made after excisional biopsy of a superficial lymph node, with the initiation of appropriate therapy. The literature regarding pediatric KS is reviewed and recommendations are offered to allow accurate and timely diagnosis of the condition.