Objective: To report a case of a human immunodeficiency virus (HIV)-negative Kaposi sarcoma (KS) associated with Cushing disease (CD).
Methods: The details of case presentation, evaluation, diagnosis, and treatment are presented and cases of KS and CD published before November 1, 2010 on PubMed and Scopus are reviewed.
Results: A 54-year-old Hispanic HIV-negative man presented with typical signs and symptoms of CD (easy bruisability, proximal muscle wasting, and abdominal fat pads). Numerous raised, purplish, nonblanching plaques 0.5 to 2 cm in diameter extended throughout his lower extremities. Biochemical tests and pituitary magnetic resonance imaging confirmed CD. A lesion biopsy showed atypical vascular proliferation positive by immunohistochemistry for human herpesvirus 8 (HHV-8), consistent with KS. He underwent 2 transsphenoidal surgeries followed by a bilateral adrenalectomy. After recovery, his KS was treated with a systemic combination of liposomal doxorubicin and paclitaxel.
Conclusion: The occurrence of CD and KS is rare. Specific therapy for CD and chemotherapy for KS are effective in the treatment of KS associated with CD.