Disseminated Cutaneous and Gastrointestinal Kaposiʼs Sarcoma - A Remnant of the Past?

Abstract

Purpose: Kaposi's sarcoma (KS) is the most common tumor among AIDS patients and can involve multiple organs such as pulmonary, GI and skin. It is linked with human herpes virus type 8 infection and is considered to be an AIDS-defining illness. AIDS-related gastrointestinal KS is the most common visceral involvement in disseminated disease, being seen in up to 50% of patients. Involvement can occur even in the absence of cutaneous disease. The protective effect of HAART has also been shown to result in a decreased incidence of KS and HIV-associated cancers. Results: 54 y/o homeless male with history of HIV (CDC C3; absolute CD4 173 cells/cmm) for 20 years, antiviral naïve - presented with fevers, chills, generalized myalgias, blurry vision and left lower extremity swelling consistent with cellulitis. His exam was notable for having numerous purple pigmented nodules, several centimeters in diameter diffusely throughout his face, trunk, arms, legs and hard palate. Ophthalmology evaluated the ocular complaints and found a right inferior conjunctival mass. These lesions were highly suspicious for KS with systemic involvement. CT chest/abdomen was done showing a 2.9 cm hemangioma and a 2.5 cm x 2 cm anterior pelvic mass representing metastasis or enlarged lymph node. His hard palate lesion along with intermittent dysphagia was suspicious for GI involvement, which prompted an upper endoscopy. EGD revealed nodules at the GE junction, gastric antrum and duodenum which pathology of GE junction and duodenum confirmed to be KS. Biopsy of a cutaneous leftring finger nodule was also consistent with KS. He was started on Atripla® and Bactrim® and referred to an oncologist for Doxorubicin chemotherapy. Conclusion: AIDS-related KS can affect any level of the gastrointestinal tract from the oropharynx to the rectum. The most frequently affected site is the duodenum. Endoscopically, the lesions appear to be red-purple, hemorrhagic and nodular, which may be confluent or isolated. The differential diagnosis of these lesions typically include opportunistic infections, lymphoma, polyps, hematogenous metastases, and Crohn's disease. Endoscopic biopsy may be negative because of the submucosal location of the tumor. High grade lesions are more likely to be associated with invasion and dissemination. Early disease is frequently asymptomatic, but complications - such as bleeding, intestinal/biliary obstruction, intussusception, perforations, diarrhea, and protein-losing enteropathy - may occur as the lesions enlarge. Treatment with liposomal anthracycline systemic chemotherapy is usually used for patients with extensive cutaneous or symptomatic visceral involvement.