Demographic profile, management, and survival of primary Gastrointestinal Kaposi Sarcoma: A USA Nationwide SEER-based study

1

Background

Primary Kaposi Sarcoma (KS) is a low-grade Angio proliferative disorder caused by human herpes virus-8 (HHV-8) infection in patients with a weakened immune system [1] . It is considered an AIDS-defining illness with the highest prevalence reported around the AIDS epidemic and a significant decrease in that population since the advent of combined antiretroviral therapy (cART). ¹ Mucosal and visceral lesions are uncommon (15–20%)in AIDS-related KS. They are rarely seen in sporadic KS [1] . Also, KS can also occur in HIV-negative individuals [2] . KS primarily involves the skin but can have multisystem manifestations. Primary KS involvement of the gastrointestinal (GI) tract is uncommon [34] . In 1872, of the five elderly male patients whom Dr. Moritz Kohn Kaposi (a Hungarian Dermatologist) first described KS, most had characteristic pigmented multiple skin lesions, while one had lesions in the gastrointestinal tract that had presented with gastrointestinal bleeding. A 10-year single-center review involving 27 patients with GI KS reported 81% of patients as men, with sexual transmission as the most common infection with HHV-8 (81%) in patients with primary GI-KS. In addition, 80% of patients had concomitant cutaneous KS [5] . Over 44% of patients with GI KS have CD-4 counts < 100/μL, and 90% with CD-4 counts < 200 μL [56] . Hence, it is established that GI (luminal and visceral) involvement of the KS is seen more frequently in AIDS patients with a low CD4 count. Also, GI KS can be seen in the absence of cutaneous KS lesions [67] .

The KS involvement of the GI tract is asymptomatic in over 80% of patients, and its occurrence is incidental [58] . However, symptoms like nausea, vomiting, perforation, GI Bleeding, obstruction, and intussusception have been reported [59] . Screening endoscopies though not performed in the USA, are done in other countries, like Japan. Diagnosing GI KS early will lead to lower morbidity and mortality and tailored appropriate early management. GI KS is considered to be underdiagnosed. Also, it is essential to note that though classic GI KS lesions could be endoscopically identified, the biopsy can still miss the histological diagnosis of KS due to the sub-mucosal lesions. The treatment usually consists of cART along with any local management [(RT-radiation therapy) or (CT-chemotherapy)]. Using a national database, our review aims to ascertain the demographic profile and survival related to primary GI KS patients. Our sub-analysis involved looking into survival based on the primary site of the KS and any intervention that was sort for and its respective survival outcomes, along with the best intervention outcomes based on site-specific GI KS.