Gastrointestinal Kaposi's Sarcoma Research Articles

S3114 Gastrointestinal Kaposi's Sarcoma Manifesting as Melena

S3114 Gastrointestinal Kaposi's Sarcoma Manifesting as Melena

The role of gastrointestinal endoscopy in Kaposi sarcoma.

We are writing to make endoscopists aware of the paramount of a prompt diagnosis of gastrointestinal Kaposi sarcoma (GI-KS). Patients with GI involvement have a two to five times higher risk of death and will benefit from chemotherapy to improve their survival. However, current evidence found that one out of three patients might have a false negative result even with HHV-8 since other entities such as gastrointestinal stromal tumors, angiosarcoma, and lymphoma shared macroscopic and histopathological characteristics. These cause a delay in treatment and significantly worsen the prognosis. We observed a trend for a positive diagnosis from ulcers and nodules. To our knowledge, this is the largest cohort of patients with GI-KS in the world. Our study suggests that in cases where a complete immunochemistry panel for KS is not available, HHV-8 remains as a bare minimum. However, other gastrointestinal lesions shared histopathological characteristics. Therefore, we suggest taking biopsies from nodular and ulcer-type lesions to increase the probability to establish a histopathological diagnosis.

Diffuse large B-cell lymphoma and gastrointestinal Kaposi's sarcoma: a rare cause of upper gastrointestinal bleeding.

A 49-year-old man with a history of human immunodeficiency virus (HIV) infection, in a state of advanced immunosuppression and current antiretroviral therapy initiation. He was admitted to the hematology department after diagnosis of diffuse large B-cell lymphoma (DLBCL) associated with Epstein-Barr virus (EBV), disseminated cytomegalovirus (CMV) infection and cutaneous Kaposi's sarcoma (KS). During admission, he presented an episode of melena with anemization, so an urgent gastroscopy was performed. In the stomach there were multiple erythematous lesions, large and elevated, suggestive of subepithelial origin. Some of them presented an umbilicated center and other were ulcerated, covered with fibrin. The bulb and second duodenal portion showed similar involvement, large violaceous ulcerated lesions. Biopsies were taken and the anatomopathological diagnosis of the duodenal lesions was KS and of the gastric lesions KS and DLBCL associated with EBV. KS is an endothelial tumor associated with HIV. The stomach is the gastrointestinal organ most frequently affected and the typical endoscopic findings are nodular lesions. The stomach is the most common site of extranodal involvement in DLBCL. In both pathologies, the manifestation as upper gastrointestinal bleeding is exceptional and endoscopic biopsies can confirm the diagnosis.

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

Kaposi Sarcoma (KS) is a Human Herpes Virus-8 (HHV-8) associated angio-proliferative disorder commonly seen in patients with HIV. It most commonly involves the skin as classic purple lesions but occasionally involves the gastrointestinal (GI) tract. To date, published data is scarce on primary GI KS. Using a national database, this study analyzes the incidence, demographics, and survival of primary GI KS. We conducted a retrospective analysis (1975–2019) on biopsy-proven primary GI KS cases from 17 registries from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database. A total of 685 patients with GI KS were identified. Female gender, Non-Hispanic Asian or Pacific Islander (NHAPI), married marital status, and large bowel site-specific primary KS to have better overall survival. Luminal gastrointestinal KS was more frequent (84.96%) than solid organ involvement (3.07% of all cases). This study is the most extensive population-based study about the epidemiological and survival data of patients with primary GI KS, revealing GI KS to be a young male disease with best outcomes in the large bowel and anal canal KS while inferior outcomes in extraintestinal GI KS.

S3585 Don’t Forget the Gut: A Case of Gastrointestinal Kaposi Sarcoma

Introduction: Kaposi sarcoma (KS) is a low grade vascular tumor associated with Human Herpesvirus-8 (HHV8) infection. The disease course is indolent with manifestations ranging from cutaneous to fulminant, visceral involvement. In the United States, KS is most often seen in those with AIDS and with increased access to highly active antiretroviral therapy (HAART) the incidence has been decreasing. GI tract involvement of KS is uncommon and few have GI symptoms; therefore, gastrointestinal Kaposi sarcoma (GI-KS) remains under-diagnosed. This case describes a patient with cutaneous involvement of KS found to have GI-KS with no GI symptoms. Case Description/Methods: A 49-year-old African American male presented with shortness of breath, non-productive cough and a 60-lb unintentional weight loss over the past year. He was diagnosed with HIV in 1999 and has been intermittently compliant with HAART therapy. In the past two years, he was started on Bictegravir/emtricitabine/tenofovir alafenamide but was non-compliant. He recently noticed the development of bilateral non-painful and non-pruritic lower skin lesions (Figure a). His CD4 count was low at 88 cells/mm3 with an elevated HIV viral load. His lower extremity skin lesion biopsy showed irregular blood vessels lined by atypical endothelial cells with an infiltrating growth pattern consistent with KS. CT chest demonstrated multiple ground glass opacities and bronchoscopy with FNA were negative for any malignancy. CT abdomen/pelvis was unremarkable. EGD and colonoscopy was done to to rule out systemic involvement which demonstrated scattered moderate mucosal changes characterized by erythema, granular nodularity in the gastric body and nodular mucosa in the rectum (Figure b/c). Biopsies demonstrated spindle cell proliferation involving the lamina propria consistent with KS. Discussion: Kaposi sarcoma is an AIDS-defining rare vascular tumor. Current recommendations for endoscopy are reserved for patients suspicious for GI involvement. This patient had poorly controlled HIV with no GI symptoms and was found to have GI-KS during endoscopy. Management of KS differs based on cutaneous versus systemic involvement. HAART therapy is the first line in management of cutaneous KS while HAART therapy and chemotherapy is first line in management for systemic KS. Therefore, undiagnosed GI-KS in patients with cutaneous KS negatively affects morbidity and mortality. Therefore, clinicians should maintain a high suspicion for GI-KS in those with AIDS and/or cutaneous KS.Figure 1.: a. Lower extremity cutaneous lesions Figure b. Gastric lesions Figure c. Rectal lesions.

S2502 Gastrointestinal Kaposi Sarcoma: A Case Report

Introduction: Kaposi sarcoma (KS) in the gastrointestinal (GI) tract is an uncommon entity specially without any cutaneous manifestation since the introduction of HAART therapy in HIV patients. Most of the gastrointestinal KS, remains undiagnosed as majority of KS is asymptomatic. Here we present a case of GI- KS in a HIV-AIDS patient. Case Description/Methods: A 21-year-old male with history of HIV-AIDS came in for nausea, vomiting and hematochezia for 1 month. Lab showed; total CD4 of 10, elevated ALP 435 U/L, AST, ALT, total and direct bilirubin, lipase was normal. There was periodic increase in ALP hence MRCP was done, which revealed multiple hepatic and intrabiliary lesions. On EGD and colonoscopy, a large infiltrative mass, with recent stigmata of bleeding was found in the duodenum (Figure) to the ileum causing a partial obstruction. The biopsy showed extensive spindle cell proliferation with immunostains positive for HHV8 , ERG which was consistent with Kaposi Sarcoma. A liver biopsy was also done which showed non caseating granuloma with lymphocytic infiltrate with portal ductal proliferation and edema. He underwent ERCP revealing multiple segmental moderate biliary strictures in the common bile duct (CBD), left and right intrahepatic branches. A biliary sphincterotomy and a plastic stent in CBD was performed. He was on HAART therapy and anthracycline based chemotherapy was considered, but the patient passed away due to multiorgan failure from sepsis. Discussion: GI- KS can present as multiple GI symptoms including nausea, vomiting, GI bleeding as in our case or abdominal pain, anemia. (1) However, most of the time gastrointestinal KS remains asymptomatic. (1) Clinician should have high suspicion for GI-KS especially in patients with HIV- AIDS, not in therapy. This will help in the earlier diagnosis and management of GI-KS.Figure 1.: Large infiltrative mass of Kaposi Sarcoma found in duodenum.

PULMONARY KAPOSI SARCOMA WITH ASSOCIATED RECURRENT CHYLOTHORAX

SESSION TITLE: Pneumothorax, Chylothorax, and Pleural Effusion Case PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/17/2022 12:15 pm - 01:15 pmINTRODUCTION: AIDS related Kaposi Sarcoma (KS) is an endothelial malignancy associated with HHV8 infection. It is the most common malignancy in patients with AIDS. Pulmonary KS is uncommon and rarely causes pleural effusions, particularly chylothorax. Here we present a case of recurrent chylothorax leading to diagnosis of pulmonary KS by pleural biopsy.CASE PRESENTATION: 40-year-old male with HIV/AIDS on HAART therapy and KS was referred for pleural effusion. He was previously treated with Doxil for gastrointestinal KS, but stopped due to neutropenia. He had persistent shortness of breath and occasional night sweats. A thoracentesis was performed, and fluid studies were consistent with a chylothorax. He had a recurrent pleural effusion two months later, and he underwent right sided pleuroscopy with pleural biopsies, talc pleurodesis, and tunneled pleural catheter placement. Diffuse hemorrhagic lesions throughout the right lung, and a raised plaque like lesion along the parietal pleura were seen during pleuroscopy. Biopsy of the parietal pleural was consistent with metastatic KS. He will discuss treatment with his oncologist.DISCUSSION: Pulmonary KS is uncommon and rarely causes pleural effusions, particularly chylothorax. Thoracoscopy is usually not needed to diagnose pulmonary KS as it should only affect visceral pleura. As in this case, thoracoscopy is typically used to assist with patient's symptoms and rule out other causes of an effusion. This is a rare case of KS causing chylothorax requiring thoracoscopy and biopsies for diagnosis.CONCLUSIONS: Here we present a case of pulmonary KS with accompanying chylothorax. This is not a typical presentation or path to diagnosis for pulmonary KS.Reference #1: Cherian S, Umerah OM, Tufail M, et al. Chylothorax in a patient with HIV-related Kaposi's sarcoma. BMJ Case Reports CP2019;12:e227641.Reference #2: Ramos A, Granado J, et al. Pulmonary Kaposi Sarcoma - An atypical clinical presentation. International Journal of Infectious Disease. 2022;115:185-188.Reference #3: Pandya K, Lal C, et al. Bilateral Chylothorax with Pulmonary Kaposi's Sarcoma. Chest. 1988;94(6):1316-1317.DISCLOSURES: No relevant relationships by Harshesh AminNo relevant relationships by Yanglin GuoNo relevant relationships by John HeneganNo relevant relationships by Rachel MullinsNo relevant relationships by Randolph Ross SESSION TITLE: Pneumothorax, Chylothorax, and Pleural Effusion Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: AIDS related Kaposi Sarcoma (KS) is an endothelial malignancy associated with HHV8 infection. It is the most common malignancy in patients with AIDS. Pulmonary KS is uncommon and rarely causes pleural effusions, particularly chylothorax. Here we present a case of recurrent chylothorax leading to diagnosis of pulmonary KS by pleural biopsy. CASE PRESENTATION: 40-year-old male with HIV/AIDS on HAART therapy and KS was referred for pleural effusion. He was previously treated with Doxil for gastrointestinal KS, but stopped due to neutropenia. He had persistent shortness of breath and occasional night sweats. A thoracentesis was performed, and fluid studies were consistent with a chylothorax. He had a recurrent pleural effusion two months later, and he underwent right sided pleuroscopy with pleural biopsies, talc pleurodesis, and tunneled pleural catheter placement. Diffuse hemorrhagic lesions throughout the right lung, and a raised plaque like lesion along the parietal pleura were seen during pleuroscopy. Biopsy of the parietal pleural was consistent with metastatic KS. He will discuss treatment with his oncologist. DISCUSSION: Pulmonary KS is uncommon and rarely causes pleural effusions, particularly chylothorax. Thoracoscopy is usually not needed to diagnose pulmonary KS as it should only affect visceral pleura. As in this case, thoracoscopy is typically used to assist with patient's symptoms and rule out other causes of an effusion. This is a rare case of KS causing chylothorax requiring thoracoscopy and biopsies for diagnosis. CONCLUSIONS: Here we present a case of pulmonary KS with accompanying chylothorax. This is not a typical presentation or path to diagnosis for pulmonary KS. Reference #1: Cherian S, Umerah OM, Tufail M, et al. Chylothorax in a patient with HIV-related Kaposi's sarcoma. BMJ Case Reports CP2019;12:e227641. Reference #2: Ramos A, Granado J, et al. Pulmonary Kaposi Sarcoma - An atypical clinical presentation. International Journal of Infectious Disease. 2022;115:185-188. Reference #3: Pandya K, Lal C, et al. Bilateral Chylothorax with Pulmonary Kaposi's Sarcoma. Chest. 1988;94(6):1316-1317. DISCLOSURES: No relevant relationships by Harshesh Amin No relevant relationships by Yanglin Guo No relevant relationships by John Henegan No relevant relationships by Rachel Mullins No relevant relationships by Randolph Ross

Histologic Variants of Kaposi Sarcoma in the Gastrointestinal Tract: A Contemporary Multi-institutional Clinicopathologic Analysis of 46 Cases.

Kaposi sarcoma (KS) can pose diagnostic challenges in biopsy specimens. Multiple histologic variants of cutaneous KS have been described; however, the histomorphologic spectrum of gastrointestinal (GI) KS has not been systematically studied. This large series comprehensively evaluated 46 cases of KS involving the GI tract and identified 7 histomorphologic variants, some that have not been previously described. Five of them are inconspicuous but have unique morphologic patterns, including lymphangioma/lymphangiectatic-like (n=17), mucosal hemorrhage/telangiectatic-like (n=17), mucosal inflammation-like (n=15), granulation tissue-like (n=13), and mucosal prolapse-like (n=4) variants. These variants can be easily misdiagnosed or misinterpreted on routine examination if KS is not considered, and if the immunohistochemical stain for human herpesvirus-8 is not performed. The other 2 morphologic variants present as spindle cell proliferations and are the GI stromal tumor-like (n=8) and inflammatory myofibroblastic tumor-like (n=2). These variants raise a broad differential diagnosis of spindle cell tumors of the GI tract and could pose diagnostic challenges. In summary, GI KS lesions exhibit variable, often unconventional histomorphologic patterns. KS should be included in the differential diagnosis even if features of conventional KS are not seen, particularly in limited biopsies in immunocompromised patients, such as those with human immunodeficiency virus infection. Although the clinical significance of these morphologic variants is yet to be determined, they are nonetheless important from a diagnostic standpoint. Misdiagnosis and delay in appropriate management can be avoided by recognizing the morphologic diversity of GI KS and appropriately utilizing the human herpesvirus-8 immunohistochemical stain.

Kaposi's sarcoma-associated herpes virus-derived microRNA K12-1 over-activates the PI3K/Akt pathway to facilitate cancer progression in HIV-related gastrointestinal Kaposi's sarcoma.

Kaposi's sarcoma-associated herpes virus (KSHV) initiate and accelerate the development of Kaposi's sarcoma (KS), and KSHV possesses many cancer-associated genes, including KSHV-derived microRNA miR-K12-1, which has been identified to be closely associated with KS progression. However, the detailed mechanisms by which miR-K12-1 facilitates HIV-related gastrointestinal KS development are still not fully delineated. This study strived to evaluate the effect of miR-K12-1 on the progression of HIV-related gastrointestinal KS. The expression levels of miR-K12-1 in HIV-related gastrointestinal KS tissues were determined by RT-qPCR. Proliferation and apoptosis were assessed by colony formation, CCK-8 and flow cytometry, respectively. The expression of all proteins was detected by Western blot. The in vivo effect of miR-K12-1 on the formation of a tumor was explored by using the mouse xenograft model. In this study, we uncovered that KSHV-miR-K12-1 was upregulated in HIV-related gastrointestinal KS tissues and associated with poor outcome in HIV-related gastrointestinal KS patients. Compared with the control group, after miR-K12-1 inhibitor transfection, BCBL-1 cell viability was decreased, and the cell apoptosis was significantly increased, whereas transfection of miR-K12-1 mimics promoted cell proliferation and mitosis. In addition, our rescuing experiments verified that miR-K12-1 promoted cell proliferation via activating the PI3K/Akt pathway, and inhibition of the PI3K/Akt pathway by LY294002 abrogated the tumor-promoting effects of miR-K12-1 in HIV-related gastrointestinal KS. In summary, we concluded that KSHV-derived miR-K12-1 activate the PI3K/Akt pathway to initiate and accelerate the development of KS, which convinces us that miR-K12-1 can be used as potential biomarkers for KS diagnosis, treatment and prognosis.

Acute Appendicitis Secondary to Kaposi's Sarcoma: Case Report and Literature Review

Acute appendicitis secondary to gastrointestinal Kaposi sarcoma is a rare but critical manifestation of this condition. An acute abdomen in patients with HIV-AIDS can be secondary to Kaposi sarcoma with appendiceal involvement. The timely identification of other clinical manifestations of pathologies associated with immunosuppression can lead to a promptly diagnosis. Regardless of the management of the underlying pathology, the treatment of choice for acute appendicitis is surgical.

Gastrointestinal: Rapid-growing multiple maculopapular and polypoid lesions in the gastrointestinal tract.

A 34-year-old human immunodeficiency virus (HIV)-infected man, who had received highly active antiretroviral therapy (HAART) for about 3 weeks with initial low CD4 count (84 cells/μL), presented to the hospital with dyspnea. Physical examination did not reveal any significant skin lesion. The patient developed melena during hospitalization, and the hemoglobin dropped to 5.8 g/dL from 11.4 g/dL within 1 week. The esophagogastroduodenoscopy and colonoscopy showed multiple 0.5- to 1.0-cm reddish maculopapular and polypoid lesions at the esophagus (Fig. 1a), stomach (Fig. 1b), colon (Fig. 1d), and with central ulceration at the duodenum (Fig. 1c), which were not present in his previous esophagogastroduodenoscopy 1 month ago. Biopsies revealed lamina propria-based lesion composed of a focal proliferation of spindle cells in fascicles with interstitial erythrocytes (Fig. 2a,b). Human herpesvirus 8 staining (Fig. 2c) was positive. An inclusion body was detected, and the cytomegalovirus (CMV) staining (Fig. 2d) was positive. In addition to the pathological characteristics of Kaposi's sarcoma, CMV stain showed positive-staining cells in the specimens of the stomach, duodenum, and colon, compatible with Kaposi's sarcoma with CMV coinfection. Kaposi's sarcoma is a low-grade tumor of the vascular endothelium, often associated with HIV. The manifestation of gastrointestinal Kaposi's sarcoma is variable, and can be asymptomatic or can cause gastrointestinal symptoms such as nausea, vomiting, malabsorption, and gastrointestinal bleeding. There are different endoscopic characteristics of Kaposi's sarcoma, such dark pink or scarlet maculopapular, polypoid lesion, or volcano-like lesions. The pathological findings include spindle cells with cytologic atypia, extravasated red blood cells, and lymphocytic and plasma cell infiltrate. Improvement in immunologic function after the initiation of HAART may unmask previous unrecognized preexisting infection, as also known as immune reconstitution inflammatory syndrome. In our case, the rapid progression of gastrointestinal Kaposi's sarcoma with CMV coinfection could be one of the manifestations of immune reconstitution inflammatory syndrome, a significant but uncommon clinical problem in the post-HAART era. Furthermore, endoscopic biopsies may still carry a risk of false-negative results, especially for the lesion in esophagus, with small size (< 1 cm) or localized in submucosa. Caution should be taken in performing endoscopy in HIV-infected patients, even for those who had received HAART.

S2212 Diffuse Gastrointestinal Kaposi Sarcoma in a Patient With HHV-8 Associated Multicentric Castleman Disease

Introduction: Castleman disease is a rare non-clonal lymphoproliferative disorder characterized by non-neoplastic lymphadenopathy that can affect any region of the body. Castleman disease adenopathy can either be localized to a single site, or more widespread. In the latter case, Multicentric Castleman Disease (MCD) is further classified as either HHV-8 negative/idiopathic MCD or HHV-8 associated MCD. MCD can present aggressively with systemic manifestations, and treatment differs based on the presence of Kaposi Sarcoma (KS). In this report, we describe a patient with HIV/AIDS that was found to have diffuse gastrointestinal KS and was ultimately diagnosed with HHV-8 associated MCD. Case Description/Methods: A 44 year old male with recently diagnosed HIV/AIDS and cutaneous KS presented with complaints of night sweats, dizziness, weight loss, and diarrhea. He reported waxing and waning lymphadenopathy present for at least one year. Physical examination was notable for diffuse lymphadenopathy and an enlarged spleen. Initial laboratory studies were remarkable for CD4 count of 70, Hgb 6.0g/dL, platelets 91 k/uL, sodium of 121mmol/L. CT of the abdomen and pelvis demonstrated diffuse lymphadenopathy as well as marked splenomegaly. CT of the chest showed thoracic lymphadenopathy, and MRI Brain revealed cervical chain lymphadenopathy. GI was consulted to evaluate for systemic/GI KS or other lesions to guide definitive treatment. EGD and colonoscopy demonstrated nodules in the stomach, ascending colon, and rectum all of which were biopsied and revealed KS. An excisional biopsy of an inguinal lymph node was obtained, pathology revealed Castleman lymphadenopathy, plasma cell variant. Oncology recommended that the patient be started on rituximab and liposomal doxorubicin for treatment of HHV-8 associated MCD with concurrent Kaposi Sarcoma. Discussion: We present a rare case of HHV-8 associated MCD with concomitant KS found diffusely in the GI tract and skin. There was initially significant diagnostic uncertainty until lymph node biopsy was performed. Interestingly, therapeutic recommendations for MCD depend on the presence or absence of KS, and gastrointestinal KS can be present without cutaneous KS. MCD without KS is treated with four weeks of rituximab, and if KS is present, doxorubicin or etoposide is included. This case highlights the potential for GI involvement of KS in MCD, and in particular the need to pursue endoscopy in patients with HH/V-8 associated MCD to evaluate for KS and guide treatment decisions.Figure 1.: Kapsoi Sarcoma in the gastric antrum (a), gastric body (b), sigmoid colon (c), and rectum (d).

Beyond T Staging in the "Treat-All" Era: Severity and Heterogeneity of Kaposi Sarcoma in East Africa.

Although many patients with Kaposi sarcoma (KS) in sub-Saharan Africa are diagnosed with AIDS Clinical Trials Group (ACTG) T1 disease, T1 staging insufficiently captures clinical heterogeneity of advanced KS. Using a representative community-based sample, we detailed disease severity at diagnosis to inform KS staging and treatment in sub-Saharan Africa. We performed rapid case ascertainment on people living with HIV, aged 18 years or older, newly diagnosed with KS from 2016 to 2019 at 3 clinic sites in Kenya and Uganda to ascertain disease stage as close as possible to diagnosis. We reported KS severity using ACTG and WHO staging criteria and detailed measurements that are not captured in the current staging systems. We performed rapid case ascertainment within 1 month for 241 adults newly diagnosed with KS out of 389 adult patients with suspected KS. The study was 68% men with median age 35 years and median CD4 count 239. Most of the patients had advanced disease, with 82% qualifying as ACTG T1 and 64% as WHO severe/symptomatic KS. The most common ACTG T1 qualifiers were edema (79%), tumor-associated ulceration (24%), extensive oral KS (9%), pulmonary KS (7%), and gastrointestinal KS (4%). There was marked heterogeneity within T1 KS, with 25% of patients having 2 T1 qualifying symptoms and 3% having 3 or more. Most of the patients newly diagnosed with KS had advanced stage disease, even in the current antiretroviral therapy "treat-all" era. We observed great clinical heterogeneity among advanced stage patients, leading to questions about whether all patients with advanced KS require the same treatment strategy.

Corticosteroid-induced Kaposi's Sarcoma Revealed by Severe Anemia: A Case Report and Literature Review.

We herein report a case of gastrointestinal (GI) Kaposi's sarcoma (KS) without cutaneous involvement in a 73-year-old man who had received immunosuppressive drugs for granulomatosis with polyangiitis. After one year of prednisolone use, he presented with tarry stool and severe anemia. Endoscopic and pathological examinations revealed bright-reddish protruding lesions with proliferating spindle cells positive for D2-40, CD34, and HHV-8, which are definitively diagnostic of GI-KS. Drug-induced KS without HIV infection or transplantation is extremely rare, and its clinical features remain unknown. Therefore, we conducted a literature review of steroid-induced KS.

2764 An Unusual Presentation of Gastric Kaposi's Sarcoma

INTRODUCTION: Kaposi sarcoma (KS) is a low grade angioproliferative tumor. AIDS-related KS is the most common and aggressive form of KS. The prevalence of AIDS-related KS is up to 30%. Skin is the most commonly affected organ in 78% of cases, The gastrointestinal (GI) tract is the most commonly affected extracutaneous site, and often asymptomatic. In advanced cases, patients may present with GI bleeding, diarrhea, protein-losing enteropathy, intussusception, or perforation. Although only 20% of KS patients experience GI symptoms, some studies have found that up to 80% of KS patients have GI lesions at autopsy. With the advent availability of HARRT therapy, the incidence of Kaposi sarcoma has decreased dramatically. Our objective is to present an unusual presentation of Kaposi sarcoma and its endoscopic findings leading to the diagnosis of KS, to share how endoscopy can be used as a tool for diagnosing and assessing the GI involvement in KS. CASE DESCRIPTION/METHODS: A 35-year-old African American male with HIV, syphilis, and hepatitis B complains of swelling of his legs and scrotum for 2 months. Physical examination revealed abdominal distension, bilateral lower extremity edema, and one skin nodule on his right foot. Laboratory studies revealed Hgb 8.7 g/dL, MCV 95.3 fL/cell, WBC 7500/cmm, CD4 count &lt; 80 cells/uL, and albumin 1.4 g/dL. CT imaging revealed significant abdominal ascites (Figure 1). The patient underwent paracentesis removing 3.5 L of milky fluid. Fluid culture, gram stain and cytology were negative. Given the ascites and hepatitis history, an esophagogastroduodenoscopy (EGD) was completed in evaluating for possible esophageal varices. EGD revealed a nodular fungating lesion that extends diffusely from the gastric body into the duodenal bulb and sweep (Figures 2 and 3). Biopsy revealed cylindrical cells positive for HHV-8, confirming the diagnosis of gastric Kaposi's sarcoma (KS). Immunohistochemical analysis of the skin nodule biopsy confirmed KS. Computed tomography scan of the chest was consistent with pulmonary metastases. Patient is being treated with HAART and will be started on chemotherapy. DISCUSSION: KS is a rare neoplasm, and an AIDS-defining illness in HIV patients. About 40-51% of HIV patients with cutaneous KS will develop gastrointestinal KS, which is typically asymptomatic until growth is extensive enough to cause abdominal pain. This case illustrates the importance of endoscopic evaluation in HIV patients for early detection of gastrointestinal involvement of KS.

1496 Visceral Involvement as the Initial Presentation of Kaposi Sarcoma Carries a Worse Prognosis

INTRODUCTION: AIDS-related Kaposi sarcoma (KS) is a vascular tumor associated with infection by the human herpesvirus-8 (HHV-8) that most commonly affects mucocutaneous sites. In its disseminated form, it can also involve lymph nodes and visceral organs, in particular the respiratory and gastrointestinal tract. Visceral involvement as the initial manifestation of KS is extremely rare and carries a worse prognosis. We present a case of a 54-year-old Male with disseminated KS with rectal involvement presenting with cough and chills. CASE DESCRIPTION/METHODS: 54-year-old Male with a past medical history significant for HIV, not adherent with HAART therapy, who presented to the Emergency Department (ED) with a chief complaint of cough and subjective chills of three days duration. He later endorsed recent sick contacts, bloody diarrhea, and a 40 lb weight loss over the last year. In the ED, he was hypotensive and tachycardic and found to have a CD4 13 with a viral load of 3 million copies/ml. A CT Chest showed evidence of bilateral pleural effusions for which he was started on broad spectrum antibiotics. He underwent bronchoscopy with BAL which showed small nodules suspicious for KS versus Lymphoma. His hospital course was complicated by microcytic anemia (Hb 6.1 on admission) with a positive fecal occult test requiring multiple blood transfusions. Gastroenterology was consulted and the patient underwent colonoscopy which showed evidence of a large, non obstructing, fungating mass in the distal rectum. The mass had a purple hue, was noncircumferential, and was confluent with multiple scattered oozing purple sessile nodules. Biopsies were taken with cold forceps for histology. The rectal biopsy was positive for Human Herpes Virus 8 (HHV-8) and he was diagnosed with disseminated KS. He was subsequently transferred to the National Institutes of Health Clinical Center in Maryland after failed initial treatment for further management and care. DISCUSSION: Visceral involvement as the initial presentation of KS is rare and carries a worse prognosis. While gastrointestinal KS lesions are usually asymptomatic, they may also cause a wide array of symptoms including nausea and vomiting, weight loss, upper or lower gastrointestinal bleeding, and abdominal pain. Because visceral involvement is rare as the initial presentation, the possibility of disseminated KS in an immunocompromised patient is often over looked and thus, a high index of suspicion is required.

P061 CYCLOSPORINE INDUCED KAPOSI SARCOMA IN AN IBD PATIENT: A DOWNWARD SPINDLE

Kaposi Sarcoma is an angioproliferative neoplasm found to be associated with human herpesvirus-8 (HHV8). Although this virus plays a role in the pathogenesis of the disease, in individuals who are human immunodeficiency virus (HIV) negative, it is exceedingly rare. Still, there have been documented cases of individuals developing gastrointestinal Kaposi sarcoma in the setting of immunosuppression, specifically solid organ transplant patients, and less frequently inflammatory bowel disease (IBD). We report a 24-year-old HIV-negative man with a history of ulcerative colitis (UC), psoriasis, atopic dermatitis, and alopecia aereata who was referred to the gastroenterology clinic after experiencing 12 episodes of watery non-bloody diarrhea per day and 20 lb. weight loss over the duration of one-month. Onset of watery diarrhea coincided with starting antibiotics (amoxicillin) for periodontal abscess. Patient reported that since his UC had been well-controlled for several years on cyclosporine prior to his presentation, he attributed the diarrhea and weight loss to antibiotic use. He was initially diagnosed with UC at age 15 and had previously been treated with multiple agents including adalimumab and intermittent prednisone without optimal response. Evaluation showed that he was positive for enteropathic E. coli and norovirus which was thought to be contributing to the diarrhea; he was negative for Giardia, Clostridium difficile, and stool cultures. Given his ongoing symptoms and history of UC, colonoscopy was performed which demonstrated multiple hemorrhagic nodules throughout the colon and terminal ileum. Biopsy results showed vasoformative spindle cell lesions compatible with Kaposi sarcoma that was HHV-8 positive and CMV negative in the terminal ileum and colon. CT chest/abdomen/pelvis demonstrated diffuse lymphadenopathy. He underwent right axillary lymph node biopsy which was consistent with Kaposi sarcoma. His cyclosporine and Truvada were discontinued. Oncology was consulted for further management. Since 1966, there have been only 16 cases of patients documented with IBD developing Kaposi sarcoma secondary to immunosuppressive therapy. Frequently, these patients were on prednisone or biologics with only two patients receiving cyclosporine for a brief period during the course of their therapy. Iatrogenic Kaposi sarcoma has been documented among transplant patients. We report a rare case of an individual with ulcerative colitis developing iatrogenic Kaposi sarcoma in the setting of long-term cyclosporine therapy. (A & B) H&E stained tissue sections of the colon biopsies demonstrates a vasoformative lesion composed of spindle cells with many small, poorly formed, slit-like vascular spaces involving the lamina propria and submucosa. Immunohistochemical stains demonstrate that this lesion is positive for endothelial markers CD31 (C) and CD34 (not pictured) as well as for Human Hepesvirus 8 (HHV-8) (D).

Intestinal Kaposiʼs Sarcoma in a Patient With Cirrhosis

We present a case of iatrogenic sarcoma or sarcoma associated with immunosuppression related to cirrhosis. Currently, this is second report in the literature of a case of cirrhosis associated with gastrointestinal Kaposi's sarcoma unrelated to liver transplant or HIV infection. The previous case reported was of a 66-year-old male patient diagnosed with alcoholic cirrhosis and portal hypertension syndrome, in whom stomach lesions were identified (1). Our purpose is to inform this infrequent relationship. This was a 66 year old male patient who consulted due to melenas. On admission he was hypotensive, and physical exam findings included edema, ascites and angiomatous lesions on his legs, thighs and palate. An endoscopy and a colonoscopy showed violaceous papular lesions in the duodenum and colon (Image 1), biopsy and immunohistochemistry confirmed Kaposi's sarcoma (Image 2 and 3). The ELISA test for HIV was negative. The patient developed acute liver failure in addition to the chronic failure, with type 1 hepatorenal syndrome. He was not a candidate for liver transplant and died during hospitalization. The true prevalence of Kaposi sarcoma in patients with cirrhosis is still unknown. Most patients with gastrointestinal Kaposi's sarcoma in the variety associated with AIDS are asymptomatic (2), therefore endoscopies carried out for other reasons should be used to study suspicious lesions. The differential diagnosis includes gastrointestinal stromal tumors, angiosarcoma, and fusiform cell melanoma (2). The identification of HHV8 is recommended for all specimens with a fusiform appearance; this marker has a 99% sensitivity and 100% specificity (2). Altered cellular immunity and HHV-8 antibodies have been described in cirrhosis (3), which would also support the role of HHV8 in tumor development. Treatment depends on the epidemiological variant, at the moment no specific treatment has been established in this subgroup of patients.1910_A Figure 1. Flat macular purplish lesion seen in colonoscopy1910_B Figure 2. Biopsy of duodenum Kaposi lesion with immunohistochemistry stain for HHV-8.1910_C Figure 3. Biopsy of duodenum Kaposi lesion with immunohistochemistry stain for CD31.

Kaposi Sarcoma of the Gastrointestinal Tract in the Era of Combination Anti-Retro Viral Therapy

AIDS related Kaposi sarcoma (KS) is the most common tumor identified in HIV patients. Classically presents as cutaneous vascular endothelial tumors associated with HHV-8 infection. Visceral manifestation in the absence of cutaneous manifestation is uncommon. The annual incidence of Kaposi sarcoma in the United States has been steadily declining with the advent of highly potent combination anti-retroviral therapy (ART). We present an unfortunate case of a HIV positive male with extensive gastrointestinal Kaposi sarcoma. 50 year old male with prior diagnosis of HIV, non-compliant with ART presented with weight loss, abdominal pain, distention, vomiting and diarrhea for 3 months. Examination revealed a cachectic appearing male, moderate abdominal distention with mild diffuse tenderness throughout the abdomen. No cutaneous lesions identified on examination. Blood work significant for anemia, leukopenia, elevated HIV viral load >210,000 with a low CD4 count of 100. Stool studies for ova and parasites were negative. CT imaging revealed long segment mural thickening extending from the terminal ileum, descending colon to the rectum with multiple dilated fluid filled loops of small bowel with scattered large to small extensive mesenteric lymphadenopathy. EGD revealed multiple raised red to purplish nodular lesions in the oropharynx, esophagus, stomach and duodenum. Similar lesions were found on colonoscopy throughout the colon and terminal ileum associated with significant edema and narrowing in the region of the terminal ileum and sigmoid colon (Image 1). Biopsy from various sites revealed spindle cell proliferation with extravasation of red blood cells (Image2). Immunostain positive for HHV-8 consistent with the diagnosis of Kaposi's sarcoma (Image3). Patient improved on ART and was later discharged home with plans for outpatient chemotherapy. Significant advancements in the field of ART has led to a decrease in the prevalence of Kaposi sarcoma in the 21st century. Incidence of KS has shown a significant decline in United states from 47 cases per 1 million people in the 1990 to 6 cases per 1 million people because of highly effective treatments available for HIV/AIDS. Gastrointestinal manifestations commonly reported are abdominal pain, malabsorption, bleeding and rarely obstruction. GI manifestation can be easily missed in the absence of cutaneous manifestation, hence a high degree of suspicion can lead to early diagnosis and improved outcomes.2984_A Figure 1. Colonoscopy images showing multiple raised red to purple nodules throughout the colon2984_B Figure 2. H&E stain of the duodenal mucosa showing scattered bland spindle cell proliferation with extravasated red blood cells.2984_C Figure 3. Immunostain positive for HHV -8

Sa1815 - Utility of Immunostaining in the Diagnosis of Gastrointestinal Kaposi's Sarcoma Related to Acquired Immunodeficiency Syndrome: A Retrospective Study

Sa1815 - Utility of Immunostaining in the Diagnosis of Gastrointestinal Kaposi's Sarcoma Related to Acquired Immunodeficiency Syndrome: A Retrospective Study