Involvement In Kaposi Sarcoma Research Articles

Cardiac Involvement in Kaposi Sarcoma, an Unexpected Finding

Cardiac Involvement in Kaposi Sarcoma, an Unexpected Finding

Abstract 27: Demographic Factors Associated with Extracutaneous Involvement in Kaposi Sarcoma

Abstract Purpose: Kaposi sarcoma (KS) is a soft tissue tumor that typically presents with red or purple skin lesions. However, extracutaneous involvement can occur in severe disease. The National Cancer Database (NCDB) compiles data from 1,400 cancer programs across the United States; to our knowledge, there has yet to be a study in the NCDB comparing demographic features and presentation of KS with cutaneous versus extracutaneous disease. Thus, there is a need to characterize the KS cohort with regards to primary site. Methods: We conducted a retrospective analysis of the NCDB from 2004 to 2019 for Kaposi sarcoma patients (n=5,587). Analysis was completed between patients with cutaneous (n=3,963) and extracutaneous (n=1,624) involvement using Pearson Chi-square and t-tests. Survival was estimated using Kaplan-Meier curves. Results: Non-cutaneous lesions made up 29.1% of diagnoses and primarily included connective/subcutaneous tissue (12.2%), as well as digestive organs (4.1%). Median survival was lower in the extracutaneous (101.7 months) cohort compared to cutaneous (131.5 months) cohort (p<0.001). Women were more likely to present with non-cutaneous lesions compared to men (37.3% versus 27.6%, p<0.001). Spanish/Hispanic subjects were less likely to have extracutaneous involvement compared to non-Spanish/Hispanic subjects (p=0.006). Patients at academic centers were less likely to have extracutaneous lesions compared to other facilities (p<0.001). Residing in the Central United States was associated with increased incidence of extracutaneous disease (p<0.001). Patients with extracutaneous disease lived further from the reporting hospital (23.95 miles) compared to patients with cutaneous (16.24 miles) involvement (p=0.004). There was no significant difference in mean age between cutaneous (58.41 years) and non-cutaneous (57.97 years) cohorts. No clear trend existed between race, insurance status, education, or income in relation to extracutaneous disease. Conclusion: To our knowledge, our study is the first to compare demographic features and trends associated with cutaneous and extracutaneous KS in the NCDB. We revealed that extracutaneous primary site is associated with poorer survival and highlighted several significant differences between the cohorts. Citation Format: Michelle Swedek, Xinxin Wu, Peter Silberstein, Victoria Vardell. Demographic Factors Associated with Extracutaneous Involvement in Kaposi Sarcoma [abstract]. In: Proceedings of the 11th Annual Symposium on Global Cancer Research; Closing the Research-to-Implementation Gap; 2023 Apr 4-6. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(6_Suppl):Abstract nr 27.

S2598 Gastric Kaposi Sarcoma Presenting as Acute Upper Gastrointestinal Hemorrhage in a Heart Transplant Patient

Introduction: Kaposi sarcoma (KS), a multifocal neoplasm of lymphatic cells, occurs more often in organ transplant patients, and its pathogenesis is related to use of immunosuppressive (IS) medications. Acute gastrointestinal hemorrhage due to KS is exceedingly rare and has never been reported in a heart transplant patient. We report a case of KS presenting as gastrointestinal hemorrhage in a heart transplant patient whose bleeding resolved with supportive care and treatment of his underlying KS. Case Description/Methods: A 63-year-old man originally from Sudan with a history of non-ischemic cardiomyopathy requiring heart transplant on IS presented with melena and acute anemia. Medical history included cutaneous KS of the right leg diagnosed 1 year following transplant previously treated with paclitaxel and in clinical remission. He was treated with intravenous proton-pump inhibitor and blood transfusions. EGD showed a patch of nodular mucosa on the lesser curvature of the stomach. Pathology of the nodule showed dilated capillaries with spindle cells in the lamina propria, and staining for human herpesvirus-8 (HHV-8) was positive in the spindled cells, supporting a diagnosis of recurrent KS. He had no further bleeding, hemoglobin stabilized, and he was discharged. He was resumed on paclitaxel for recurrent KS after discharge and had no further bleeding (Figure 1). Discussion: We report a case of KS presenting with GI bleeding in a patient on chronic IS following heart transplant. While GI bleeding from KS has been reported in renal transplant recipients, this is the first such report we are aware of in a heart transplant patient. The GI tract is a common site of visceral involvement in KS, and presentation can include vomiting, diarrhea, and occult blood loss. Overt GI bleeding in KS is rare. Endoscopic appearance varies from flat lesions to polypoid or nodular growths, and histopathology shows spindle cells positive for HHV-8. Management of GI bleeding in KS depends on severity, and in less severe cases, the role of endoscopy is to confirm the visceral spread of KS with biopsy. Thereafter, management includes IS reduction and chemotherapy for refractory disease. Though KS is a rare cause of overt GI bleeding, it should be considered in patients with immunodeficiency or on chronic IS, and biopsy for suspicious lesions should be performed to confirm diagnosis.Figure 1.: Lesser curvature gastric nodule.

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 < 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.

Bilateral eyelid Kaposi's sarcoma in an HIV-negative patient

Abstract Case report We report a case of 70-year-old male from Sicily, who presented with a bilateral eyelid mass involving the tarsal conjunctiva, found to be Kaposi's sarcoma on histologic examination. Cutaneous and pulmonary Kaposi's sarcoma involvement was documented. The patient had no prior diagnosis of human immunodeficiency virus infection. This case was managed successfully after the completion of five cycles of chemotherapy with liposomal doxorubicin, and his eyelid, skin and pulmonary lesions disappeared. Conclusions Location in the eyelid is a possible, though rare, initial solitary manifestation of Kaposi's sarcoma in elderly HIV-negative patients. Liposomal doxorubicin is a safe and effective treatment.

Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease

BackgroundEndemic Kaposi sarcoma (KS) was first described in African children over fifty years ago, but has recently been overshadowed by HIV-related disease. We aimed to evaluate the similarities and differences between endemic HIV-negative and epidemic HIV-positive pediatric KS in a KS-associated herpesvirus-endemic region of Africa.MethodsWe describe clinical characteristics of 20 HIV-negative children with endemic KS over a six-year period and compare findings with a historical control—an HIV-related pediatric KS cohort from Lilongwe, Malawi.ResultsThe HIV-negative endemic KS cohort was 70% male with a median age of 9.3 years. Lymph node involvement was present in 50%, hyperpigmented skin lesions in 45%, and woody edema in 40%. One patient (5%) presented with oral KS involvement and no patients presented initially with visceral KS. Significant anemia (hemoglobin < 8 g/dL) and thrombocytopenia (platelet count < 100 × 109/L) were found at time of original KS diagnosis in 45 and 40% respectively. In both HIV-negative and HIV-positive cohorts, lymphadenopathy was the most common presentation, prototypical skin lesions were often absent, severe cytopenias were a common clinical feature, and treatment outcomes were similar. Patients with endemic KS demonstrated less frequent oral involvement (5% versus 29%, P = 0.03) and a lower proportion of patients with visceral involvement (0% versus 16%, P = 0.06).ConclusionsThese data suggest clinical overlap between epidemiological variants. Treatment protocols for pediatric KS in sub-Saharan Africa should be devised to include both endemic HIV-negative and epidemic HIV-related disease to better define the clinical and biological comparison.

Sarcoma de Kaposi palpebral bilateral en un paciente virus de la inmunodeficiencia humana negativo

Case reportWe report a case of 70-year-old male from Sicily, who presented with a bilateral eyelid mass involving the tarsal conjunctiva, found to be Kaposi's sarcoma on histologic examination. Cutaneous and pulmonary Kaposi's sarcoma involvement was documented. The patient had no prior diagnosis of human immunodeficiency virus infection. This case was managed successfully after the completion of five cycles of chemotherapy with liposomal doxorubicin, and his eyelid, skin and pulmonary lesions disappeared. ConclusionsLocation in the eyelid is a possible, though rare, initial solitary manifestation of Kaposi's sarcoma in elderly HIV-negative patients. Liposomal doxorubicin is a safe and effective treatment.

Case Report: Pulmonary Kaposi Sarcoma in a non-HIV patient.

Kaposi Sarcoma (KS) is an angioproliferative tumor associated with human herpes virus 8 (HHV-8). Often known as one of the acquired immunodeficiency syndrome (AIDS)-defining skin diseases, pulmonary involvement in KS has only been discussed in a handful of case reports, rarely in a non-HIV patient. Herein we report the case of a 77 year-old- male who presented with a 6-week history of progressive dyspnea on exertion accompanied by productive cough of yellow sputum and intermittent hemoptysis. His past medical history was significant for Non-Hodgkin's Follicular B-Cell Lymphoma (NHL). Patient also had biopsy-confirmed cutaneous KS.His physical exam was notable for a 2cm firm, non-tender, mobile right submandibular lymph node. Lungs were clear to auscultation. He had multiple violet non-tender skin lesions localized to the lower extremities. CT scan of the chest showed numerous nodular opacities and small pleural effusions in both lungs. A thoracenthesis was performed, showing sero-sanguineous exudative effusions. Histopathology failed to demonstrate malignant cells or lymphoma. A subsequent bronchoscopy revealed diffusely hyperemic, swollen mucosa of the lower airways with mucopurulent secretions. Bronchoalveolar lavage PCR for HHV-8 showed 5800 DNA copies/mL. It was believed that his pulmonary symptoms were likely due to disseminated KS. This case illustrates the potential for significant lung injury from KS. It also demonstrates the use of PCR for HHV-8 to diagnose KS in a bronchoalveolar lavage sample in a case when bronchoscopic biopsy was not safe. Furthermore, this case is unique in that the patient did not match the typical KS subgroups as HIV infection and other immune disorders were ruled out. Recognition of this syndrome is critical to the institution of appropriate therapy. As such, this case should be of interest to a broad readership across internal medicine including the specialties of Pulmonology and Critical Care.

Kaposi sarkomunda gastrik tutulum ve Helicobacter pylori

Giris ve Amac: Kaposi sarkom siklikla cilt bulgulari ile ortaya cikar. Gast- rointestinal sistem nadiren tutuldugu icin endoskopistler gastrik lezyonlara asina degildir. Bu calismada klasik Kaposi sarkomu ve kazanilmis immun yetmezlik sendromu iliskili Kaposi sarkomu olgularimizi gastrik semptomlar, gastrik tutulum ve Helicobacter pylori enfeksiyonu birlikteligi acisindan de- gerlendirmeyi amacladik. Gerec ve Yontem: Kaposi sarkomu tanisi ile takip edilen 18 olgu calismaya dahil edildi. Kontrol grubu olarak poliklinige basvu- ran ve Helicobacter pylori acisindan test edilen 40 olgu calismaya dahil edildi. Kaposi sarkomlu tum olgularin evreleri, aldiklari tedaviler, cilt ve oral muaye- nesi kaydedildi. Olgularin gastrik yakinmalarinin olup olmadigi sorgulandi. Tum olgulara gastroduodenoskopi yapildi. Histopatolojik olarak gastrik tutu- Background and Aims: Kaposi's sarcoma usually presents as skin lesions. Since the gastrointestinal tract is rarely involved in Kaposi's sarcoma patients, endoscopists are not familiar with gastric lesions. In the present study, we aimed to evaluate patients with classical Kaposi's sarcoma and acquired im- munodeficiency syndrome -associated Kaposi's sarcoma with respect to gas- tric symptoms, gastric involvement, and the presence of Helicobacter pylori. Materials and Methods: This study included 18 patients with Kaposi's sar- coma. Forty patients, admitted to the outpatient clinic for Helicobacter pylori, were included as the control group. Stage of the disease, therapy, and der- matological and oral examination findings were recorded. All patients were questioned regarding gastric symptoms and underwent upper gastrointesti- nal endoscopy. Biopsies were taken from areas suspected as having Kaposi's sarcoma involvement and from the corpus and antrum for the presence of Helicobacter pylori, and results were compared with the control group. Re- sults: Among the 18 patients included in the study, 17 (94.4%) of them were male, and the mean age of the patients was 59.3 (39-79) years. Fif- teen patients (83.3%) had classical Kaposi's sarcoma, while 3 (16.7%) had acquired immunodeficiency syndrome -associated Kaposi's sarcoma. Of the 5 patients suspected of having Kaposi's sarcoma on upper gastrointestinal endoscopy, the diagnosis of Kaposi's sarcoma was verified in 3 patients his- topathologically. The fundus and corpus were involved in 2 patients and the antrum in 1 patient. None of the biopsies taken from areas other than gas- tric lesions had any findings associated with Kaposi's sarcoma. In general, 10 patients (55.6%) were symptomatic. Two of 3 (66.6%) patients with gastric involvement and 8 of 15 (55.3%) patients without gastric involvement were symptomatic. Helicobacter pylori was positive in 66.6% of all patients with Kaposi's sarcoma and in 52.5% of patients in the control group (p=0.206). Two patients with Kaposi's sarcoma and gastric involvement were positive for Helicobacter pylori, and these patients had oral involvement as well as ac- quired immunodeficiency syndrome -associated Kaposi's sarcoma, and they were younger. Conclusions: Presence of gastric symptoms was not enough to assume gastric involvement in Kaposi's sarcoma patients. Gastric involve- ment is more frequent in patients with oral involvement and in those with acquired immunodeficiency syndrome -associated Kaposi's sarcoma. There was no difference between patients with and without Kaposi's sarcoma in terms of Helicobacter pylori positivity.

Bilateral Tonsillar and Esophageal Kaposi Sarcoma in an HIV-Negative Patient

Tonsillar involvement in Kaposi sarcoma is extremely rare, as only a few such cases have been reported; all but 1 of these previously reported cases occurred in patients with human immunodeficiency virus (HIV) infection. We describe what to the best of our knowledge is the first reported case of concurrent bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. A 68-year-old man presented with sore throat and dysphagia. Clinical examination revealed the presence of bilateral and asymmetrical tonsillar masses, as well as generalized lymphadenopathy in the cervical chain. The masses were resected, and findings on histopathologic analysis were consistent with Kaposi sarcoma. In addition, human herpesvirus 8 was demonstrated on a tonsil specimen by polymerase chain reaction, and microinvasive squamous cell carcinoma was also detected. Later, another Kaposi sarcoma lesion was detected in the lower third of the esophagus. We recommend that clinicians not discount the possibility of oral classic Kaposi sarcoma in the workup of an immunocompetent patient with oral vascular lesions.

Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology

Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent edema or tumor infiltration, and areas of ground-glass attenuation correspond to edema and the filling of air spaces with blood. These findings are a result of the propensity of Kaposi sarcoma to grow in the peribronchial and perivascular axial interstitial spaces, often as continuous sheets of tumor tissue.In conclusion, radiological findings can play a major role in the diagnosis of pulmonary Kaposi sarcoma since characteristic patterns may be observed. The presence of these patterns in patients with AIDS is highly suggestive of Kaposi sarcoma.

Pulmonary Kaposi sarcoma in six children

Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults.

Malignant vascular tumors of the liver: radiologic-pathologic correlation.

Although benign vascular tumors of the liver are extremely common (hemangioma is the most common), malignant vascular tumors of the liver are very rare. In the adult, these tumors are angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma. All hepatic malignant vascular tumors share histologic characteristics, grow around and into vessels, and are grossly multifocal. They may be misdiagnosed histologically, particularly if only a biopsy sample is available. Although imaging findings are often non-specific, some features are suggestive or even characteristic of these neoplasms. Such features include previous exposure to thorium dioxide (Thorotrast) in cases of angiosarcoma, coalescence of multiple nodules into large peripheral masses in epithelioid hemangioendothelioma, and association between acquired immunodeficiency syndrome and cutaneous involvement in Kaposi sarcoma. Because hepatic malignant vascular tumors are often multiple, the main differential diagnosis is metastatic disease. There is no effective treatment for these tumors, and although survival time is variable, the prognosis is generally unfavorable.

Letter: Chemotherapy for Kaposi sarcoma.

To Editor.— I read with great interest article Chemotherapy for Advanced Kaposi Sarcoma in theArchives(111:1331-1333, 1975) by Lanzotti et al. It was a well-written article that detailed combination chemotherapy of advanced Kaposi sarcoma. However, authors stated that their case represented the first verified response of visceral Kaposi sarcoma to chemotherapy reported in literature. I should like to point out that I reported two patients in 1974 ( Chest 66:522-525) who responded to chemotherapy with intravenously administered vinblastine. One patient had marked pulmonary infiltrates and other had pericardial involvement. While treatment was only palliative, both patients still experienced a marked regression of their disease with chemotherapy. Internal involvement in non-African Kaposi sarcoma is being recognized with increasing frequency, and experience with chemotherapy has been limited in this advanced stage of disease. Successful combination chemotherapy for internal involvement in Kaposi sarcoma as described by