Eyelid Kaposi Sarcoma in HIV-Negative Patient

Introduction: Kaposi sarcoma disease is a proliferative and multifocal disorder with dual components, vascular and fibroblastic cellular, cutaneous and visceral expression. Kaposi Sarcoma can affect the ocular surface and adnexa and can masquerade as other entities, delaying prompt diagnosis can lead to diagnostic wandering delaying treatment. Our aim is to describe a case of KS of the eyelid in an HIV seronegative patient. Case Presentation: A seventy-year-old man developed a bilateral growing tumoral reddish purple vascular mass on both the lower and upper eyelid involving rapidly for 6 months. Both feet and the two shanks show the presence of a brown-violet tumor-shaped formation. The patient was negative for HIV. Histology examination showed a nodular tumor-like mass with a fibro hemangioma-epitheliomatous. Polymerase chain reaction was positive for human herpes virus 8. Initial chemotherapy followed by surgery was proposed to the patient. Unfortunately, the patient rejected treatment and was lost to follow-up. Conclusion: This case reports the difficulty of managing KS in developing countries.

Hepatic Kaposi sarcoma: A case report and review of the literature

Kaposi sarcoma(KS) is an aggressive cancer caused by human herpesvirus-8, primarily seen in immunocompromised patients. As opposed to the well-described cutaneous manifestations and pulmonary complications of KS, hepatic KS is rarely reported before death as most patients with hepatic KS do not manifest symptoms or evidence of liver injury. In patients with acquired immune deficiency syndrome, hepatic involvement of KS is present in 12%-24% of the population on incidental imaging and in approximately 35% of patients with cutaneous KS if an autopsy was completed after their death. Patients with clinically significant hepatic injury due to hepatic KS usually have an aggressive course of disease with hepatic failure often progressing to multi-organ failure and death. Here we report an unusual presentation of acute liver injury due to hepatic KS and briefly review the published literature on hepatic KS.

Coexistent Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes after pediatric liver transplantation:A case report

BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph nodes is very rare.CASE SUMMARY We report the case of a 19-mo-old boy,who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation.Six cervical lymph nodes were biopsied,and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels,extravasated erythrocytes,and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes.The immunohistochemical analyses of spindle cells revealed positive expression of CD34,CD31,erythroblast transformation-specific-related gene,friend leukemia integration 1,and human herpesvirus-8.The lymphoproliferative lesions expressed CD38,CD138,and multiple myeloma 1.Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells.Finally,we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder(plasmacytic hyperplasia)in the same lymph nodes.Treatment strategy included anti-CD20 monoclonal antibody(rituximab)and discontinuation of the immunosuppressant therapies.Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia.CONCLUSION The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.

Diagnostic value of endothelial markers and HHV-8 staining in gastrointestinal Kaposi sarcoma and its difference in endoscopic tumor staging

AIM: To clarify the diagnostic values of hematoxylin and eosin (HE), D2-40, CD31, CD34, and HHV-8 immunohistochemical (IHC) staining in gastrointestinal Kaposi's sarcoma (GI-KS) in relation to endoscopic tumor staging. METHODS: Biopsy samples (n = 133) from 41 human immunodeficiency virus-infected patients were reviewed. GI-KS was defined as histologically negative for other GI diseases and as a positive clinical response to KS therapy. The receiver operating characteristic area under the curve (ROC-AUC) was compared in relation to lesion size, GI location, and macroscopic appearances on endoscopy. RESULTS: GI-KS was confirmed in 84 lesions (81.6%). Other endoscopic findings were polyps (n = 9), inflammation (n = 4), malignant lymphoma (n = 4), and condyloma (n = 2), which mimicked GI-KS on endoscopy. ROC-AUC of HE, D2-40, blood vessel markers, and HHV-8 showed results of 0.83, 0.89, 0.80, and 0.82, respectively. For IHC staining, the ROC-AUC of D2-40 was significantly higher (P < 0.05) than that of HE staining only. In the analysis of endoscopic appearance, the ROC-AUC of HE and IHC showed a tendency toward an increase in tumor staging (e.g. , small to large, patches, and polypoid to SMT appearance). D2-40 was significantly (P < 0.05) advantageous in the upper GI tract and for polypoid appearance compared with HE staining. CONCLUSION: The diagnostic value of endothelial markers and HHV-8 staining was found to be high, and its accuracy tended to increase with endoscopic tumor staging. D2-40 will be useful for complementing HE staining in the diagnosis of GI-KS, especially in the upper GI tract and for polypoid appearance.

Kaposi Sarcoma in HIV Positive Nigerian Children: A Case Series

Background: HIV associated KS is relatively rare in children and has been reported to be higher in East Africa compared to other regions. Literature on cases of histologically proven Kaposi sarcoma in children with HIV infection in West Africa is scanty. Case presentation: This communication presents three cases of KS seen among children in a paediatric HIV unit. The first case was an eleven year old HIV positive boy who had oral candidiasis that resolved with treatment and subsequently developed a painless, erythematous swelling at the middle of the dorsum of the tongue with central loss of papillae. He also had multiple discoid hyperpigmented flat lesions on the legs and soles of the feet. In addition to switching to second line antiretroviral therapy, he had chemotherapy. The lesions regressed. The second case was a double orphan who had KS involvement of the right eye, nasal cavity and lymph nodes. The tumour rapidly progressed and the child died before ART and chemotherapy could be commenced. The last case was a five year old girl with mild KS of the skin but also had other manifestations of severe HIV disease which she succumbed to. Incisional biopsies of the lesions revealed an invasive epithelial lined vascular tumour destroying the upper layers of skeletal fibres within the connective tissue stroma in keeping with KS. Human Herpes Virus type 8 (HHV8) screen was also positive for all the cases. Conclusion: A high index of suspicion must be entertained and biopsy of suspicious muco-cutaneous lesions is necessary to exclude a diagnosis of KS which is an indicator of severity and progression of HIV.

Bullous Pemphigoid With Nail Damage Associated With Kaposi Sarcoma:A Case Report

Introduction:Bullous pemphigoid is an acquired autoimmune bullous skin disease that occurs in the elderly,and nail involvement is relatively rare.Kaposi sarcoma(KS) is a rare opportunistic tumor in patients with iatrogenic immunosuppression and is rarely associated with autoimmune bullotic disease.Case presentation:The patient was diagnosed with bullous pemphigoid based on her history,bullous pemphigoid antibody,and skin pathological examination.And nail involvement occurred 20 days prior to the recurrence of the disease.Moreover,KS occurred during high-dose glucocorticoid therapy in this patient.Discussion:The patient was admitted to hospital because of a five-month history of erythema and blisters and a one-month history of nodules and plaques.The diagnosis of bullous pemphigoid was followed by treatment with glucocorticoids and intravenous gamma globulin.During treatment,the disease recurred due to discontinuation of glucocorticoids.There were erythema and blisters all over the body,and purple nodules,plaques appeared in the base of some blisters.Pathological examination of nodules was consistent with KS.In addition,nail involvement occurred 20 days before recurrence.The particularity of this case lies in the simultaneous occurrence of nail involvement,bullous pemphigoid,and KS.Conclusion:Patients with autoimmune bullous skin disease may suddenly develop abnormal nails,and such patients should be vigilant about recurrence of the disease.Doctors should be aware of the possibility of KS when rapidly progressing purplish red plaques or nodules appear in patients receiving high doses of corticosteroids or immunosuppressants.

Genotypic analysis on the ORF-K1 gene of human herpesvirus 8 from patients with Kaposi's sarcoma in Xinjiang,China

Human herpesvirus 8 （HHV-8） is thought to be essential for the development of all forms of Kaposi＇s sarcoma （KS）. HHV-8 DNA is present virtually in all KS tumor biopsy samples. Genes at both ends of the HHV-8 genome have been shown to vary considerably. Seven major molecular subtypes of HHV-8 were defined based on the amino acid sequence of the open reading frame K1 （ORF-K1）, generally known as A, B, C, D, E, F, and Z. Most strains collected worldwide were clustered into two subtypes （A and C）. Here, the K1/VRI region of HHV-8 was amplified by nested PCR in 22 （81.48%） of 27 cases from Xinjiang Uygur Autonomous Region, a province in northwestern China. Phylogenetic analysis on the basis of the K1/VR1 amino acid sequence indicated that the majority of these KS patients were infected by subtype C HHV-8 （n = 18, including 15 belonging to the C2 group）, and several by subtype A （n = 4, including 3 being the A1 group）. This is the first report of subtype A HHV-8 in China. Furthermore, the correlations between different forms and lesions of KS and different subtypes of HHV-8 were analyzed. The findings showed that subtype A HHV-8 resulted in significantly more frequent mucosal KS lesions than subtype C. However, there was no obvious correlation between different forms of KS and different subtypes of HHV-8.

Human herpesvirus-8 positive iatrogenic Kaposi's sarcoma in the setting of refractory ulcerative colitis

Although Kaposi sarcoma(KS) has been more traditionally considered an AIDS-defining illness,it may also be seen in individuals on immunosuppresive therapy.We report a case of a patient who presented to the hospital in the setting of increasingly refractory ulcerative colitis.Computed tomography scan of the abdomen was consistent with sigmoid diverticulititis and blood cultures were positive for Klebsiella.After a course of antibiotics with resolution of infection,a colonoscopy was performed to evaluate his diverticulitis and incidentally revealed a new rectal tumor.Immunohistochemistry showed the tumor was consistent with KS,with cells staining strongly positive for human herpesvirus-8.This case not only illustrates a rare case of KS found in an HIV-negative individual,but it also highlights the importance of considering an alternative diagnosis in a patient refractory to medical treatment.We discuss the management and care of an ulcerative colitis patient diagnosed with KS on immunosuppressive therapy.

Gastrointestinal Kaposi's sarcoma: Case report and review of the literature

Kaposi's sarcoma(KS) of the gastrointestinal tract is not an uncommon disease among individuals with acquired immunodeficiency syndrome(AIDS). The majority is asymptomatic, and for this reason, gastrointestinal KS(GI-KS) remains undiagnosed. With continued tumor growth, considerable variation in clinical presentation occurs including abdominal pain, nausea, vomiting, iron deficiency anemia(either chronic or frank gastrointestinal bleeding), and rarely mechanical obstruction alone or combined with bowel perforation. Endoscopy with biopsy allows for histological and immunohistochemical testing to confirm the diagnosis of GI-KS among those with clinical symptoms. In previous studies, dual treatment with highly active antiretroviral therapy and systemic chemotherapy have been associated with improved morbidity and mortality in individuals with visceral KS. Therefore, investigators have suggested performing screening endoscopies in select patients for early detection and treatment to improve outcome. In this review, we describe a 44 years old man with AIDS and cutaneous KS who presented for evaluation of postprandial abdominal pain, vomiting, and weight loss. On upper endoscopy, an extensive, infiltrative,circumferential, reddish mass involving the entire body and antrum of the stomach was seen. Histologic examination later revealed spindle cell proliferation, and confirmatory immunohistochemical testing revealed human herpes virus 8 latent nuclear antigen expression consistent with a diagnosis of gastric KS. Following this, we present a comprehensive review of literature on KS with emphasis on gastrointestinal tract involvement and management.

Kaposi’s sarcoma manifested as lower gastrointestinal bleeding in a HIV/HBV-co-infected liver cirrhosis patient: A case report

BACKGROUND Kaposi’s sarcoma(KS)is one of the most common cancers in human immunodeficiency virus(HIV)-positive patients and leads to a high prevalence of morbidity and mortality.It usually appears as cutaneous or mucous lesions.Patients with visceral KS are asymptomatic and clinically silent.As the disease advances,patients may progress from a normal condition to exhibiting severe symptoms.CASE SUMMARY A 27-year-old man presented with a 2-mo history of fever,bearing-down pain,and rectal bleeding.His hepatitis B virus DNA level was 2.7×107 IU/mL.Abdominal computed tomography(CT)indicated liver cirrhosis.Before he was admitted to our hospital,he was diagnosed with HIV infection.His CD4 count was 24 cells/μL.Pelvic cavity CT suggested a thickened rectum wall accompanied by multiple enlarged lymph nodes.The patient was initially treated as having haemorrhoidal varices with bleeding,telbivudine for anti-hepatitis B virus treatment,and antibiotics for anti-infection.After half a month of treatment,the patient felt that his lower lumbus ache and bearing-down pain had not improved,and a colonoscopy was conducted.The result revealed a rectal mass that was histologically confirmed as KS with rectal spindle cells that were positive for cluster of differentiation 117(CD117),CD34,human herpes virus 8,and CD31.He was administered systemic chemotherapy with 36 mg/d liposomal doxorubicin six times.The patient experienced no sign of lower gastrointestinal bleeding again.CONCLUSION This case highlights the diagnosis of primary KS with lower gastrointestinal bleeding in HIV-positive patients,which means visceral KS could not be excluded.The gold standard relies on colonoscopy and biopsy findings.

Pathogenesis and Associated Diseases of Kaposi's Sarcoma-associated Herpesvirus

Kaposi's sarcoma-associated herpesvirus(KSHV)is the primary etiological agent of Kaposi's sarcoma,primary effusion lymphoma and muticentric Castleman's disease.In common with the other herpesviruses,KSHV exhibits both latent and lytic life cycles,both of which are characterized by distinct gene expression profiles and programs.KSHV encodes proteins which play essential roles in the inhibition of host adaptive and innate immunity,the inhibition of apoptosis,and the regulation of the cell cycle.KSHV also encodes several proteins which have transforming and intrcellular signalling activity.

The effect of ABV regimen on CD4 lymphocyte count in patients with advanced HIV related Kaposi’s sarcoma

Objective: The combination of highly active antiretroviral therapy (HAART) and chemotherapy with ABV regimen (doxorubicin, bleomycin and vincristine) is a promising approach for the treatment of advanced HIV-related Kaposi's sarcoma (KS). Here we analyzed the relationship between the CD4 lymphocyte cell count and the clinical response to chemotherapy. Methods: The 176 HIV infected patients with advanced KS who failed to respond to prior HAART were selected. All these patients were then preceded to chemotherapy with ABV regimen which was administered at 3 weekly intervals for 6 cycles. For each patient CD4 cell count was done before starting chemotherapy and after finishing 6 cycles of chemotherapy. The difference of CD4 cell counts pre chemotherapy and post chemotherapy was compared with the clinical progress of the patients after 6 cycles of chemotherapy. Results: The overall clinical remission was shown in 93.7% patients. Progressive disease (PD) and no change in clinical condition (NC) was shown in 6.3% patients. The increase in CD4 cell count post chemotherapy was found in 89.8% patients and the decrease in CD4 cell count was seen in 10.2% patients. The difference of the mean CD4 cell counts for patients in group CR + PR (complete relief + partial relief) before and after chemotherapy was highly significant. The difference of the mean CD4 cell counts for patients in group NC + PD before and after chemotherapy was not significant. The difference in CD4 cell counts in CR + PR and NC + PD groups before and after chemotherapy was highly significant. Conclusion: The HIV related KS patients on HAART benefit from the chemotherapy as it increases the CD4 cell count and it has positive impact on clinical remission of KS.

Facial nerve palsy, headache, peripheral neuropathy and Kaposi's sarcoma in an elderly man

We present a case of an elderly man, who initially presented with right facial nerve palsy, ipsilateral headache, elevated erythrocyte sedimentation rate(ESR) and no fever. A presumptive diagnosis of giant cell arteritis was made and the patient was treated with highdose steroids. A temporal artery biopsy was negative. Several months later, while on 16 mg of methylprednisolone daily, he presented with severe sensorimotor peripheral symmetric neuropathy, muscle wasting and inability to walk, uncontrolled blood sugar and psychosis. A work-up for malignancy was initiated with the suspicion of a paraneoplastic process. At the same time a biopsy of the macular skin lesions that had appeared on the skin of the left elbow and right knee almost simultaneously was inconclusive, whereas a repeat biopsy from the same area of the lesions that had become nodular, a month later, was indicative of Kaposi'ssarcoma. Finally, a third biopsy of a similar lesion, after spreading of the skin process, confirmed the diagnosis of Kaposi's sarcoma. He was treated with interferon α and later was seen in very satisfactory condition, with no clinical evidence of neuropathy, normal muscle strength, no headache, normal electrophysiologic nerve studies, involution of Kaposi's lesions and a normal ESR.

Non-AIDS-related Kaposi’s sarcoma:A single-institution experience

AIM:To evaluate the outcomes and potential prognostic factors in patients with non-acquired immunodeficiency syndrome(AIDS)-related Kaposi’s sarcoma(KS).METHODS:Patients with histologically proven non-AIDS-related KS treated with systemic chemotherapy were included in this retrospective analysis.In some cases,the human herpes virus 8 status was assessed by immunohistochemistry.The patients were staged according to the Mediterranean KS staging system.A multivariable model was constructed using a forward stepwise selection procedure.A P value<0.05 was considered statistically significant,and all tests were two-sided.RESULTS:Thirty-two cases were included in this analysis.The average age at diagnosis was 70 years,with a male/female ratio of approximately 2:1.Eighty-four percent of the cases had classic KS.All patients received systemic chemotherapy containing one of the following agents:vinca alkaloid,taxane,and pegylated liposomal doxorubicin.Ten patients(31.5%)experienced a partial response,and a complete response was achieved in four patients(12.4%)and stable disease in sixteen cases(50%).Two patients(6.2%)were refractory to the systemic treatment.The median progression-free survival(PFS)was 11.7 mo,whereas the median overall survival was 28.5 mo.At multivariate analysis,the presence of nodular lesions(vs macular lesions only)was significantly related to a lower PFS(hazard ratio:3.09;95%CI:1.18-8.13,P=0.0133).CONCLUSION:Non-AIDS-related KS appears mostly limited to the skin and is well-responsive to systemic therapies.Our data show that nodular lesions may be associated with a shorter PFS in patients receiving chemotherapy.

Intestinal Kaposi's sarcoma may mimic gastrointestinal stromal tumor in HIV infection

Diffuse intestinal Kaposi＇s sarcoma shares macroscopic and histopathologic features with gastrointestinal stromal tumors. Correct diagnosis may pose a clinical challenge. We describe the case of a young HIV-1-infected African lady without advanced immunodeficiency, who presented with a diffuse spindle cell tumor of the gut. Initial diagnosis was of a gastrointestinal stromal tumor, based on endoscopy and histopathology. Further evaluation revealed evidence for human herpesvirus 8 （HHV8） and the diagnosis had to be changed to diffuse intestinal Kaposi＇s sarcoma. Antiretroviral triple therapy together with chemotherapy was commenced, and has led to the rapid remission of intestinal lesions. With a background of HIV infection, the presence of HHV8 as the causative agent of Kaposi＇s sarcoma should be determined, as distinct treatment is indicated.

Mathematical Model of Classical Kaposi’s Sarcoma

In this paper, the global properties of a classical Kaposi’s sarcoma model are investigated. Lyapunov functions are constructed to establish the global asymptotic stability of the virus free and virus (or infection) present steady states. The model considers the interaction of B and progenitor cells in the presence of HHV-8 virus. And how this interaction ultimately culminates in the development of this cancer. We have proved that if the basic reproduction number, R0 is less than unity, the virus free equilibrium point, ε0, is globally asymptotically stable (GAS). We further show that if R0 is greater than unity, then both the immune absent and infection persistent steady states are GAS.

Angiogenesis,Kaposi’s Sarcoma and Kaposi’s Sarcoma-associated Herpesvirus

Tumor angiogenesis is the uncontrolled growth of blood vessels in tumors,serving to supply nutrients and oxygen,and remove metabolic wastes. Kaposi’s sarcoma (KS),a multifocal angioproliferative disorder characterized by spindle cell proliferation,neo-angiogenesis,inflammation,and edema,is associated with infection by Kaposi's sarcoma-associated herpesvirus (KSHV). Recent studies indicate that KSHV infection directly promotes angiogenesis and inflammation through an autocrine and paracrine mechanism by inducing pro-angiogenic and pro-inflammatory cytokines. Many of these cytokines are also expressed in KS lesions,implicating a direct role of KSHV in the pathogenesis of this malignancy. Several KSHV genes are involved in KSHV-induced angiogenesis. These studies have provided insights into the pathogenesis of KS,and identified potential therapeutic targets for this malignancy.

Splenic Kaposi’s sarcoma in a human immunodeficiency virusnegative patient:A case report

BACKGROUND Kaposi’s sarcoma(KS)is a malignancy that usually affects the skin of the lower extremities,and may involve internal organs.It originates from the vascular endothelium.It is well known that the development of KS is associated with human herpes virus 8(i.e.HHV8)infections.Sporadic KS cases have mainly been found in Africa.Isolated splenic KS in Asia has rarely been reported.We present here a case of KS primarily involving the spleen in a human immunodeficiency virus(HIV)-negative Chinese patient.CASE SUMMARY A 50-year-old male patient was admitted to hospital due to abdominal distension and discomfort,reduced food intake and weight loss.Medical examination revealed that the patient had moderate anemia,a low platelet count,slight fatty liver and a huge mass in the spleen.Spleen lymphoma was considered.An anti-HIV test was negative.The whole spleen was surgically excised.The final pathological diagnosis was nodular stage spleen KS,and the patient underwent total splenectomy.He recovered well and was discharged from hospital 12 d after surgery.Two weeks later,the patient developed liver metastasis and died within 1 mo after surgery.CONCLUSION KS is difficult to diagnose and pathological examination is necessary.KS has a poor prognosis and should be diagnosed and treated early to improve survival.

Kaposi’s Sarcoma Associated with Gastrointestinal Bleeding: A Rare Complication of Long-Term Corticosteroid Therapy

Corticosteroid therapy has revolutionized the treatment of many diseases in medicine. The most concerned diseases are chronic inflammatory diseases. Its use may be either short-term or long-term, thus generating several side effects, some of which are conventional and known, but others are rare, such as Kaposi’s sarcoma. We report here a particular clinical observation. Observation: This is a patient of 37 years, who consults in Internal Medicine for the treatment of mucocutaneous lesions, papular nodular, hyper pigmented, budding and disseminated appeared after six months of corticosteroid therapy. This treatment was introduced for the treatment of acoustic neurinoma. The patient is not diabetic or alcoholic and has a negative HIV status. The diagnosis of Kaposi’s sarcoma was made by histology of biopsied skin lesions which concluded for a regular proliferation without atypia spindle cell or mitosis. The lesions persisted over a month despite discontinuation of corticosteroid therapy. Treatment with Bleomycin was necessary. The outcome was favorable under treatment with progressive and complete disappearance of lesions after 15 treatments. Conclusion: The classical side effects of long-term corticosteroid are known, but Kaposi sarcoma complicating long-term corticosteroid is rare in the literature. It is vital that any long-term corticosteroid should be the subject of careful and regular monitoring.

Treatment of Kaposi’s Sarcoma by Combination of Zinc Sulfate and Propranolol

Kaposi’s sarcoma (KS) in Iraq has been reported as sporadic cases of elderly of Iraqi population but after exposure to depleted uranium radiation in early 1991, the frequency of the disease is increasing appearing in younger age with more wide spread and aggressive in nature. There is no satisfactory oral treatment to control this disease. The aim of the present work is to record a new regime of therapy using oral zinc sulfate and oral propanolol. Four patients with wide spread Kaposi’s sarcoma (KS) were treated with oral and topical zinc sulfate solution and oral propranolol. The duration of treatment was between 6 - 12 months and the response started few weeks and was obvious after one month. Most old lesions were resolved leaving post inflammatory hyperpigmentation and few lesions appeared during course of treatment.