Kaposi's Sarcoma. A Case Report.

AIM: The aim of this case report is to present the case of a patient with iatrogenic Kaposi's sarcoma afflicting several organs, ocular manifestation. CASE REPORT: In a 74-year-old kidney transplant patient receiving immunosuppressive therapy, iatrogenic Kaposi's sarcoma (KS) developed in both lower eyelids. Subsequently, KS was confirmed in the region of the left forearm, with suspicion of lesions in the lungs. The ocular tumor was surgically removed with negative margins, requiring no further therapy. The lesion on the left forearm was completely excised. The patient underwent radiotherapy for the lung lesions, and immunosuppressive therapy was reduced. CONCLUSION: The case highlights the importance of early identification of KS, its histological verification, radical resection, and multidisciplinary collaboration. Knowledge of the epidemiology of this condition is a key factor in determining the correct diagnosis.

Lymphedema and Kaposi sarcoma: A narrative review.

BACKGROUND: Kaposi sarcoma (KS), due to HHV-8 infection is classified in 4 subtypes: epidemic, endemic, HIV-related and iatrogenic essentially after organ transplant. Lymphedema is a complication of KS. We reviewed the interactions between HHV-8 infection and lymphedema according an analysis of the literature. MAIN BODY: HHV-8 can infect different types of cells, among them a privileged tropism for lymphatic endothelial cells. It induces multi-centric endothelial proliferation leading to the occlusion of lymphatic vascular lumen. Lymphatic obstruction progressively lead to the blockage of lymphatic drainage, lymph stasis and lymphedema. Lymphedema mostly involved the lower limb affected by KS. It can then develop simultaneously or after the appearance of KS lesions but also be the first sign of KS, a long time before KS skin lesion onset. Lymphedema diagnosis is clinical and lymphoscintigraphy can confirm it if necessary. Lymphedema may be associated with active lesions of KS or non-evolutive, with only cicatricial lesions. KS should be treated according to the KS subtype, aggressive form, with local or systemic treatments associating with causal treatment, such as HIV infection or reducing immuno-suppressive drugs in transplant patients. In most of the cases, KS treatment may slightly reduce (or not) lymphedema volume which remains a chronic disease. Lymphedema management should be associated in order to reduce the volume and then stabilizing it. Low-stretch bandage, elastic garments and skin care are the cornerstone of treatment. CONCLUSION: Lymphedema is a frequent complication of KS, and may reveal KS or occurs throughout its course. Association of KS and lymphedema must be known because lymphedema is a chronic disease affecting the quality of life. Beyond the treatment of KS, its management must be specific including a long follow-up to optimize the patient's observance required to maintain the best lymphedema control.

Rare solid tumors in a patient with Wiskott-Aldrich syndrome after hematopoietic stem cell transplantation: case report and review of literature.

Background and aims: Wiskott-Aldrich syndrome (WAS) is an X-linked recessive primary immunodeficiency disorder characterized by severe eczema, recurrent infections, and micro-thrombocytopenia. Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative therapeutic option for patients with classic form. The risk of developing post-transplant tumors appears to be higher in patients with WAS than in other inborn errors of immunity (IEIs), but the actual incidence is not well defined, due to the scarcity of published data. Methods: Herein, we describe a 10-year-old patient diagnosed with WAS, treated with HSCT in the first year of life, who subsequently developed two rare solid tumors, kaposiform hemangioendothelioma and desmoid tumor. A review of the literature on post-HSCT tumors in WAS patients has been performed. Results: The patient received diagnosis of classic WAS at the age of 2 months (Zhu score = 3), confirmed by WAS gene sequencing, which detected the nonsense hemizygous c.37C>T (Arg13X) mutation. At 9 months, patient underwent HSCT from a matched unrelated donor with an adequate immune reconstitution, characterized by normal lymphocyte subpopulations and mitogen proliferation tests. Platelet count significantly increased, even though platelet count never reached reference values. A mixed chimerism was also detected, with a residual WASP- population on monocytes (27.3%). The patient developed a kaposiform hemangioendothelioma at the age of 5. A second abdominal tumor was identified, histologically classified as a desmoid tumor when he reached the age of 10 years. Both hematopoietic and solid tumors were identified in long-term WAS survivors after HSCT. Conclusion: Here, we describe the case of a patient with WAS who developed two rare solid tumors after HSCT. An active surveillance program for the risk of tumors is necessary in the long-term follow-up of post-HSCT WAS patients.

Kaposi's Sarcoma in Children After Hematopoietic Stem Cell Transplantation: Two Cases of Rare Primary Tumor Localizations in the Lungs and Lymph Node.

Kaposi's sarcoma (KS) is a vascular / mesenchymal tumor with an indefinite degree of malignancy, caused by complex etiopathogenetic factors including Human Herpes Virus-8 infection of immunocompromised patients. For example, KS is more common in adult men with HIV. We describe 2 very rare cases of iatrogenic KS in children after hematopoietic stem cell transplant with isolated organ damage (case 1: lung; case 2: inguinal lymph node). KS is a potential complication of bone marrow transplant in pediatric patients and can occur in different age groups and at atypical sites.

Epidemiological profile of cancer at the laboratory of anatomy and pathological cytology of mungbere in the Democratic Republic of Congo.

INTRODUCTION: Cancer is the second leading cause of death worldwide, causing about 10 million deaths per year, 70 % of which occur in low- and middle-income countries. In the DRC, the absence of a national cancer registry is a handicap to the definition of a strategy to combat this disease. The purpose of this study is to establish an epidemiological profile of cancer in this laboratory in order to overcome this deficit in this part of the country. METHODOLOGY: We conducted a descriptive study of 1636 histopathological analysis reports from 2015 to 2021 at the Anatomy and Pathological Cytology Laboratory of Anualite Hospital in Mungbere. RESULTS: A total of 502 cases of cancer have been identified; female accounted for 51.4 % of cases; all age groups are affected; The most common cancers in both sexes are Kaposi's sarcoma (17.9 %), breast cancer (15.3 %), lymphoma (13.7 %), cervical cancer (9.6 %) and squamous cell carcinoma of the skin (9 %). In women, breast cancer (27.1 %), cervical cancer (18.6 %), Kaposi's sarcoma (10.1 %), lymphoma (7.4 %) and squamous cell carcinoma of the skin (5.8 %) and in men Kaposi's sarcoma (26.2 %), lymphoma (20.5 %), liver cancer (13.1 %)) and squamous cell carcinoma of the skin (12.3 %). CONCLUSION: Cancer affects all age groups with a slight female predominance. The most common in both sexes are Kaposi's sarcoma, breast cancer, lymphoma, cervical cancer and squamous cell carcinoma of the skin. For an effective fight against cancer, the creation of a national cancer registry is an emergency in our country.

Disseminated visceral Kaposi's sarcoma after allogeneic hematopoietic stem cell transplantation: a case report.

Cases of disseminated visceral Kaposi's sarcoma (KS) after allogenic hematopoietic stem cell transplantation (HSCT) are very rare worldwide, and disseminated visceral KS is often rapidly progressive and life-threatening, especially in paediatric patients. Here, the case of a 6-year-old female patient with disseminated visceral KS after allogeneic HSCT for treating severe aplastic anaemia is presented. The authors encountered difficulties in making the diagnosis due to lack of experience, but the diagnosis was achieved relatively quickly and accurately using metagenomic next-generation sequencing. After tapering and withdrawal of immunosuppressant drugs, the patient's condition was controlled. In conclusion, although HSCT-related KS is very rare, it should be considered during differential diagnosis.

Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis.

BACKGROUND: The taxonomy of Kaposi Sarcoma (KS) is based on a classification system focused on the description of clinicopathological features of KS in geographically and clinically diverse populations. The classification includes classic, endemic, epidemic/HIV associated and iatrogenic KS, and KS in men who have sex with men (MSM). We assessed the medical relevance of the current classification of KS and sought clinically useful improvements in KS taxonomy. METHODS: We reviewed the demographic and clinicopathological features of 676 patients with KS, who were referred to the national centre for HIV oncology at Chelsea Westminster hospital between 2000 and 2021. RESULTS: Demographic differences between the different subtypes of KS exist as tautological findings of the current classification system. However, no definitive differences in clinicopathological, virological or immunological parameters at presentation could be demonstrated between the classic, endemic or MSM KS patients. Reclassifying patients as either immunosuppressed or non-immunosuppressed, showed that the immunosuppressed group had a significantly higher proportion of adverse disease features at presentation including visceral disease and extensive oral involvement, classified together as advanced disease (chi2 P = 0.0012*) and disseminated skin involvement (chi2 P < 0.0001*). Immunosuppressed patients had lower CD4 counts, higher CD8 counts and a trend towards higher HHV8 levels compared to non-immunosuppressed patients, however overall survival and disease specific (KS) survival was similar across groups. CONCLUSION: The current system of KS classification does not reflect meaningful differences in clinicopathological presentation or disease pathogenesis. Reclassification of patients based on the presence or absence of immunosuppression is a more clinically meaningful system that may influence therapeutic approaches to KS.

Kaposi sarcoma at the base of the tongue in a renal transplant patient.

Oral Kaposi Sarcoma (OKS) commonly occurs in patients with AIDS. The incidence of Kaposi sarcoma (KS) is greatly increased in renal transplant recipients compared with the general population, with particular prevalence in certain ethnic groups where it can occur in up to 5% of transplant recipients. From them, only 2% can manifest first with OKS.A man in his early 40s, 2 years after kidney transplantation, presented with a reddish-purple hypertrophic ulcerated lesion at the base of the tongue. Cervical ultrasonography revealed enlarged lymph nodes, and pathological examination of biopsies revealed KS. The patient had HIV-negative status. Following an investigation, calcineurin inhibitor treatment was stopped, and an mTOR (mammalian target of rapamycin) inhibitor treatment was started. Fibreoptic examination 3 months after beginning mTOR inhibitor treatment revealed no traces of the disease in the base of the tongue.An isolated oral lesion should not distract clinicians from further systemic investigation for metastatic disease.OKS is a rare but serious complication in kidney transplant patients after receiving calcineurin inhibitor that could result in airway obstruction due to mass effect or bleeding and aspiration.Early diagnosis and management of OKS in a renal transplant patient who received a calcineurin inhibitor carry a good prognosis. OKS can be managed by changing the treatment regime to an mTOR inhibitor followed by radiation therapy. This contrasts with KS treatment in non-renal transplant patients without calcineurin inhibitors who may need treatment using different modalities such as surgery and chemotherapy.We emphasise the importance of this case for nephrologists responsible for patient follow-up after renal transplantation who prescribed calcineurin inhibitors. These patients must be advised that if they feel any physical mass in the tongue, they should immediately seek an examination by an ear, nose and throat specialist. Nephrologists and patients should be aware that these symptoms should not be underestimated.

Electrochemotherapy with bleomycin as an effective local treatment for Kaposi's sarcoma: a case report.

An elderly female patient with a long-standing history of Kaposi's sarcoma of the lower limbs was referred to the Surgical Department after the subsequential failure of multiple lines of systemic chemotherapy. The patient was also complaining of increasing symptoms including intractable pruritus, which negatively impacted her quality of life. She underwent palliative electrochemotherapy with bleomycin (15 g/m 2 ) on the sarcomatous lesions of the left foot and ankle, which lead to complete clinical response and resolution of symptoms; no adverse events were reported. Electrochemotherapy is a valid option in the palliative treatment of Kaposi's sarcoma, as it may lead to satisfactory clinical response and symptom control.

Kaposi sarcoma in three pediatric liver transplantation recipients.

BACKGROUND: Kaposi sarcoma (KS) is an endothelial cell tumor, rare in children. It is 200 times more frequent after solid organ transplantation than in the general population. METHODS: We report three cases of pediatric patients who developed KS after liver transplantation (LT). RESULTS: Case 1, a 4-year-old boy undergoing LT due to familial intrahepatic cholestasis. Five months after LT, he presented with fever, dyspnea, and cough with enlarged lymph nodes and splenomegaly, anemia, thrombocytopenia, elevated liver enzymes, and positive EBV viral load. Lymph node biopsy diagnosed KS with an elevated HHV8 viral load. Case 2, a 4-year-old boy who underwent LT due to secondary biliary cirrhosis resulting from extrahepatic biliary atresia. Two years later, graft dysfunction was noticed with positive EBV viral load, thrombocytopenia, massive cervical lymph node enlargement, and splenomegaly. Lymph node biopsy diagnosed KS, Castleman's disease, and plasmablastic lymphoma related to HHV8 infection. Case 3, a 15-month-old girl, who received two LT due to biliary cirrhosis. Six months later, she presented with diarrhea, abdominal distension, anemia, thrombocytopenia, enlarged lymph nodes, splenomegaly, and positive CMV viral load. Axillary lymph node biopsy diagnosed KS and HHV8 infection was confirmed. In all three cases, tacrolimus was discontinued and, after diagnosis, sirolimus was started. All recovered without relapse and have a good graft function. CONCLUSIONS: Kaposi sarcoma is a rare disease post-LT in children. Recognizing keywords and early diagnosis is crucial for timely treatment and survival.

Gastrointestinal Kaposi Sarcoma Involving Stomach and Colon: Diagnostic Pitfall for Pathologists with Expression of CD117.

Kaposi sarcoma (KS) is a low-grade vascular tumor caused by human herpes virus type 8 (HHV8). Gastrointestinal involvement of KS is rare and most commonly clinically silent. Gastrointestinal KS may mimic gastrointestinal stromal tumors (GISTs) histologically as the tumor formed by morphologically spindle-shaped cells, which is mostly located in the mucosa and submucosa. In the present study, we describe a case of Kaposi sarcoma that was first diagnosed in the gastrointestinal tract of a 73-year-old female patient who presented to the clinic with nausea and diarrhea. Immunohistochemical staining showed cytoplasmic CD117 expression both in stomach and colon biopsies. Although involvement of KS is rarely seen in the gastrointestinal tract (GIT), the differential diagnosis of low-grade spindle cell lesions without significant pleomorphism, KS should definitely be considered, and it should be known that CD117 positivity is also present in these neoplasms.

Cutaneous and subcutaneous Kaposi's sarcoma lesions treated with electrochemotherapy.

BACKGROUND AND OBJECTIVES: Kaposi's sarcoma (KS) is a locally aggressive mesenchymal tumor that involves the lymphovascular system, with a tendency to become multifocal. Electrochemotherapy (ECT) is considered a valuable treatment option in selected patients with cutaneous and subcutaneous KS lesions. METHODS: We report a retrospective study that included 14 classic and endemic KS patients that underwent ECT sessions for the treatment of KS cutaneous and subcutaneous lesions at our institution. RESULTS: According to Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 criteria, our patients had an overall response rate (ORR) of 100% to the ECT treatment. A complete response (CR) was obtained in 92.8% of patients after one or more ECT sessions. Only one patient had a progressive disease (PD). The treatment was well tolerated with a low complication rate, mainly transitory local pain or skin ulceration. CONCLUSIONS: ECT represents a locoregional therapy for containment and symptomatic control of classic and endemic KS cutaneous and subcutaneous lesions. Further studies including different subtypes of KS patients should also be performed.

CE: HIV-Associated Kaposi Sarcoma in the Combination Antiretroviral Therapy Era.

ABSTRACT: Kaposi sarcoma is a tumor caused by Kaposi sarcoma herpesvirus, also known as human herpesvirus 8. Its occurrence is associated with an immunocompromised state. Kaposi sarcoma that occurs among people living with HIV (PLWH) is known as epidemic Kaposi sarcoma. Despite the decline in HIV-associated complications because of the introduction of combination antiretroviral therapy two decades ago, Kaposi sarcoma continues to affect PLWH worldwide. It affects young African American men more than other age and racial groups and can result in multiorgan dysfunction, leading to short-term and chronic debilitating symptoms as well as death. While some patients with epidemic Kaposi sarcoma are managed as outpatients, others may require higher levels of care and their acuity may fluctuate throughout their life span. Therefore, nurses, regardless of their specialty, may experience caring for a patient with epidemic Kaposi sarcoma at some point in their career. Learning about this condition and the needs of patients who have it will help nurses provide effective care. Here, the authors describe Kaposi sarcoma in general as well as the epidemiology, characteristics, and management of epidemic Kaposi sarcoma. They also describe specific nursing considerations in the care of PLWH who have the disease.

Gastroduodenal nodules in a HIV positive patient: don't forget the skin!

Question: A 35-year-old male with a history of HIV infection presented in our department for endoscopy with the complaints of dyspepsia and epigastric pain. Endoscopy revealed flat, maculopapular, reddish or purplish patchy nodular lesions, with different sizes and shapes, involv- ing both the duodenum and stomach (Figure 1 A-B). There was no sign of complications such as hemorrhage, perforation or obstruction. Physical examination re- vealed that the patient had also purple patchy cutaneous lesions (Figure 2). What is the diagnosis? Answer: Histological assessment from the maculopapular and nodular lesions in endoscopic and cutaneous biopsies revealed the diagnosis as Kaposi sarcoma (KS). KS is a low-grade vascular tumor caused by human herpes virus type 8. KS manifests primarily as a cutaneous disorder, with visceral involvement considered to occur subsequently. Gastrointestinal involvement of KS is rare and most commonly clinically silent. AIDS-related KS that is the most common form of KS in the USA and Europe and the most common malignancy in patients with AIDS. GI involvement by KS is a rare endoscopic finding, still scarcely characterized in the literature (1). In conclusion, involvement of the gastrointestinal tract by KS is often asymptomatic, has multiple endoscopic appearances, and a high diagnostic suspicion is needed in this setting.

The role of infections in the causation of cancer in Kenya.

Cancer constitutes a major health care burden in the world today with the situation worsening in resource poor settings as seen in most Sub-Saharan African (SSA) countries. Infections constitute by far the most common risk factors for cancer in SSA and being a typical country in this region, Kenya has experienced an upsurge in the incidence of various types of cancers in the last few decades. Although there is limited population-based data in Kenya of infections-associated cancers, this review provides an up-to-date literature-based discussion on infections-associated cancers, their pathogenesis, and preventive approaches in the country. The primary infectious agents identified are largely viral (human immunodeficiency virus, human papillomavirus (HPV), Kaposi's sarcoma-associated herpes virus, Epstein-Barr virus, hepatitis B virus (HBV), hepatitis C virus), and also bacterial: Helicobacter pylori and parasitic: Schistosomiasis haematobium. Cancers associated with infections in Kenya are varied but the predominant ones are Non-Hodgkin lymphoma, Kaposi's sarcoma, Hodgkin lymphoma, Burkitt's lymphoma, cervical, liver, and gastric cancers. The mechanisms of infections-induced carcinogenesis are varied but they mainly seem to stem from disruption of signaling, chronic inflammation, and immunosuppression. Based on our findings, actionable cancer-preventive measures that are economically feasible and aligned with existing infrastructure in Kenya include screening and treatment of infections, implementation of cancer awareness and screening, and vaccination against infections primarily HBV and HPV. The development of vaccines against other infectious agents associated with causation of cancer remains also as an important goal in cancer prevention.

Oral and maxillofacial manifestations of human immunodefficiency virus infection.

Oral manifestations occur at all stages of human immunodeficiency virus (HIV) infection. Their clinical expressions and severity depend on the evolution of the infection and become critical at the stage of acquired immunodeficiency (AIDS). They are essentially infectious, tumoral, and, starting a few years ago, iatrogenic. Infections are mostly fungal (candidiasis), viral (herpes, zoster, human papillomavirus infections, etc.), and less frequently bacterial (streptococcemia). Cases of sexually transmitted diseases, particularly syphilis, are multiplying preoccupyingly. The most frequently observed tumors are Kaposi's sarcoma. Drug intolerance is common; the symptoms are mostly dermatological, but also oral (Stevens-Johnson syndrome and toxic epidermal necrolysis) when sulfonamides and certain antiretrovirals are used. The advent of prolonged Highly Active Anti-Retroviral Therapies (HAARTs) has led to a decline in the incidence of most opportunistic conditions, infections and tumors (except for multiple oral warts and zoster). HAARTs also provoke adverse reactions such as lipodystrophy syndromes (signs of peripheral atrophy and central hypertrophy, associated in varying degrees with metabolic syndromes). Extended survival and the new methods for prophylaxis of opportunistic infections have gradually modified diagnostic and therapeutic strategies for oral manifestations of HIV infections.

Epidemiology of Kaposi's sarcoma in sub-Saharan Africa.

Kaposi's sarcoma (KS) has become a common AIDS-defining cancer in sub-Saharan Africa. Kaposi's sarcoma-associated human herpesvirus strongly modulated by HIV-related immune suppression are the principal causes of this cancer. No other risk factors have been identified as playing a strong role. HIV prevention programs and good coverage of antiretroviral therapy (ART) in developed countries resulted in a remarkable decline in HIV-KS incidence and better KS prognosis. By contrast, in sub-Saharan Africa, population ART rollout has lagged, but clinical studies have shown positive results in reduction of KS incidence and better KS prognosis. However, the effect of ART rollout in relation to population KS incidence is unclear. We describe the incidence of KS in sub-Saharan Africa, in four time-periods, (1) before 1980 (before HIV/AIDS era); (2) 1981-2000 (early HIV/AIDS era, limited or no ART coverage); (3) 2001-2010 (early ART coverage period); and (4) 2011-2016 (fair to good ART coverage period). We used KS incidence data available from WHO-International Agency for Research on Cancer (IARC) publications and the Africa Cancer Registry Network. National HIV prevalence and ART coverage data were derived from UNAIDS/WHO. A rapid increase in KS incidence was observed throughout sub-Saharan Africa as the HIV epidemic progressed, reaching peak incidences in Period 2 (pre-ART rollout) of 50.8 in males and 20.3 per 100 000 in females (Zimbabwe, Harare). The overall unweighted average decline in KS incidence between 2000 and 2010 and 2011-2016 was 27%, but this decline was not statistically significant across the region. ART rollout coincides with a decline in KS incidence across several regions in sub-Saharan Africa. The importance of other risk factors such as reductions in HIV incidence could not be ascertained.

Gastrointestinal Manifestations of Immunodeficiency: Imaging Spectrum.

There is a wide spectrum of hereditary and acquired immunodeficiency disorders that are characterized by specific abnormalities involving a plethora of humoral, cellular, and phagocytic immunologic pathways. These include distinctive primary immunodeficiency syndromes due to characteristic genetic defects and secondary immunodeficiency syndromes, such as AIDS from HIV infection and therapy-related immunosuppression in patients with cancers or a solid organ or stem cell transplant. The gut mucosa and gut-associated lymphoid tissue (the largest lymphoid organ in the body), along with diverse commensal microbiota, play complex and critical roles in development and modulation of the immune system. Thus, myriad gastrointestinal (GI) symptoms are common in immunocompromised patients and may be due to inflammatory conditions (graft versus host disease, neutropenic enterocolitis, or HIV-related proctocolitis), opportunistic infections (viral, bacterial, fungal, or protozoal), or malignancies (Kaposi sarcoma, lymphoma, posttransplant lymphoproliferative disorder, or anal cancer). GI tract involvement in immunodeficient patients contributes to significant morbidity and mortality. Along with endoscopy and histopathologic evaluation, imaging plays an integral role in detection, localization, characterization, and distinction of GI tract manifestations of various immunodeficiency syndromes and their complications. Select disorders demonstrate characteristic findings at fluoroscopy, CT, US, and MRI that permit timely and accurate diagnosis. While neutropenic enterocolitis affects the terminal ileum and right colon and occurs in patients receiving chemotherapy for hematologic malignancies, Kaposi sarcoma commonly manifests as bull's-eye lesions in the stomach and duodenum. Imaging is invaluable in treatment follow-up and long-term surveillance as well. Online supplemental material is available for this article. ©RSNA, 2022.