Isolated limb perfusion is an effective treatment modality for locally advanced Kaposi sarcoma of the extremities.

INTRODUCTION: Kaposi sarcoma (KS) is a rare soft tissue sarcoma. In case of locally advanced disease, mutilating surgery such as amputations or major reconstructive procedures are sometimes inevitable. The aim of this study was to evaluate the effectiveness of isolated limb perfusion (ILP) in patients with locally advanced KS of the extremities. MATERIAL AND METHODS: All patients who underwent ILP for KS between 1996 and 2018 at Erasmus MC, Rotterdam were identified. Clinical data was obtained from either a prospectively maintained database or retrospective assessment of patient files. RESULTS: A total of 14 primary ILP's were performed in 11 patients. Median follow-up from primary ILP was 30 months (range, 5-98). The overall response rate of primary ILP was 100%, with a complete response (CR) rate of 50%. Only minimal local toxicity (Wieberdink I-III) was observed. Local progressive disease occurred after eight primary ILP's (57%) with a median local progression free survival (PFS) of 18 months (95% confidence interval [CI]: 7.0-28.9). Subsequently, four (46%) patients received a total of 5 recurrent ILP's. After the recurrent ILP on the same leg, the overall response rate was 75% and a CR-rate of 50%. One patient needed amputation post-operatively resulting in a limb salvage rate of 91%. One (9%) patient developed metastases four months after ILP. CONCLUSIONS: ILP is a highly effective treatment modality with very limited morbidity rates for patients with locally advanced KS of the extremity. ILP should be considered as a treatment modality for locally advanced KS of the extremities.

Clinical and endoscopic findings of patients with cutaneous Kaposi sarcomaand gastrointestinal involvement. Experience in a single center of Lima-Peruin the last 3 years.

INTRODUCTION: Kaposi sarcoma is a low-grade angioproliferative neoplasm strongly associated with infection by herpes virus type 8 (HHV-8). Gastrointestinal (GI) involvement is an infrequent finding, whose clinical and endoscopic characteristics are poorly defined in the literature. OBJECTIVE: The aim of our study was to describe the clinical and endoscopic findings of patients with gastrointestinal Kaposi Sarcoma. MATERIALS AND METHODS: We reviewed all clinical histories, endoscopic and anatomopathologic reports of all patients with cutaneous Kaposi sarcoma (CKS) who came to Cayetano Heredia Gastroenterology Service during the period between August 2015 to October 2018. We included all patients with CKS that had gastrointestinal involvement confirmed with biopsy. RESULTS: We found 50 patients with cutaneous Kaposi sarcoma. Thirteen patients had gastrointestinal Kaposi sarcoma (26%). 53.8% (7/13 cases) were asymptomatic. 92.3% (12/13 cases) had HIV infection. Nine of the twelve HIV+ patients had CD4 count below 200 cells/µl. When Kaposi affects GI tract, the mayority have multiple GI organs affected. Stomach and colon are the most common sites affected. CONCLUSION: Gastrointestinal involvement was presented in 26% of our patients with cutaneos Kaposi sarcoma, a half of them had no GI symptoms. The majority of cases were young male and had HIV in AIDS stage. The mortality in our series was 15.3% at 6 months of follow-up.

Clinical and endoscopic findings of patients with cutaneous Kaposi sarcoma and gastrointestinal involvement: Experience in a single center of Lima-Peru in the last 3 years / Hallazgos clínicos y endoscópicos en pacientes con sarcoma de Kaposi cutáneo y compromiso gastrointestinal: Experiencia de un centro de Lima - Perú en los últimos 3 años

Introduction: Kaposi sarcoma is a low-grade angioproliferative neoplasm strongly associated with infection by herpes virus type 8 (HHV-8). Gastrointestinal (GI) involvement is an infrequent finding, whose clinical and endoscopic characteristics are poorly defined in the literature. Objective: The aim of our study was to describe the clinical and endoscopic findings of patients with gastrointestinal Kaposi Sarcoma. Materials and methods: We reviewed all clinical histories, endoscopic and anatomopathologic reports of all patients with cutaneous Kaposi sarcoma (CKS) who came to Cayetano Heredia Gastroenterology Service during the period between August 2015 to October 2018. We included all patients with CKS that had gastrointestinal involvement confirmed with biopsy. Results: We found 50 patients with cutaneous Kaposi sarcoma. Thirteen patients had gastrointestinal Kaposi sarcoma (26%). 53.8% (7/13 cases) were asymptomatic. 92.3% (12/13 cases) had HIV infection. Nine of the twelve HIV+ patients had CD4 count below 200 cells/μl. When Kaposi affects GI tract, the mayority have multiple GI organs affected. Stomach and colon are the most common sites affected. Conclusion: Gastrointestinal involvement was presented in 26% of our patients with cutaneos Kaposi sarcoma, a half of them had no GI symptoms. The majority of cases were young male and had HIV in AIDS stage. The mortality in our series was 15.3% at 6 months of follow-up.

Introducción: El Sarcoma de Kaposi es una neoplasia angioproliferativa de bajo grado altamente asociada con la presencia del herpes virus tipo 8 (HHV-8). El compromiso gastrointestinal es un hallazgo infrecuente, cuyas características clínicas y endoscópicas son pobremente descritas en la literatura. Objetivos: El objetivo del estudio fue describir las características clínicas y endoscópicas de pacientes con Sarcoma de Kaposi gastrointestinal. Materiales y métodos: Nosotros revisamos todas las historias clínicas, reportes endoscópicos y anatomo patológicos de todos los pacientes con Sarcoma de Kaposi cutáneo que fueron al Servicio de Gastroenterología del Hospital Cayetano Heredia durante el periodo de Agosto del 2015 a Octubre del 2018. Se incluyeron todos los pacientes con SKC que tuvieron compromiso gastrointestinal confirmado en la biopsia. Resultados: Nosotros encontramos 50 pacientes con Sarcoma de Kaposi cutáneo. 13 pacientes tuvieron compromiso gastrointestinal (26%). 53.8% (7/13) fueron asintomáticos. 92.3% (12/13 casos) tuvieron infección con virus de VIH. Nueve de trece pacientes con VIH+ tuvieron conteos de CD4 menores de 200 cel/μl. Cuando el Kaposi afectaba el aparato digestivo, la mayoría tenía compromiso de múltiples segmentos. El estómago y el colon eran los lugares más comprometidos. Conclusión: El compromiso gastrointestinal se presentó en 26% de los pacientes con Sarcoma de Kaposi cutáneo, la mitad de ellos no tenían síntomas digestivos. La mayoría de los casos fueron varones jóvenes y tenían infección por VIH estadío SIDA. La mortalidad en nuestra serie fue 15.3% a los 6 meses de seguimiento.

Radiotherapy in the management of classic Kaposi's sarcoma: A single institution experience from Northeast Turkey.

Kaposi sarcoma is a rare adult neoplasm and there has been no certain consensus on standard treatment, either local or systemic. Radiotherapy is an effective, suitable treatment modality. Between 1996 and 2016, patients who were diagnosed with Kaposi sarcoma and referred to our clinic for radiotherapy were included in this retrospective study. Ninety-two patients were examined in total and it was diagnosed that all the patients had non-HIV associated Kaposi's sarcoma. There were 36 (39%) females and 56 (61%) males and female to male ratio was 2/3. Median age at presentation was 72 (30-93) years. Sixty-eight patients (77%) were treated with 8 Gy (1 fraction), 15 patients (16%) were treated with 20 Gy (2 Gy/fraction), four patients (4%) were treated 25 Gy (2.5 Gy/fraction), and five patients (6%) were treated 30 Gy (3 Gy/fraction). The median follow-up time was 72 (5-192) months. The complete response at 5 years was 91.6% with >20 Gy and 89.6% with 8 Gy. Radiotherapy is an effective, suitable treatment modality of Classic Kaposi sarcoma and usually, radiotherapy is well tolerated with minimal side effects.

Pulmonary Kaposi Sarcoma with Osseous Metastases in an Human Immunodeficiency Virus (HIV) Patient: A Remarkable Response to Highly Active Antiretroviral Therapy.

BACKGROUND Kaposi sarcoma (KS) is known to involve the mucocutaneous tissues and the aero-digestive tracts. In acquired immune deficiency syndrome (AIDS) patients, KS has an aggressive course and carries poor prognosis. We present a case of pulmonary KS with osseous metastases as the first presentation of human immunodeficiency virus (HIV) infection in a young male. The lesions impressively decreased in size and numbers following initiation of highly active antiretroviral therapy (HAART). CASE REPORT A 34-year-old heterosexual male presented with a one month history of cough and 15-20 pound weight loss within six months. Examination revealed oral thrush, decreased breath sounds and crackles on the right lower lung base. Imaging showed a large right perihilar mass with multiple lytic lesions involving thoracic and lumber vertebrae, ribs, sternum, and clavicles. Blood and sputum cultures, smears for acid fast bacilli, and a QUANTIferon gold test were all negative. He tested positive for HIV and his CD4 count was 7 cells/uL. Bronchoscopy with biopsy was unrevealing. Pathology of the right hilar mass was diagnostic of KS. Following initiation of antiretroviral therapy his condition dramatically improved; repeat chest CT scan showed marked regression of the bony and pulmonary lesions. CONCLUSIONS The dual action of HAART on the recovery of the immune system and against human herpes virus 8 (HHV-8) may essentially cause regression of KS lesions.

Treatment efficacy with electrochemotherapy: A multi-institutional prospective observational study on 376 patients with superficial tumors.

BACKGROUND: Cutaneous metastases represent a therapeutic challenge. An increasing body of experience suggests that electrochemotherapy (ECT) provides effective tumor control, although its evidence basis should be strengthened. METHODS: This prospective, multicenter, observational study enrolled patients with superficial metastases, who underwent ECT at 10 centers between 2008 and 2013. Outcomes included adherence to European Standard Operating Procedures of ECT (ESOPE), tumor response, local progression-free survival (LPFS), toxicity and patient-reported outcomes (PROs, EORTC QLQ-C30 plus an 8-item questionnaire). RESULTS: We enrolled 376 eligible patients. Tumor histotype distribution was as follows: melanoma, 56%; squamous cell carcinoma, 11%; Kaposi sarcoma, 11%; breast carcinoma, 8%; basal cell carcinoma, 6%; soft tissue sarcomas, 3%; others, 5%. We registered 1304 target tumors (median size 1 cm). Treatment adhered to ESOPE in 88% of patients as to the route of drug administration, and in 70% as to electrode application. The procedure was mainly performed under sedation (64.6%) and by using intravenous chemotherapy (93.4%). Tumor response rate at 60 days was 88% (complete, 50%). Small tumor size predicted complete response achievement (OR 2.24, p = 0.003), higher LPFS (HR 0.68, p = 0.004) and improved PROs (Global Health Status, p < 0.001; wound bleeding, p < 0.001; healing, p = 0.002; and aesthetics, p < 0.001). Skin toxicity (grade ≥3, 7.8%) was lower in patients with tumors <2 cm (p≤0.001). One-year LPFS was 73.7% (95%CI 68.4-78.3). CONCLUSIONS: ECT represents a valuable skin-directed therapy across a range of malignancies. The most frequently applied treatment modality is intravenous chemotherapy under sedation. Small tumor size predicts durable tumor control, fewer side-effects and better PROs.

Two rare diagnoses during chronic lymphocytic leukaemia follow-up: Kaposi's sarcoma and Merkel cell carcinoma.

Chronic lymphocytic leukaemia often has a clinical presentation characterised by increased neoplastic lymphocytes which are mostly mature looking due to B lymphocytes. Increased secondary cancer prevalence has been detected among patients with chronic lymphocytic leukaemia diagnosis. In this report, we present three chronic lymphocytic leukaemia patients who developed secondary rare cancers during their follow-up at our clinic. Case 1: A 54-year-old female patient was diagnosed with stage I chronic lymphocytic leukaemia in 2003 and was diagnosed with Merkel cell carcinoma in February 2013. Case 2: A 66-year-old male patient was diagnosed with stage II chronic lymphocytic leukaemia in 2009 and was diagnosed with Kaposi's sarcoma in March 2013. Case 3: A 77-year-old male patient was diagnosed with stage I chronic lymphocytic leukaemia in 2006 and was diagnosed with Kaposi's sarcoma in 2011. In conclusion, secondary cancers are observed in patients diagnosed with chronic lymphocytic leukaemia. Therefore, follow-up of chronic lymphocytic leukaemia requires additional attention in this context.

HIV-related Kaposi's Sarcoma with Musculoskeletal Involvement in the Modern Antiretroviral Era.

AIM: To describe the patterns of disease and clinical outcomes of MSK-KS in people living with HIV in the era of (combination anti-retroviral therapy cART). PATIENTS AND METHODS: We reviewed our prospectively collected dataset of patients with HIV with biopsy-proven KS; 17 out of 1,489 seropositive patients were identified with subsequent evidence of MSK involvement by KS. We collected data with regards to clinicopathological parameters and radiological patterns of disease. RESULTS: Fourteen patients (82.4%) had AIDS Clinical Trials Group T1 stage disease at presentation including four (23.5%) with non-nodal visceral disease. At the time of MSK-KS diagnosis, more than 80% of 14 patients had excellent HIV control. The median interval between initial KS to MSK-KS diagnosis was 3.3 years. Five-year overall survival rate from initial KS diagnosis was 76%, and 60% from MSK-KS diagnosis. The majority of patients were asymptomatic and MSK-KS involvement was demonstrated during imaging prompted by progression of their mucocutaneous KS. The majority of lesions were lytic with cortical involvement on cross-sectional imaging, whereas a soft-tissue component was commonly associated with long-bone involvement. CONCLUSION: MSK-KS continues to be a rare entity in the modern era of cART, however patients appear to experience significantly improved survival.

Soft tissue sarcomas in skin: presentations and management.

Soft tissue sarcomas are a rare but heterogeneous family of malignant tumors that are predominantly found deep to the integumentary layer. Only a small number of these primary mesenchymal tumors actually originate from the dermal layers. A systematic approach to the evaluation and workup of these neoplasms can prevent inappropriate management. After staging evaluation, most of these tumors are primarily managed with en-bloc surgical resection. Other adjuvant therapies routinely employed include chemotherapy and radiation therapy. Proper treatment typically involves participation of a multidisciplinary care team for optimal outcome. General principles and treatment strategies will be discussed along with a review of the more common cutaneous manifestations of sarcoma.

Occurrence of double primary malignancies in an African renal transplant recipient.

A 63-year-old African male with end stage renal disease who received a renal transplantation from his daughter after successful treatment of hepatitis C virus, type 1 genotype developed metastatic Kaposi's sarcoma and subsequently adenocarcinoma of the prostate. He was successfully treated with chemotherapy and reduction of immunosuppression and switch over to rapamycin.

Disseminated Kaposi's sarcoma-a missed diagnosis.

BACKGROUND: Kaposi's sarcoma is significantly prevalent among men infected with the human immunodeficiency virus, accounting for >90% of all cases. The early presentation of KS typically involves mucocutaneous lesions and lymphadenopathy, and more advanced disease can affect the lungs and other organs. OBJECTIVE: Our aim was to remind emergency physicians to remain suspicious of clinical presentations despite previous diagnoses or patient statements, particularly in patients with risk factors. CASE REPORT: We present a case of a young man having skin lesions and respiratory problems remaining undiagnosed, despite, and possibly due to, multiple recent physician contacts. CONCLUSIONS: Respiratory illnesses are common presentations in the emergency department and are typically benign and attributed to viral causes. However, the emergency physician must always be on the look out for more dangerous causes of respiratory complaints, especially in patients with risk factors and in those found to be refractory to recent treatment for more common illnesses.

Kaposi's sarcoma: a population-based cancer registry descriptive study of 57 consecutive cases diagnosed between 1977 and 2009.

BACKGROUND: There are few epidemiological data available on rare skin cancer, including Kaposi's sarcoma (KS), which is a multifocal illness affecting the skin, mucosa, and viscera. Four different types of KS have been described: classic, AIDS-associated, iatrogenic, and African. The purpose of this study was to describe the epidemiology and evolution of the different types of KS in the Doubs region of France. PATIENTS AND METHODS: A retrospective population-based study was conducted, including 57 patients with KS from the Doubs region between 1977 and 2009. These patients were identified by the tumor registry of the Doubs region. RESULTS: A larger proportion of AIDS-associated KS (61%) was observed compared to classic KS (30%) and iatrogenic KS (9%). No cases of African KS were observed. Most new cases were observed between 1987 and 1996 due to the AIDS explosion. The patients were predominantly male, with a male/female ratio at 10 : 4 (34 for AIDS-associated KS). The mean age of patients was 50.2 (higher in classic and iatrogenic KS and lower in AIDS-associated KS). Mucosal and visceral forms were more common in AIDS-associated KS, explaining the higher rate of mortality due to KS (21%). CONCLUSION: KS, which used to be a rare illness, as it existed only in the classic form, has become more common since the appearance of HIV and, to a lesser extent, of immunosuppressive treatment.

Electrochemotherapy for the management of cutaneous and subcutaneous metastasis: a series of 39 patients treated with palliative intent.

BACKGROUND AND OBJECTIVES: Electrochemotherapy (ECT) is technique for local control of skin metastasis. This study is primarily aimed at assessing the clinical activity of ECT in a prospective cohort of patients, and evaluating the association between primary tumor histology, number of metastatic lesions and size of tumor deposits and objective response rate. METHODS AND RESULTS: Thirty-nine patients with skin metastases from melanoma and other tumors underwent intravenous bleomycin ECT with palliative intent. No serious adverse events (SAE) or CTC grade 3 or 4 were observed. Overall response rate (ORR) was 66.6%. Response rate significantly correlated at univariate analysis both with the number (< or >10) and the size (< or >2 cm) of metastases. No relationship was observed for the histology of primary tumor. At multivariate analysis the size of the nodules under 2 cm was associated with a positive response and the correlation was statistically significant. CONCLUSIONS: ORR was 66.6% and all patients obtained a subjective clinical benefit from the treatment with minimal side effects. The most suitable patients were the ones with nodules <2 cm.

Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and childhood. This tumor results in poor prognosis, and therefore, development of a more effective treatment is needed. METHODS AND RESULTS: We describe an 11-year-old boy presenting with left facial palsy caused by aggressive KHE of the left temporomastoid region. He was treated with paclitaxel-based chemotherapy, because of the difficulty with complete surgical resection for anatomic factor, multiple lung metastases on diagnosis, and no response to conventional treatments. This treatment reduced the volume of primary tumor and lung metastatic lesions, but the efficacy was transitory. CONCLUSIONS: Paclitaxel-based chemotherapy for aggressive KHE may be effective, therefore the multimodality therapy including paclitaxel of aggressive KHE, particularly in the head and neck, needs to be investigated in further studies.

Vertebral Lesions from AIDS-Related Kaposi's Sarcoma.

BACKGROUND: Kaposi's sarcoma is commonly described in HIV/AIDS patients but usually manifests as overt skin lesions or visceral involvement. Bone involvement, particularly vertebral, is uncommon, especially when there is no adjacent cutaneous lesion but a small number of cases have been reported. Unlike many other diseases associated with HIV, Kaposi's sarcoma can occur despite a normal CD4 count. CASE PRESENTATION: A 44 year-old HIV positive Nigerian man presented with a 20 day history of severe, worsening lumbar back pain, nearly three years after an earlier diagnosis of a single cutaneous lesion consistent with Kaposi's sarcoma, for which he received chemo-radiotherapy. Despite varying previous compliance with his anti-retroviral therapy, he was thought to be taking his medications at time of presentation and his CD4 count was 408 cells/mm(3). No other organ involvement was found but a pathological fracture was seen on magnetic resonance imaging affecting L1 vertebra. A CT-guided needle aspiration biopsy was performed and a histological diagnosis subsequently confirmed Kaposi's sarcoma. The patient was treated with further courses of radiotherapy but had little clinical improvement. Indeed, a follow-up MRI four months later showed new involvement of a further four vertebrae, fortunately in the absence of progressive focal neurology. CONCLUSION: Vertebral Kaposi's sarcoma is a rare diagnosis but can be accurately diagnosed with CT or MRI imaging in conjunction with a histological diagnosis. An immunosuppressed patient presenting with bone pain should be thoroughly investigated for Kaposi's sarcoma as modern chemotherapeutic agents alongside anti-retroviral therapy may delay or prevent further devastating complications such as spinal cord compression.