Classic Kaposi Sarcoma in an Immunocompetent Male From Haiti: An Unusual Epidemiological Presentation.

Kaposi sarcoma (KS) is an angioproliferative disorder caused by human herpesvirus-8 (HHV-8) infection. KS manifests as vascular and mucosal nodules and is classified into four subtypes based on epidemiology, clinical presentation, histopathology, and HHV-8/human immunodeficiency virus serology. Here, we present a unique case of classic KS in an 84-year-old immunocompetent Haitian male patient, highlighting the rarity of this variant in this population. Additionally, our article delves into the broader context by reviewing a few documented cases of classic KS in the Caribbean region.

Classic Kaposi Sarcoma: A Comprehensive Case Report on Multisite Involvements and Therapeutic Strategies.

Classic Kaposi sarcoma (CKS), a variant of Kaposi sarcoma (KS), predominantly affects elderly men of Mediterranean and Ashkenazi descent. It is primarily seen in immunocompetent patients, often as cutaneous manifestations in the lower extremities. Treatment of CKS ranges from radiation therapy, chemotherapeutic agents, surgical excision, cryosurgery, and immunotherapy, and the treatment selection is contingent on disease-specific manifestations. This study presents the case of an 83-year-old immunocompetent male of Mediterranean descent, diagnosed with CKS five years ago, exhibiting an onset of painful violaceous papulonodular lesions on the right medial plantar surface and painless papulonodular lesions on the right upper arm and medial thigh. The case highlights the intricacies of CKS diagnosis and management, shedding light on the diverse treatments targeted for lesions across various anatomical locations.

The Midwest Sarcoma Trials Partnership: Bridging Academic and Community Networks in a Collaborative Approach to Sarcoma.

The treatment of sarcoma necessitates a collaborative approach, given its rarity and complex management. At a single institution, multidisciplinary teams of specialists determine and execute treatment plans involving surgical, radiation, and medical management. Treatment guidelines for systemic therapies in advanced or nonresectable soft tissue sarcoma have advanced in recent years as new immunotherapies and targeted therapies become available. Collaboration between institutions is necessary to facilitate accrual to clinical trials. Here, we describe the success of the Midwest Sarcoma Trials Partnership (MWSTP) in creating a network encompassing large academic centers and local community sites. We propose a new model utilizing online platforms to expand the reach of clinical expertise for the treatment of advanced soft tissue sarcoma.

Primary intraosseous alveolar soft part sarcoma: Report of two cases with radiologic-pathologic correlation.

Alveolar soft part sarcoma (ASPS) accounts for less than 1 % of all soft tissue sarcomas. ASPS presents a poor prognosis and develops frequent metastases, especially in the lungs, brain and bones. Current therapies, such as surgery, radiotherapy and chemotherapy, are not fully effective and other alternative treatments are currently being studied. ASPS is predominantly found in the deep soft tissues of the lower extremities. To our knowledge, only thirteen primary intraosseous ASPS have been reported in the literature. In this study, we report two new cases of this exceedingly rare entity. Both cases already had multiple metastases since diagnosis; one of them represents the first case of a primary bone ASPS in the ulna and is also the primary intraosseous ASPS with the longest reported case of survival, after having maintained long periods of stabilization despite not having received any systemic treatment.

Survey of sarcoma surgery principles among orthopaedic oncologists.

BACKGROUND: Surgical principles and techniques used during primary sarcoma excision focus on acquiring negative margins, reducing the risk of local recurrence, and minimizing contamination. These principles and techniques within orthopaedic oncology are not well documented in the literature. No standardized surgical hand-off or approach to education across disciplines on orthopaedic oncology principles and techniques has been published. Currently, education on intraoperative approaches is passed down by oral tradition. OBJECTIVES: Our objective was to survey members of the Musculoskeletal Tumor Society (MSTS) to identify their core principles and practices in orthopaedic oncology. We aimed to 1) provide descriptive analyses of surgeon technique patterns; 2) determine correlations between individual practice patterns; and 3) identify distinct clusters of surgeons on the basis of common practice tendencies. METHODS: A web-based, 16-question survey regarding orthopaedic oncology intraoperative principles and techniques was distributed online to all 349 members of the MSTS in 2021. There were 137 (39%) unique respondents, all of whom completed the entire survey. The 16 survey questions were grouped into 4 key aspects of sarcoma excision: pre-incision, exposure of the mass, delivery of the mass, and closure. The questions inquired about respondent preference on draping, back table setup, instrument use, and intraoperative decision making. These questions were selected on the basis of existing reports, as well as the senior author's experience. We analyzed the responses using 3 methods: 1) descriptive statistics, 2) correlations between question responses, and 3) clustering analysis. We used an artificial intelligence-based clustering algorithm to cluster respondents according to their practice patterns. The results of our correlation analyses are reported as Spearman's rho (ρ) correlation coefficients. RESULTS: Most respondents (mean, 71%; standard deviation, 22%) reported using the described surgical techniques "most of the time" or "in all cases." A strong positive correlation was found between respondents who answered "yes" to both of the following questions: "Do you change your surgical gloves after passing off the tumor specimen?" and "Does your entire surgical team change their gloves after passing off the tumor specimen?" (ρ = 0.88). A moderate positive correlation was found between those who answered "yes" to both of the following questions: "Do you change your surgical gloves after passing off the tumor specimen (i.e., prior to closure)?" and "Do you use new and/or unused surgical instruments for the final closure?" (ρ = 0.60). The cluster analysis identified 3 distinct clusters of respondents. The conservative technique cluster (N = 42) was more likely to answer "yes" to 9 of the 10 questions regarding incision management, consultant team communication, gloving, and instrument use, whereas the permissive technique cluster (N = 41) was more likely to answer "no" to questions regarding gloving, draping, and instrument use. CONCLUSIONS: Our findings indicate that most respondents perform the surveyed techniques, and there is homogeneity in the practice patterns of members of the MSTS; however, we identified distinct clusters of respondents who were significantly more likely to perform certain techniques. These results support establishing a standardized set of intraoperative techniques and formal surgical education regarding intraoperative principles and techniques in orthopaedic oncology.

The Biology of Synovial Sarcoma: State-of-the-Art and Future Perspectives.

OPINION STATEMENT: New molecular insights are being achieved in synovial sarcoma (SS) that can provide new potential diagnostic and prognostic markers as well as therapeutic targets. In particular, the advancement of research on epigenomics and gene regulation is promising. The concrete hypothesis that the pathogenesis of SS might mainly depend on the disruption of the balance of the complex interaction between epigenomic regulatory complexes and the consequences on gene expression opens interesting new perspectives. The standard of care for primary SS is wide surgical resection combined with radiation in selected cases. The role of chemotherapy is still under refinement and can be considered in patients at high risk of metastasis or in those with advanced disease. Cytotoxic chemotherapy (anthracyclines, ifosfamide, trabectedin, and pazopanib) is the treatment of choice, despite several possible side effects. Many possible drug-able targets have been identified. However, the impact of these strategies in improving SS outcome is still limited, thus making current and future research strongly needed to improve the survival of patients with SS.

A rare case of synovial sarcoma with lung, heart and adrenal gland metastasis: a caution for patients and clinicians.

A 68-year-old man presented to the Urology Clinic, West China Hospital, Chengdu, with a suspected right adrenal gland mass that had persisted for two months. He had no associated lumbodynia, dizziness or palpitation. Abdominal computed tomography (CT) revealed an uneven density and contrast-enhanced oval-like mass with smooth edges in the right adrenal gland. Laparoscopic right adrenal gland resection followed by histopathology confirmed a diagnosis of metastatic synovial sarcoma. The patient had a history of synovial sarcoma on the right upper leg 3 years previously that was surgically treated, but he had not undergone further treatment. Approximately 1.5 years later, he had undergone surgery for heart and lung metastasis from the synovial sarcoma of the thigh. At 5 months following laparoscopic right adrenal gland resection, abdominal CT showed a significant sign of right adrenal recurrence, and targeted therapy of 12 mg oral anlotinib, daily, was initiated. This relatively rare but alarming case highlights the importance of patient understanding and compliance to treatment.

Utilization of Interdisciplinary Tumor Boards for Sarcoma Care in Germany: Results from the PROSa Study.

BACKGROUND: Data on institutional structures of sarcoma care in Germany are scarce. The utilization of an interdisciplinary tumor board (IDTB) is an essential part of modern cancer care. We investigated to which extent and when IDTB are used in sarcoma care. We hypothesized that IDTB before treatment initiation were used more often at certified cancer centers and at high-volume centers and that IDTB utilization increased over time. METHODS: From 2017 to 2020 we conducted a prospective cohort study, undertaking major efforts to include the whole spectrum of sarcoma treatment facilities. To analyze potential predictors of IDTB utilization, we calculated multivariable logistic regressions. RESULTS: Patients and survivors (n = 1,309) from 39 study centers (22 tertiary referral hospitals, 9 other hospitals, and 8 office-based practices) participated; 88.3% of the patients were discussed at some stage of their disease in an IDTB (56.1% before treatment, 78% after therapy, and 85.9% in metastatic disease). Hypotheses were confirmed regarding the utilization of IDTB in certified cancer centers (vs. all others: OR = 5.39; 95% CI 3.28-8.85) and the time of diagnosis (2018/2019 vs. until 2013: OR = 4.95; 95% CI 2.67-9.21). CONCLUSION: Our study adds to the evidence regarding the institutional structures of sarcoma care in Germany. Utilization of a tumor board before therapy seems to be in an implementation process that is making progress but is far from complete. Certification is a possible tool to accelerate this development.

Bone sarcoma surgery in times of COVID-19 pandemic lockdown-early experience from a tertiary centre in India.

BACKGROUND AND OBJECTIVES: Coronavirus disease 2019 (COVID-19) lockdown has presented a unique challenge for sarcoma care. The purpose of this study is to evaluate the early results and feasibility of surgeries for bone sarcomas during the COVID-19 lockdown. METHODS: Our prospectively collected orthopaedic oncological database was reviewed to include two groups of patients- those who underwent surgery in the immediate 4 weeks before lockdown (non-lockdown group) and those operated in the first 4 weeks of lockdown (lockdown group). All patients were followed-up clinically and telephonically to collect the outcome data. RESULTS: Out of the 91 patients who qualified for inclusion, fifty were classified into the non-lockdown group while 41 patients formed the lockdown group. Both the groups were comparable with respect to baseline demographic parameters. However, during the lockdown period 37 patients (90%) had undergone a major surgical intervention as against 24 patients (48%) in the non-lockdown group (P < .001). There was no significant difference in type of anaesthesia, median estimated blood loss and procedure duration. None of the patients/health care workers had evidence of severe acute respiratory syndrome-coronavirus 2 infection at 15 days follow-up. CONCLUSION: Our study results suggest that appendicular bone tumours can be safely operated with adequate precautions during the lockdown period.

Current State of Sarcoma Care in Germany: Results of an Online Survey of Physicians.

BACKGROUND: Sarcoma treatment is a complex and multidisciplinary process. Little is known about the actual status of sarcoma care in Germany. OBJECTIVES: We evaluated: (1) on an institutional level, which physician diagnosed and treated sarcomas; (2) whether guidelines and tumor board recommendations were routinely implemented; and (c) access to which diagnosis and treatment options was regarded as problematic. We also examined factors that were associated with access problems. METHODS: A cross-sectional online survey was employed among German sarcoma physicians between June 2017 and February 2018 with convenience sampling. RESULTS: Two hundred fourteen physicians participated; 46% were oncologists and 27% surgeons, 38% worked in hospitals of maximum care, 34% were office based and 27% worked in other hospitals, 68% of all of the physicians consulted established guidelines, and 93% presented their patients in multidisciplinary tumor boards. The most common access problems were: isolated limb perfusion (39%), deep-wave hyperthermia (33%), and FDG-PET (27%), and 42% reported no access problems at all. Those physicians who treat more than 100 patients per year reported "no access problems" more frequently compared to centers with lower patient numbers (vs. 0-10 patients, OR 0.14; 95% CI 0.03-0.61; vs. 11-100 patients, OR 0.21; 95% CI 0.06-0.73). CONCLUSIONS: Access to multidisciplinary tumor boards seems to be largely guaranteed in the participants of our survey. The use of guidelines could be further implemented and expanded. The number of treated patients appears to be a significant factor to avoid access problems to treatment options.

Treatment at low-volume hospitals is associated with reduced short-term and long-term outcomes for patients with retroperitoneal sarcoma.

BACKGROUND: Retroperitoneal sarcomas (RPS) are rare tumors for which complete surgical resection remains the mainstay of treatment. The objective of the current study was to determine the impact of hospital case volume on outcomes in patients with RPS. METHODS: A total of 6950 patients with primary RPS who underwent surgical resection were identified from the National Cancer Data Base (1998-2011). Treating hospitals were classified by annual case volume; low-volume hospitals (LVHs) and high-volume hospitals (HVHs) were defined as those with ≤10 cases per year and >10 cases per year, respectively. Overall survival (OS) was compared using Kaplan-Meier curves. Cox proportional hazard models were created to compare risks. RESULTS: Of the 1131 reporting hospitals, the majority (1127 hospitals; 99.6%) were LVHs treating the majority of patients (6270 patients; 90.2%). Patients treated at LVHs were more likely to have lower grade and smaller tumors, receive radiotherapy, and undergo incomplete macroscopic (R2) resection. Patients treated at HVHs had lower 30-day readmission rates (1.8% vs 3.4%; P<.001), 30-day (1.9% vs 3.1%; P=.004) and 90-day (3.2% vs 5.7%; P=.007) mortality, longer median OS (76.2 months vs 64.2 months; P<.001), and higher 5-year OS rates (58% vs 52%; P<.001). After controlling for age, sex, insurance status, tumor size, tumor grade, surgical resection margin status, and radiotherapy administration, treatment at an HVH was found to be independently associated with a reduced risk of death (hazard ratio, 0.77; 95% confidence interval, 0.65-0.91 [P=.003]). CONCLUSIONS: Primary RPS are rare tumors, and to our knowledge few surgeons and institutions have significant experience and expertise in their multidisciplinary management and surgical resection. Although additional studies are needed, patient outcomes may be impacted by the case volume and expertise of the treating facility.

Three-Dimensional Patient-Derived In Vitro Sarcoma Models: Promising Tools for Improving Clinical Tumor Management.

Over the past decade, the development of new targeted therapeutics directed against specific molecular pathways involved in tumor cell proliferation and survival has allowed an essential improvement in carcinoma treatment. Unfortunately, the scenario is different for sarcomas, a group of malignant neoplasms originating from mesenchymal cells, for which the main therapeutic approach still consists in the combination of surgery, chemotherapy, and radiation therapy. The lack of innovative approaches in sarcoma treatment stems from the high degree of heterogeneity of this tumor type, with more that 70 different histopathological subtypes, and the limited knowledge of the molecular drivers of tumor development and progression. Currently, molecular therapies are available mainly for the treatment of gastrointestinal stromal tumor, a soft-tissue malignancy characterized by an activating mutation of the tyrosine kinase KIT. Since the first application of this approach, a strong effort has been made to understand sarcoma molecular alterations that can be potential targets for therapy. The low incidence combined with the high level of histopathological heterogeneity makes the development of clinical trials for sarcomas very challenging. For this reason, preclinical studies are needed to better understand tumor biology with the aim to develop new targeted therapeutics. Currently, these studies are mainly based on in vitro testing, since cell lines, and in particular patient-derived models, represent a reliable and easy to handle tool for investigation. In the present review, we summarize the most important models currently available in the field, focusing in particular on the three-dimensional spheroid/organoid model. This innovative approach for studying tumor biology better represents tissue architecture and cell-cell as well as cell-microenvironment crosstalk, which are fundamental steps for tumor cell proliferation and survival.