scholarly journals Epidemiology of Soft Tissue Sarcoma and Bone Sarcoma inItaly: Analysis of Data from 15 Population-Based Cancer Registries

Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Sabrina Fabiano ◽  
Paolo Contiero ◽  
Giulio Barigelletti ◽  
Anna D’Agostino ◽  
Andrea Tittarelli ◽  
...  

Sarcomas are a heterogeneous group of rare cancers of mesenchymal origin. In this study, we provide updated, world age-standardised incidence rate (ASR) and European age-standardised incidence rate for malignant soft tissue sarcoma (ICD-O-3 topographic code C47–C49) and bone sarcoma (C40, C41) in Italy, by area (north, centre, and south) and by cancer registry. We also assess morphology in relation to site and area and assess metastases at diagnosis. We analysed 1,112 cases, with incidence 2009–2012, provided by 15 cancer registries (CRs) affiliated to the Association of Italian Cancer Registries (AIRTUM). Overall, ASR was 1.7/100,000/year for soft tissue sarcoma and 0.7 for bone sarcoma. Central Italy had the highest (2.4) ASR and south Italy had the lowest (1.6) ASR for soft tissue sarcoma. Central Italy had the highest (1.1) ASR and north Italy had the lowest (0.7) ASR for bone sarcoma. By CR, ASRs ranged from 1.1 to 2.6 for soft tissue sarcoma and from 0 to 1.4 for bone sarcoma. The most frequent soft tissue sarcomas were sarcoma not otherwise specified (NOS) (29.4%) and liposarcoma (22.2%); the most common bone sarcoma was chondrosarcoma (37.6%). Soft tissue sarcomas occurred most frequently (35.6%) in lower limb connective tissue; bone sarcomas arose mainly (68.8%) in long bones. The frequencies of morphologies arising at different sites varied considerably by Italian area; for example, 20% of hemangiosarcomas occurred in the head and neck in south Italy with 17% at this site in the centre and 6% in the north. For soft tissue sarcoma, the highest ASRs of 2.6 and 2.4 contrast with the lowest ASRs 1.1 and 1.3, suggesting high-risk hot spots that deserve further investigation. The marked variations in morphology distribution with site and geography suggest geographic variation in risk factors that may also repay further investigation particularly since sarcoma etiology is poorly understood.

SICOT-J ◽  
2019 ◽  
Vol 5 ◽  
pp. 23
Author(s):  
Takuya Kakimoto ◽  
Akihiko Matsumine ◽  
Kunihiro Asanuma ◽  
Takao Matsubara ◽  
Tomoki Nakamura ◽  
...  

Introduction: Reconstruction using a total femur prosthesis (TFP) remains a challenging procedure in musculoskeletal tumor surgery. The purpose of this study was to show the clinical outcomes of total femur replacement (TFR) in our institute. Methods: Nine patients underwent reconstruction with a TFP after the wide resection of malignant bone and soft-tissue tumors of the femur between January 2003 and April 2014. The mean age of the patients at the time of TFR was 47.5 years, and the mean follow-up period was 52.9 months. The histological diagnoses were as follows: bone sarcoma (n = 4), soft-tissue sarcoma invading the femoral bones (n = 4), and metastatic bone tumor (n = 1). Results: The oncological outcomes were as follows: three patients achieved continuous disease free, two patients were alive with disease, and four patients died from disease. The 3- and 5-year overall survival rates were 88.9% and 55.6%, respectively. The rate of the overall survival in patients with primary bone tumors (100% at 5 years) was significantly better than that in patients with primary soft tissue sarcomas (0% at 5 years) (p = 0.015). A deep infection occurred postoperatively in one patient, but the patient was successfully treated with surgical debridement and revision surgery. There were no patients who showed dislocation or aseptic loosening. The mean Musculo-Skeletal Tumor Society functional score was 58.5% (46.7–80.0), with scores of 65.5% in patients with a primary bone tumor and 50.8% in those with a primary soft-tissue sarcoma. Discussion: In the present study, the patients who underwent TFR due to bone invasion by soft tissue sarcoma had a worse prognosis than the bone sarcoma patients.


2000 ◽  
Vol 18 (10) ◽  
pp. 2081-2086 ◽  
Author(s):  
Jaap Verweij ◽  
Siow Ming Lee ◽  
Wlodzimir Ruka ◽  
Jose Buesa ◽  
Robert Coleman ◽  
...  

PURPOSE: To assess antitumor response and time to progression (TTP) with docetaxel compared with doxorubicin in first-line treatment of advanced and/or metastatic soft tissue sarcoma. PATIENTS AND METHODS: Patients with measurable soft tissue sarcoma lesions and adequate bone marrow, liver, and renal function were entered onto the study. They were randomized to either docetaxel 100 mg/m2 given as a 1-hour intravenous infusion every 3 weeks or doxorubicin 75 mg/m2 given as a bolus injection every 3 weeks. A maximum of seven cycles of treatment were scheduled. The study was designed as a randomized phase III study evaluating TTP by log-rank model. There was a clause for premature closure of the trial if fewer than five responses were observed among the first 25 assessable patients in the docetaxel treatment arm. RESULTS: Eighty-six patients were entered onto the study; 85 were assessable for toxicity and 83 for response. The rate of severe granulocytopenia was not significantly different between the two arms. Nausea (P = .001), vomiting (P < .001), and stomatitis (P = .005) were more common with doxorubicin therapy, whereas neurotoxicity was more frequent with docetaxel treatment. The response rate to doxorubicin therapy was 30% (95% confidence interval, 17% to 46%), whereas no responses to docetaxel therapy were seen (P < .001). In view of this, the trial was closed prematurely and the phase III study part was not conducted. CONCLUSION: Docetaxel is inactive in soft tissue sarcomas and cannot be recommended for further use in treatment of this disease.


2016 ◽  
Vol 34 (7_suppl) ◽  
pp. 211-211
Author(s):  
Minggui Pan ◽  
Andrew Fang ◽  
Maihgan Kavanagh ◽  
Brian Kim ◽  
Jason D Lee ◽  
...  

211 Background: Bone and soft tissue sarcomas are relatively uncommon and their management is extremely complex requiring coordinated multidisciplinary expertise for optimal care. Because Kaiser Permanente operates across many geographic regions in the country, in-person tumor board is not practical. Methods: The Kaiser Permanente Northern California medical oncology team has developed a system-based virtual tumor board (VTB) and electronic consultation (EC) platform comprised of experts from several key disciplines including musculoskeletal/surgical oncology, musculoskeletal radiology, pathology, radiation oncology, brachytherapy, medical adult and pediatric oncology. The VTB and EC cases are physician referral-based. The VTB occurs once every two weeks via video conferencing reviewing imaging studies and pathology while EC is available for more urgent cases whenever requested. This platform is available to Kaiser Permanente physicians in Northern and Southern California and other states including Hawaii, Mid-Atlantic, Northwest, Colorado, and Georgia. We conducted a survey targeting referring physicians to assess the value of this service platform in improving sarcoma care. Results: From March 2013 to October 2015, approximately 500 cases have been referred to the VTB and EC. Approximately 2/3 of the cases were referred to the VTB and 1/3 to the EC. The cases include a vast spectrum of bone and soft tissue sarcoma cases that were either newly diagnosed or at the time of relapse or progression. The physician survey on the quality and utilization of this sarcoma platform provided very positive feedback, with 100% responses stating that VTB and EC improved patient care and their confidence in managing patients with sarcoma, 90% stating VTB and EC changed their management to a certain extent. Approximately 90% responders rated VTB and EC very to extremely helpful. Conclusions: We conclude that a system-based VTB and EC can provide an excellent platform for improving quality of care and physician confidence in managing patients with bone and soft tissue sarcoma in an integrated healthcare system.


2019 ◽  
Vol 37 (35) ◽  
pp. 3436-3445 ◽  
Author(s):  
Ruth A. Kleinerman ◽  
Sara J. Schonfeld ◽  
Byron S. Sigel ◽  
Jeannette R. Wong-Siegel ◽  
Ethel S. Gilbert ◽  
...  

PURPOSE Survivors of hereditary retinoblastoma have excellent survival but substantially increased risks of subsequent bone and soft-tissue sarcomas, particularly after radiotherapy. Comprehensive investigation of sarcoma risk patterns would inform clinical surveillance for survivors. PATIENTS AND METHODS In a cohort of 952 irradiated survivors of hereditary retinoblastoma who were originally diagnosed during 1914 to 2006, we quantified sarcoma risk with standardized incidence ratios (SIRs) and cumulative incidence analyses. We conducted analyses separately for bone and soft-tissue sarcomas occurring in the head and neck (in/near the radiotherapy field) versus body and extremities (out of field). RESULTS Of 105 bone and 124 soft-tissue sarcomas, more than one half occurred in the head and neck (bone, 53.3%; soft tissue, 51.6%), one quarter in the body and extremities (bone, 29.5%; soft tissue, 25.0%), and approximately one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). We noted substantially higher risks compared with the general population for head and neck versus body and extremity tumors for both bone (SIR, 2,213; 95% CI, 1,671 to 2,873 v SIR, 169; 95% CI, 115 to 239) and soft-tissue sarcomas (SIR, 542; 95% CI, 418 to 692 v SIR, 45.7; 95% CI, 31.1 to 64.9). Head and neck bone and soft-tissue sarcomas were diagnosed beginning in early childhood and continued well into adulthood, reaching a 60-year cumulative incidence of 6.8% (95% CI, 5.0% to 8.7%) and 9.3% (95% CI, 7.0% to 11.7%), respectively. In contrast, body and extremity bone sarcoma incidence flattened after adolescence (3.5%; 95% CI, 2.3% to 4.8%), whereas body and extremity soft-tissue sarcoma incidence was rare until age 30, when incidence rose steeply (60-year cumulative incidence, 6.6%; 95% CI, 4.1% to 9.2%), particularly for females (9.4%; 95% CI, 5.1% to 13.8%). CONCLUSION Strikingly elevated bone and soft-tissue sarcoma risks differ by age, location, and sex, highlighting important contributions of both radiotherapy and genetic susceptibility. These data provide guidance for the development of a risk-based screening protocol that focuses on the highest sarcoma risks by age, location, and sex.


2000 ◽  
Vol 86 (5) ◽  
pp. 381-383 ◽  
Author(s):  
Gloria Costani ◽  
Paolo Rabitti ◽  
Adelmo Mambrini ◽  
Edoardo Bai ◽  
Franco Berrino

Background and objectives After the notification by a general practitioner of a high frequency of soft tissue sarcomas among subjects living close to the industrial area of the city of Mantua (northern Italy), the local Medical Association carried out a formal epidemiologic investigation to corroborate or falsify the hypothesized excess. Several industrial activities of the area were hypothesized to be a source of environmental pollution that might cause soft tissue sarcomas. Methods All general practitioners working in the area were requested to report the cases of soft tissue sarcoma diagnosed in the study area. Person-years of observation were computed for all subjects who ever resided in the area between 1984 and 1997. Expected incidence was computed from the cancer registry covering a nearby province (Varese) and from the pool of Italian cancer registries. Peripheral soft tissue sarcoma and visceral sarcomas were included in the analysis. Results and conclusions Overall, 20 cases were observed in a 13-year period, 8.87 were expected from the Varese province cancer registry and 7.72 from the pool of Italian registries. The corresponding standardized morbidity ratios were 2.25 (95% confidence interval, 1.34–3.47) and 2.6 (95% confidence interval, 1.6–4.0), respectively. A significant excess persisted after excluding 5 cases that had been the object of the original notification. We hypothesize that the soft tissue sarcoma excess may have resulted from environmental pollution by industrial toxic emissions, which likely included 2,3,7,8-tetra-chloro-dibenzo-dioxin.


Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 774
Author(s):  
Pietro Scicchitano ◽  
Maria Chiara Sergi ◽  
Matteo Cameli ◽  
Marcelo H. Miglioranza ◽  
Marco Matteo Ciccone ◽  
...  

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.


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