The epidemiology of desmoid tumors in Denmark.

INTRODUCTION/AIM: The epidemiology, demographic, clinical, treatment, and healthcare resource utilization (HRU) characteristics of desmoid tumor (DT) patients treated at two sarcoma centers in Denmark is described. METHODS: Using Danish health registers, we studied DT patients treated at two sarcoma centers between 2009 and 2018. For each patient, ten persons from the general population were randomly matched on birth year, sex, and region of residence. RESULTS: Of the 179 DT patients identified, 76% were female and the median patient age was 38 years at diagnosis (interquartile range: 31-50). An average annual incidence of DTs over the study period was 3.2 per 1000,000 individuals with the observed annual incidence of DTs ranging from 2.2 (2011) to 4.3 (2017) per 1000,000 individuals. No notable linear time trend in incidence was observed. Anatomical DT sites included extra-abdominal (49%), abdominal wall (40%), and intra-abdominal or retroperitoneal areas (8%). In total, 56% of patients were initially treated surgically. However, while 75% of patients diagnosed with DT between 2009 and 2014 were initially treated surgically, this was true for only 32% of patients diagnosed with DT between 2015 and 2018. A total of 56% of DT patients used chemotherapeutic agents, tyrosine kinase inhibitors, NSAIDs, opioids, antidepressants, or steroids at some point during the three years before their DT diagnoses. In contrast, 70% of surgically treated and 63% of non-surgically treated patients used one of these drugs in the subsequent three years, including NSAIDs (45% surgical vs. 33% non-surgical), opioids (39% surgical vs. 27% non-surgical), and steroids (22% surgical vs. 18% non-surgical). The average number of inpatient and outpatient visits, days of hospitalization, and additional surgical procedures were higher among DT patients than the comparison cohort. CONCLUSION: DTs are rare but have a large impact on patients' health, HRU, and medication utilization.

[Diagnostics and treatment of lipomas].

In this case report, a woman of 55 years suddenly developed foot pain and swelling 13 years after treatment for breast cancer. X-ray was found to be normal, and the symptoms were in three months interpreted as ligamentous sprains. Due to persistent pain and functional impairment together with a renewed X-ray suspicious for malignancy, an MRI scan was performed and signs of malignancy were confirmed. A biopsy from talus showed metastasis from a breast carcinoma, and the patient was treated with an amputation.

Changes in Functional Outcome and Quality of Life in Soft Tissue Sarcoma Patients within the First Year after Surgery: A Prospective Observational Study.

Soft tissue sarcoma (STS) patients undergoing surgery may experience reduced functional outcome (FO) and quality of life (QoL) compared to the general population. The aim of this study was to evaluate the development of FO and QoL in patients with STS in the extremities within the first year after first-time limb-sparing surgery. Twenty-nine out of 40 eligible patients were included in the present study. QoL and FO were evaluated by questionnaires while FO was also evaluated by objective tests. Patients were assessed before surgery and at fixed time points within the first year after surgery. Patients with STS in the extremities had an average strength of 82.34% (95% CI: 68.57-96.11) of the expected strength at one year post surgery. Multivariate, repeated ANOVA showed statistically significant reductions in strength in the disease-affected extremity when compared with the healthy side after surgery. Multivariate, repeated ANOVA showed a statistically significant improvement in FO and QoL within the first year post surgery. Limb-sparing surgery for STS significantly reduced strength in the disease-affected extremity and generally reduced FO and QoL in the first months after surgery. Improvements were observed for FO and QoL at one year after surgery.

The association between socioeconomic position and tumour size, grade, stage, and mortality in Danish sarcoma patients - A national, observational study from 2000 to 2013.

Background: Survival in sarcoma patients depends on a range of prognostic factors. An association between cancer survival and socioeconomic position is known for several other cancers. We therefore examined the relations between three socioeconomic factors and risk of presenting with known tumour related prognostic factors, and the overall mortality of the different socioeconomic and prognostic factors in 1919 patients diagnosed with sarcoma in Denmark 2000-2013.Material and methods: Patients with sarcoma in extremities or trunk wall aged 30 years or more at diagnosis were identified in the Danish Sarcoma Registry and linked on an individual level to Danish national registries. We obtained data on educational level, disposable income and cohabitation status. Odds ratios (ORs) were estimated for the association between the socioeconomic factors and grade, stage and tumour size. Hazard ratios (HRs) were estimated using Cox proportional hazard models.Results: In adjusted analyses, educational level, income and cohabitation status were not associated with high grade or dissiminated stage at time of diagnosis. However, living alone was statistically significantly associated with having a large soft tissue sarcoma (≥5 cm) at time of diagnosis (OR 1.51; CI1.12-2.03). The overall mortality was statistically significantly increased in the group of patients with ≤10 years of education (HR 1.27; CI 1.02-1.57), in patients with the 20% lowest income (HR 1.30; CI 1.00-1.67) and nearly in patients living alone (HR 1.16; CI 0.99-1.36).Conclusion: In this nationwide, multicentre, population-based study, soft tissue sarcoma patients living alone had greater risk of having a large tumour at time of diagnosis. Soft tissue and bone sarcoma patients with a short education, low income, or living alone, had a higher mortality. This might indicate that the social differences in mortality might be related to treatment aspects and the biology of the disease rather that the diagnostic process.

[Purtscher's retinopathy as a complication to a cemented resection tumour prosthesis].

In this case report, a 53-year-old female with a pathological subtrochanteric femur fracture received a cemented resection tumour prosthesis. The patient was post-operatively diagnosed with Purtscher's retinopathy due to fat embolisation. Purtscher's retinopathy has previously been described in trauma patients, however, no previous reports exist with patients treated with prosthesis. No treatment guideline is available. There is a risk of permanent visual impairment, and it is recommended, that patients presenting reduced vision post-operatively are referred to an ophthalmologist without delay for correct diagnosis.

Chondrosarcoma: the impact of comorbidity - 30 years of experience from a population-based database including 199 consecutive chondrosarcoma patients.

BACKGROUND: Adjustment for comorbidity when investigating potential prognostic factors, especially in elderly cancer patients, is imperative. Patients diagnosed with chondrosarcoma are elderly and more comorbidity is expected for these patients. Demographic changes are awaited in the future resulting in more and more elderly patients with comorbidity. The aims of this study were to characterize patients with chondrosarcoma treated at a single institute and to evaluate various prognostic factors for survival adjusted for comorbidity. MATERIAL AND METHODS: Between 1979 and 2008, 199 patients were treated at the Sarcoma Centre of Aarhus University Hospital, for chondrosarcoma. The incidence was calculated as a WHO age-standardized incidence rate (IR) per million per year. The endpoints were overall mortality and disease-specific mortality. Possible prognostic factors were analyzed for patients with intermediate/high-grade localized tumors by the uni- and multivariate Cox-proportional hazard method. RESULTS: The WHO age-standardized IR in western Denmark in the period 1979-2008 was 2.4/million inhabitants/year (95% CI: 2.2;2.6). The 5-year overall and disease-specific mortality for the 199 patients were 29% (95% CI: 23;36) and 22% (95% CI: 16;27), respectively. The 5-year disease-specific mortality for patients with metastatic disease was significantly higher than for patients with localized disease. The median time to relapse was 2.0 years. Patients who relapse within 1 year after the primary diagnosis have a significantly higher 5-year overall mortality compared to patients who relapse after 1 year. The presence of comorbidity and high-grade tumors were independent prognostic factors for both the overall mortality and the disease-specific mortality of chondrosarcoma patients. CONCLUSION: Patients with comorbidity had a significantly increased overall mortality and disease-specific mortality. We found that adjusting for comorbidity is important when investigating a cohort of elderly patients.

Use of Healthcare Services Two Years before Diagnosis in Danish Sarcoma Patients, 2000-2013.

BACKGROUND: Sarcoma is a rare type of cancer with nonspecific symptoms and uncertain aetiology. Thus, timely diagnosis of sarcomas is a clinical challenge. The aim of this study was to investigate the use of healthcare services 24 months preceding a sarcoma diagnosis compared to a matched cohort. MATERIALS AND METHODS: The study was a retrospective, population-based, matched cohort registry-study. Patients with sarcoma in Denmark in 2000-2013 were identified in the Danish Sarcoma Registry (n = 2167) and matched 1 : 10 on gender, age, and listed general practice. Using a binomial regression model, incidence rate ratios were calculated for face-to-face contacts in general practice, inpatient and outpatient visits, surgery, paraclinical examinations, and diagnostic imaging. Analyses were stratified for sarcoma subtypes, grade, stage, gender, and presence of comorbidity. RESULTS: The sarcoma patients had significantly increased incidence rate ratios in use of healthcare services compared to the matched cohort a year before their diagnoses. An increase in consultation rates was seen 11 months before diagnosis for inpatient visits, 9 months before diagnosis in general practice and outpatient visits, 8 months before diagnosis for paraclinical examinations, and 4 and 3 months before diagnosis for diagnostic imaging and surgery, respectively. There were no clinical significant differences in length of increased consultation rates between sarcoma type, stage, and grade. Sarcoma patients with comorbidity had persistently higher consultation rates compared to patients without comorbidity. CONCLUSIONS: The use of healthcare services among sarcoma patients increased several months before diagnosis in all healthcare sectors. The results reveal a diagnostic time window and a potential to refer, diagnose, and treat sarcoma patients in a timelier manner.

Validation of the Danish version of the musculoskeletal tumour society score questionnaire.

BACKGROUND: The musculoskeletal tumour society score (MSTS) is a well-known questionnaire for measuring functional outcome in patients with neoplasms in the extremities. Standardized guidelines for cross-cultural translation and validation ensure the equivalence of content between the original and translated versions. The translation and validation provide the possibility to compare different sarcoma populations on an international level. This study is based on the hypothesis that the Danish MSTS questionnaire is a valid tool for measuring the end result after surgery for neoplasms in the extremities. AIM: To validate the Danish version of the upper and lower extremity version of the MSTS. METHODS: The translation of the MSTS was conducted in accordance with international guidelines. Patients operated for sarcomas and aggressive benign tumors were invited to participate in the study. The psychometric properties of the Danish version of the MSTS were tested in terms of validity and reliability and for the risk of floor or ceiling effect. Spearman's rank coefficient was used to test the validity by comparing with the Toronto Extremity Salvage Score (TESS). The Intraclass Correlation Coefficient (ICC) was used to evaluate inter-rater reliability. Cronbach's alpha was used to test for internal consistency. Spearman's rank coefficient was used to compare the MSTS lower extremity version with the objective test, Timed Up and Go (TUG). RESULTS: The upper extremity version demonstrated an ICC of 0.95 in the inter-rater reliability test. The lower extremity version had an ICC of 0.88 in the inter-rater reliability test, respectively. Both MSTS versions showed a ceiling effect. The validity of the MSTS was measured by Spearman's rank correlation coefficient by comparing the MSTS with the TESS and found it to be of 0.80 (P < 0.01) and 0.83 (P < 0.01) for the upper extremity and lower extremity version, respectively. A Spearman's rank correlation coefficient of - 0.26 (P < 0.01) was found between the TUG and the MSTS questionnaire. A Spearman's rank correlation coefficient of - 0.38 (P < 0.01) was found between the TUG and the lower extremity version of the TESS questionnaire. CONCLUSION: The Danish version of the MSTS questionnaires were found to have good reliability and validity, however a substantial ceiling effect was identified.

Is there consensus regarding surgical treatment of bone sarcomas?

AIM: To perform an Internet based survey on the surgical management of bone sarcomas in the lower extremity amongst sarcoma surgeons. METHODS: All orthopedic surgical members of the Scandinavian Sarcoma Group were invited to participate in an online questionnaire. The questionnaire consisted of a clinical case involving resection of a malignant bone tumor. Several questions were asked, subdivided into categories. Among these, surgical/technical considerations, e.g., choice of implant; choice of antibiotics, dosage, and duration of treatment, choice of antithrombotic drug, initial start-up, dosage, and duration were included. RESULTS: In terms of choice of implant fixation, the majority of surgeons preferred an uncemented prosthesis in younger patients until the age of 50. All participants administer intravenous prophylactic antibiotics for endoprosthetic reconstructive surgery. First choice of antibiotics was cephalosporin. Less common used was glycopeptide, penicillin, or a combination. Duration of prophylactic antibiotics ranged from less than one day to more than four days. All participants used low molecular weight heparins as antithrombotic prophylaxis and 55% of the participants answered that initial treatment was started preoperatively, 3% perioperatively and 42% postoperatively. Duration of the antithrombotic treatment ranged from five days to more than twenty-eight days. CONCLUSION: The use of resection prosthesis in the treatment of bone sarcomas is a well-established procedure. However, therse is a significant discrepancy in the surgical treatment algorithm between the sarcoma centers. Still the treatment is mainly based on best clinical practice, due to the lack of evidence-based medicine in the surgical management of bone sarcomas.

The impact of comorbidity on mortality in Danish sarcoma patients from 2000-2013: A nationwide population-based multicentre study.

INTRODUCTION: Sarcoma is a rare type of cancer. The incidence increases with age and elderly patients may have comorbidity that affects the prognosis. The aim of this study was to describe the type and prevalence of comorbidity in a nationwide population-based study in Denmark from 2000-2013 and to analyse the impact of the different comorbidities on mortality. MATERIAL AND METHODS: The Danish Sarcoma Registry is a national clinical database containing all patients with sarcoma in the extremities or trunk wall from 2000 and onwards. By linking data to other registries, we were able to get patient information on an individual level including date and cause of death as well as the comorbidity type up to 10 years prior to the sarcoma diagnosis. Based on diseases in the Charlson Comorbidity Index, we pooled the patients into six categories: no comorbidity, cardiopulmonary disease, gastrointestinal disease, neurovascular disease, malignant neoplasms, and miscellaneous (diabetes, renal and connective tissue diseases). 2167 patients were included. RESULTS: The prevalence of comorbidity was 20%. For patients with localized disease, comorbidity increased the disease-specific mortality significantly (HR 1.70 (95% CI 1.36-2.13)). For patients with metastatic disease at the time of diagnosis, comorbidity did not affect the disease-specific mortality (HR 1.05 (95% CI 0.78-1.42)). The presence of another cancer diagnosis within 10 years prior to the sarcoma diagnosis was the only significant independent prognostic factor of disease-specific mortality with an increase of 66% in mortality rate compared to patients with no comorbidity (HR 1,66 (95% CI 1.22-2.25)). CONCLUSION: Comorbidity is a strong independent prognostic factor of mortality in patients with localized disease. This study emphasizes the need for optimizing the general health of comorbid patients in order to achieve a survival benefit from treatment of patients with localized disease, as this is potentially modifiable.

Outcome Measures After Sclerotherapy of Venous Malformations: A Systematic Review.

OBJECTIVE: Sclerotherapy has become an important treatment option in the management of vascular malformations. However, little is known about success rate for treatment of venous malformations. This systematic review assesses the available published literature on outcome measures of sclerotherapy for venous malformations. DATA SOURCE: PubMed and EMBASE. REVIEW METHOD: A systematic search was conducted, and studies from March 2008 to October 2016 were included. Based on the identified search results, study selection, data extraction, and assessment of study quality were, according to PRISMA, undertaken independently by two reviewers. RESULTS: One randomized controlled trial and 44 cohort studies were included. The most frequently used measuring methods were subjective measurements, objective measurements, imaging like MRI and ultrasound, and finally patient reported outcome measure survey. Approximately 60% of the studies used more than one measuring method. Outcome measures were pain, swelling, disfigurement, cosmetic complains, patient satisfaction, physical difficulties, volume, diameter, mass, color, shape, size, venous flow, occlusion of venous space, blood pool ratio, and radioisotope uptake. CONCLUSIONS: The published literature over the past 10 years shows no clear method to evaluate the effect of sclerotherapy for venous malformation. We suggest that a standard set of outcome measures should be defined.

A Validated Prognostic Biomarker Score for Adult Patients with Nonmetastatic Soft Tissue Sarcomas of the Trunk and Extremities.

BACKGROUND: The prognostic value of serum biomarkers in soft tissue sarcoma (STS) is limited, and its clinical applicability is compromised by a common inability to adjust for important confounders. The aim of this study was to determine the prognostic value of pretreatment biomarkers on disease-specific survival (DSS) adjusted for confounders. METHODS: The study included 818 patients with localized STS. Pretreatment levels of albumin, C-reactive protein, hemoglobin, neutrophils, and lymphocytes were tested individually and combined in prognostic scores: neutrophil/lymphocyte ratio (NLR), Glasgow Prognostic Score (GPS), and Aarhus Composite Biomarker Score (ACBS) which includes all five biomarkers. Patients were randomly split into a test cohort and a validation cohort. The prognostic value of biomarkers on DSS was estimated using crude and adjusted Cox proportional hazard models. The different biomarker scores were compared using Akaike's information criteria. RESULTS: In the test cohort of 403 patients, all biomarkers except lymphocyte count were significant prognostic factors for DSS also after adjusting for confounders. NLR, GPS, and ACBS were independently associated with decreased survival; however, ACBS was significantly superior to NLR (P=.02) and GPS (P=.002). These findings were validated in the randomly assigned validation cohort of 415 patients. In the pooled data of 818 patients, the ACBS performed better than GPS and NLR. ACBS 2 was independently associated with decreased DSS compared to ACBS 0, hazard ratio 2.3[95% confidence interval: 1.5-3.5], P<.001. CONCLUSION: Patients with abnormal values in more than one serum biomarkers had a significant additional risk of dying compared to patients with only one abnormal value. ACBS was validated as an independent prognostic factor that is superior to both NLR and GPS.

Factors associated with reduced functional outcome and quality of life in patients having limb-sparing surgery for soft tissue sarcomas - a national multicenter study of 128 patients.

BACKGROUND: Limb-sparing surgery for sarcomas has become possible in most cases. However, the impact of the procedure on the functional outcome has only been investigated in a few studies. The aim of this study has been to identify tumor- and patient-related factors associated with reduced functional outcome and quality of life after limb-sparing surgery in soft tissue sarcoma patients. MATERIAL AND METHODS: In total, 128 patients (mean age = 58, female/male = 54/74) who were treated with limb-sparing surgery without bone resection for soft tissue sarcomas in Denmark during the period 1 January 2009 to 31 December 2011 were included. Patients were asked to participate at least one year after surgery, and patients who had experienced local recurrence or metastatic disease were excluded. The Toronto Extremity Salvage Score (TESS) measured functional disability, while the Musculoskeletal Tumor Society Score (MSTS) measured functional impairment. European Organisation for Research and Treatment of Cancer (EORTC) QLQ-C30 measured quality of life. Tumor- and patient-related factors (age, gender, tumor depth, tumor size, malignancy, comorbidity, location, and radiotherapy) were extracted from the Danish National Sarcoma Database. Wilcoxon rank-sum test and Kruskal-Wallis were used for univariable analysis. Adjusted odds ratios were estimated by using multiple logistic regression models. RESULTS: In the multiple regression analysis, it was found that female gender (p = 0.03), lower extremity tumors (p < 0.01) and radiotherapy (p = 0.02) resulted in an increased risk of a lower TESS score. Initial reduced postoperative function was found to be associated with a lower functional outcome. Patients with reduced functional outcome have increased risk for reduced quality of life (p < 0.01). CONCLUSION: The results of this study show that patient- and tumor-related factors have an important role in the functional outcome.

Imaging investigations before referral to a sarcoma center delay the final diagnosis of musculoskeletal sarcoma.

Background and purpose - The use of point-of-care or local investigations before referral to specialist sarcoma centers as part of a fast-track diagnostic pathway varies, and may affect the time to diagnosis. We wanted to investigate differences in time intervals and proportion of malignancy in patients who were referred after initial diagnostic investigations were performed locally and in patients who were referred without these investigations. Patients and methods - We included 545 consecutive patients who were referred to Aarhus Sarcoma Center for suspected musculoskeletal sarcoma. Data on time intervals and investigations performed were collected from questionnaires and patient records. Patients who were referred from outside Aarhus uptake area after initial MRI/CT or histology performed locally were compared with patients who were referred from Aarhus uptake area without these investigations. Results - The median total interval from first symptom to diagnosis was 166 days for outside patients referred with MRI/CT or histology, which was 91 (95% CI: 76-106) days longer than for local patients who were referred without MRI/CT or histology. Comparing the same groups, the median diagnostic interval was 41 (95% CI: 30-51) days longer for outside patients including both primary care and hospital intervals. Both the proportion of malignancies (38% vs. 14%) and the proportion of sarcomas (24% vs. 7%) were higher in the outside group referred with MRI/CT or histology than in the local group without MRI/CT or histology. Interpretation - Pre-referral investigations at a local hospital increased the diagnostic interval by at least 1 month for 50% of the patients, and the proportion of malignancy was more than doubled-to almost 40%. If investigations are to be performed before referral to a sarcoma center, they should be part of the fast-track pathway in order to ensure timely diagnosis.

The Prognostic Value of Serum Biomarkers in Localized Bone Sarcoma.

OBJECTIVE: Certain biomarkers such as the C-reactive protein, serum albumin, and the neutrophils to lymphocyte ratio are of prognostic significance regarding survival in different types of cancers. Data from sarcoma patients are sparse and mainly derived from soft tissue sarcoma and/or metastatic cases. Adjusting for confounders such as comorbidity and age is an essential safeguard against erroneous conclusions regarding the possible prognostic value of these biomarkers. The aim of this study was to assess the prognostic value of a battery of pretreatment biomarkers in the serum of patients with localized bone sarcomas and to adjust for potential confounders. MATERIAL AND METHODS: All patients diagnosed with localized intermediate and high-grade bone sarcoma during 1994 to 2008 were extracted from the Aarhus Sarcoma Registry. The serum levels of albumin, C-reactive protein, hemoglobin, neutrophils, lymphocytes, and sodium were collected from the patient records. The prognostic values of overall and disease-specific mortality were tested for each individual biomarker as well as for the Glasgow prognostic score (GPS) and for a new composite score incorporating five biomarkers (Aarhus composite biomarker score: ACBS). Adjustments were made for comorbidity as well as other possible prognostic factors, such as size, histological type, margin, chemotherapy, and soft tissue extension, using the Cox proportional hazard model. RESULTS: A total of 172 patients with high- or intermediate-grade localized bone sarcoma were included. Of these patients, 63 were diagnosed with chondrosarcoma and 109 patients with Ewing/osteosarcoma. The median age was 55 years for chondrosarcoma and 19 years for Ewing/osteosarcoma patients. The overall 5-year mortality was 31% [95% confidence interval (CI): 21-44] and 41% (95% CI: 33-51), whereas the 5-year disease-specific mortality was 21% (95% CI: 12-34) and 39% (95% CI: 31-49) for chondrosarcoma and Ewing/osteosarcoma, respectively. Comorbidities were present in 12% of the Ewing/osteosarcoma patients and in 24% of the chondrosarcoma patients. After adjustment for comorbidity and other confounders, it was found that elevated levels of CRP, low hemoglobin, low sodium, high GPS, and high ACBS were associated with increased overall mortality. Furthermore, elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were associated with increased disease-specific mortality. CONCLUSION: Elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were all independent prognostic factors for both overall and disease-specific mortality. ACBS is a new three-level score of five biomarkers, but its value has to be confirmed in an independent data set.

Characteristics of 64 sarcoma patients referred to a sarcoma center after unplanned excision.

BACKGROUND AND METHODS: Unplanned excision of sarcoma before referral to specialist centers can affect prognosis and surgical outcome. The diagnostic pathway of these patients is uncertain and needs to be reviewed. We aimed to describe patient and tumor characteristics, initial symptoms, initial and final diagnosis, and explore reasons for unplanned excision in this patient group. From a previous study on 258 sarcoma patients, we identified 64 patients referred after surgery. Medical records were reviewed. RESULTS: The majority were soft tissue sarcomas, most often with thoracic location. Leiomyosarcoma was the most frequent final diagnosis, lipoma, and fibroma/dermatofibroma the most frequent initial diagnoses. Fifty percent were superficial small tumors, and 60.9% had not received diagnostic imaging before surgery. Fifty percent were referred from public surgical departments, and 1/3 from private specialists. Twenty-three patients had initial presence of alarm symptoms registered before surgery, the remaining 2/3 fell outside referral criteria or alarm symptoms were not discovered. CONCLUSIONS: Patients referred after unplanned excision often have small superficial tumors and the majority fall outside of defined referral criteria. Referral criteria are not a guarantee for detection of all sarcomas and surgeons should always be aware of the possibility of malignancy when removing a tumor.

Routes to Diagnosis for Suspected Sarcoma: The Impact of Symptoms and Clinical Findings on the Diagnostic Process.

Background and Objectives. Sarcoma patients often experience delay before diagnosis. We examined the association between presenting symptoms/signs and time intervals for suspected sarcoma patients. Methods. 545 consecutive patients suspected for sarcoma referred over a one-year period were included. Median time intervals in routes to diagnosis were collected from medical records and questionnaires. Results. 102 patients (18.7%) had a sarcoma; 68 (12.5%) had other malignancies. Median interval for the patient (time from first symptom to first doctor visit), primary care, local hospital, sarcoma center, diagnostic, and total interval for sarcoma patients were 77, 17, 29, 17, 65, and 176 days, respectively. Sarcoma patients visited more hospital departments and had longer median primary care (+10 days) and diagnostic intervals (+19 days) than patients with benign conditions. Median primary care (-19 days) and sarcoma center (-4 days) intervals were shorter for patients with a lump versus no lump. Median patient (+40 days), primary care (+12 days), diagnostic (+17 days), and total intervals (+78 days) were longer for patients presenting with pain versus no pain. GP suspicion of malignancy shortened local hospital (-20 days) and total intervals (-104 days). Conclusions. The main part of delay could be attributed to the patient and local hospitals. Length of time intervals was associated with presenting symptoms/signs and GP suspicion.

Computed tomography-guided radiofrequency ablation is a safe and effective treatment of osteoid osteoma located outside the spine.

INTRODUCTION: The aim of this study was to evaluate the long-term clinical outcome after computed tomography (CT)-guided radiofrequency ablation (RFA) in patients diagnosed with osteoid osteoma (OO) located in the upper and lower extremities. METHODS: The study population included 52 patients with a typical clinical history and radiologically confirmed OO who received CT-guided RFA treatment from 1998 to February 2014 at Aarhus University Hospital, Denmark. The clinical outcome was evaluated based on patient-reported outcome measures and medical record review. RESULTS: The response rate was 52/60 (87%). Pain relief after the first RFA treatment was found in 46/52 (88%) of the patients and after re-RFA in 51/52 (98%) of the patients. One patient underwent open resection after RFA. No major complications occurred, and four patients reported minor complications in terms of small skin burn, minor skin infection and hypoaesthesia at the entry point. In all, 50 of 52 (96%) patients reported to be "very satisfied" with the RFA treatment. CONCLUSION: CT-guided RFA is a safe and effective treatment with high patient satisfaction and it provides robust pain relief and improves the patients' quality of life. RFA should be the treatment of choice for most OO. FUNDING: not relevant. TRIAL REGISTRATION: The Danish Data Protection Agency approved the project with record number 2007-58-0010.

The importance of standardized treatment in high-grade osteosarcoma: 30 years of experience from a hospital-based database.

BACKGROUND: Treatment of high-grade osteosarcoma remains a major challenge in orthopedic oncology as no major breakthrough in overall survival has occurred in the past 20 years. Due to the rarity of the disease, comparing the results of a single institution to best standard practice needs the establishment of clinical databases. The aim of this study was to report the cumulative 30-years' experience of a single institution and to assess the incidence, survival and prognostic factors of high-grade osteosarcoma using a recently validated, hospital-based database, representing all citizens living in western Denmark, the Aarhus Sarcoma Registry. MATERIAL AND METHODS: Between 1979 and 2008, 169 patients were treated at the Sarcoma Centre of Aarhus University Hospital for high-grade osteosarcoma. The incidence was calculated as a WHO age-standardized incidence per million per year. The endpoint was overall survival, analyzed by the Kaplan-Meier method and log-rank. Possible prognostic factors were analyzed by the uni- and multivariate Cox proportional hazard method. RESULTS: The incidence of high-grade osteosarcoma in western Denmark from 1979 to 2008 was 2.7/million inhabitants/year. The five-year overall survival was 42% (95% CI 34; 49) for the whole cohort of patients with high-grade osteosarcoma and 54% (95% CI 43; 64) for patients with localized disease treated with wide excision and chemotherapy. For patients treated with curative intent, no soft tissue extension, treatment with sufficient surgical margin and standard chemotherapy, as well as a high degree of necrosis after chemotherapy were all independent prognostic factors for overall survival. CONCLUSION: The data from this hospital-based, validated database confirms the relevance of the known prognostic factors of high-grade osteosarcoma and emphasizes the importance of adequate surgical margins and chemotherapy.

Relative mortality in soft tissue sarcoma patients: a Danish population-based cohort study.

BACKGROUND: Cancer-specific survival estimates rely on precise and correct data on the cause of death; however, these data can be difficult to acquire, particularly in elderly patients where comorbidity is common. Furthermore, while some deaths are directly related to cancer, others are more complex, with cancer merely contributing. Another, more precise, method is to assess the relative mortality, i.e., mortality in patients compared to the general population. The aims of this study were to describe the relative mortality in soft tissue sarcoma, and to compare the relative mortality with the cancer-specific mortality. METHODS: We included 1246 patients treated for soft tissue sarcoma and 6230 individually age- and sex-matched individuals from the general population. The relative mortality was estimated as rates, and rate ratios adjusted for comorbidity. Mortality rate ratios were computed using the Cox proportional hazard model for 0-5 years and 5-10 years, according to age, sex and level of comorbidity. The cancer-specific mortality was compared to the corresponding relative mortality. RESULTS: The overall 5- and 10-year relative mortality was 32.8% and 36.0%. Patients with low-grade soft tissue sarcoma did not have increased mortality compared with the general population. Soft tissue sarcoma patients had a 4.4 times higher risk of dying within the first five years after diagnosis and a 1.6 times higher risk between five and ten years compared with the general comparison cohort. The relative mortality varied according to age, grade, stage at diagnosis, and level of comorbidity, being highest in younger patients and in patients without comorbidity. The overall 5- and 10-year cancer-specific mortality was underestimated by 1.5 and overestimated by 0.7 percentage points compared to the relative mortality, respectively. No statistical significant difference between the relative and the cancer-specific mortality was found. CONCLUSION: The relative mortality provides an unbiased and accurate method to differentiate between cancer-specific and non-cancer-specific deaths. However, when data on the cause of death is of a sufficient quality, there is no difference between relative mortality and disease-specific mortality based on death certificates.