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.

Metastatic pattern, local relapse, and survival of patients with myxoid liposarcoma: a retrospective study of 45 patients.

Purpose. To assess the metastatic pattern of the histological subtype myxoid liposarcoma (MLS) with no or few round cells. Methods. Forty-five patients (F/M = 27/18, mean age 49 (range 17-85) years) were diagnosed with MLS at two Danish sarcoma centres in the period 1995-2004. A retrospective review of patients' files combined with an extraction of survival data from the Danish Centralised Civil Register was performed. Results. Seven patients had distant metastases during the observation period. Two patients had metastases at the time of diagnosis, while metastases occurred within 2.5 years in four patients, and in one patient 11.9 years after primary diagnosis. All metastases occurred at extrapulmonary sites. The first local relapse occurred within 3 years after surgery in six patients, in one patient after 4.0 years, and in one patient 7.7 years after surgery. The 5- and 10-year overall survival was 80% and 69%, respectively. Both the 5- and 10-year distant metastases-free survival was, respectively, 86%. The 5- and 10-year local relapse-free survival was, respectively, 83% and 80%. Conclusions. Patients with MLS had only extra-pulmonary metastases, and no lung metastases were found. Most local relapses and distant metastases occurred within the first 2-3 years after surgery.

External validation of the Bayesian Estimated Tools for Survival (BETS) models in patients with surgically treated skeletal metastases.

BACKGROUND: We recently developed two Bayesian networks, referred to as the Bayesian-Estimated Tools for Survival (BETS) models, capable of estimating the likelihood of survival at 3 and 12 months following surgery for patients with operable skeletal metastases (BETS-3 and BETS-12, respectively). In this study, we attempted to externally validate the BETS-3 and BETS-12 models using an independent, international dataset. METHODS: Data were collected from the Scandinavian Skeletal Metastasis Registry for patients with extremity skeletal metastases surgically treated at eight major Scandinavian referral centers between 1999 and 2009. These data were applied to the BETS-3 and BETS-12 models, which generated a probability of survival at 3 and 12 months for each patient. Model robustness was assessed using the area under the receiver-operating characteristic curve (AUC). An analysis of incorrect estimations was also performed. RESULTS: Our dataset contained 815 records with adequate follow-up information to establish survival at 12 months. All records were missing data including the surgeon's estimate of survival, which was previously shown to be a first-degree associate of survival in both models. The AUCs for the BETS-3 and BETS-12 models were 0.79 and 0.76, respectively. Incorrect estimations by both models were more commonly optimistic than pessimistic. CONCLUSIONS: The BETS-3 and BETS-12 models were successfully validated using an independent dataset containing missing data. These models are the first validated tools for accurately estimating postoperative survival in patients with operable skeletal metastases of the extremities and can provide the surgeon with valuable information to support clinical decisions in this patient population.

[Surgical treatment of skeletal metastases in the pelvis and extremities]. / Operativ behandling af knoglemetastaser i baekken og ekstremiteter.

INTRODUCTION: In choosing a surgical treatment for skeletal metastases, an assessment of the individual patient's prognosis fis essential. The aim of surgical treatment is to alleviate pain and maintain function, thus improving the patient's quality of life. We analysed the survival of patients with skeletal metastases who had been surgically treated at the University Hospital in Aarhus from September 1999 to March 2003. MATERIALS AND METHODS: A consecutive series of 87 of 90 operations for non-spinal skeletal metastases was done. Three percent of the patients were operated on for more than one metastasis. Carcinomas of the breast, prostate, kidney and lung were the dominating primary tumors. RESULTS: The survival rate was 0.35 at 1 year, 0.22 at 2 years and 0.14 at 3 years. Univariate analysis showed that survival was related to sex, bone localization, skeletal metastatic load, the presence of visceral metastases, Karnofsky performance score, primary tumor type, presence of a complete pathological fracture and preoperative haemoglobin content. Multivariate regression analysis showed that pathological fracture, haemoglobin content < 7 mmol/l and a poor Karnofsky score were negative prognostic factors for survival. Breast cancer was the sole independent positive prognostic factor for survival. DISCUSSION: Most patients with two or three independent negative prognostic factors have an extremely short survival time, whereas patients with no negative prognostic factors may have a good prognosis. Surgical and reconstruction procedures should be chosen to minimise the risk of long-term failure in patients with a good prognosis and be as simple as possible in patients with a poor prognosis.

[Surgical treatment of giant cell tumor of bone]. / Kirurgisk behandling af kaempecelletumorer i knogler.

INTRODUCTION: This study analyses a 5-year follow-up after treatment of giant cell tumour of bone during a 31-year period. MATERIAL AND METHODS: Sixty patients with giant cell tumours of bone had been treated at the Centre for Musculoskeletal Tumours at Aarhus University Hospital. All patients were followed at regular intervals for 5-35 years, median = 11 years. RESULTS: There were 54 benign giant cell tumours of bone, whereas six were classified as malignant. The most common location was around the knee. There were nine cases of recurrence. The 5-year recurrence-free survival was 77%. The lowest frequency of recurrence appeared after resection (6%) and the highest after bone-transplantation (22%). DISCUSSION: The classical microscopic and macroscopic criteria for malignancy are not applicable for this rare tumour. All giant cell tumours of bone should be considered as potentially malignant, and therefore patients should be referred to a Centre for Musculoskeletal Tumours.