Solitary enchondromas-diagnosis and surveillance : Danish guidelines.

Enchondromas (EC) are frequent incidental findings on magnetic resonance imaging (MRI) performed for the diagnosis of joint pathology, especially observed on MRI examinations of the knee and shoulder. Enchondroma has potential for malignant transformation to chondrosarcoma (CS), and it may be difficult to distinguish EC from low-malignant CS on the basis of imaging or histopathology studies. Therefore, EC is mostly followed up to monitor any growth and/or changes indicating aggressive tumor.There is no consensual evidence on when and how to follow up patients with EC with regard to potential malignant transformation. Therefore, the Danish Multidisciplinary Cancer Groups initiated and supported the elaboration of Danish guidelines in 2020 based on a literature review. The guidelines are presented here, in addition to a summary of the background literature.

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.

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.

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.

Cancer Patient Pathways shortens waiting times and accelerates the diagnostic process of suspected sarcoma patients in Denmark.

Cancer Patient Pathways (CPPs) for suspected cancer were implemented in Denmark to reduce waiting times for cancer diagnosis and treatment. Our study describes developments in time intervals and tumour size in a natural experiment before and after implementation of the CPP for sarcomas (January 1st, 2009). Medical files for patients referred with suspected sarcoma from other hospitals to Aarhus Sarcoma Centre during 2007-2010 (n=1126) were reviewed for data on milestones, time intervals, performed diagnostics, and tumour size. Results showed a statistically significant reduction in median number of work days in the phase "referral to first appointment" for all patients. For bone sarcomas, median time was significantly reduced from 11 to five work days in the phase "first appointment to decision of treatment", for soft tissue sarcomas it was reduced from 28 to 18 work days in the phase "referral to start of treatment". Passive waiting time was reduced, and delays in the fast-track programme were caused mostly by supplementary diagnostics. Median tumour size for soft tissue sarcomas was reduced from 7.0 to 4.9cm, possibly a secondary effect of increased awareness. CPPs have accelerated the diagnostic process for sarcomas, and our results may aid international development of similar initiatives.

[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.