Aim. The aim of this study was to assess the incidence of low-grade fibromyxoid sarcoma (LGFMS), present treatment results of metastatic LGFMS, and investigate the clinical significance of the FUS gene rearrangement. Methods. This study included 14 consecutive LGFMS patients treated at the Aarhus Sarcoma Centre in 1979–2010. Fluorescent in situ hybridization (FISH) analysis for FUS break-apart was performed for all patients. Results. The incidence of LGFMS was 0.18 per million, representing 0.6% of all soft tissue sarcomas. Four patients needed multiple biopsies/resections before the correct diagnosis was made. Four patients experienced local recurrence, and three patients developed metastases. The treatment of metastatic LGFMS varied from multiagent chemotherapy to repeated, selective surgery of operable metastases. The best response to chemotherapy was short-term stabilization of disease progression, seen with Trabectedin. The prevalence of the FUS break-apart was 21.4%. We found no significant difference in clinical characteristics and outcomes in correlation with the FUS break-apart. Conclusion. LGFMS is a rare disease with multiple challenges. The FUS break-apart was not associated with local recurrence or metastases in our study. To date the only treatment resulting in disease-free periods is surgery; however further investigation into the management of metastatic LGFMS is necessary.

No abstract

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.

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.

Background and purpose —The Danish Cancer Patient Pathway for sarcoma defines a set of alarm symptoms as criteria for referral to a sarcoma center. This may exclude cancer patients without alarm symptoms, so we investigated the presence of alarm symptoms (defined as being indicative of a sarcoma) in patients who had been referred to the Aarhus Sarcoma Center.Patients and methods —We reviewed the medical records of all 1,126 patients who had been referred, with suspected sarcoma, from other hospitals in the period 2007–2010 for information on symptoms, clinical findings, and diagnosis. Alarm symptoms were analyzed for predictive values in diagnosing sarcoma.Results —179 (69%) of 258 sarcoma patients were referred with alarm symptoms (soft-tissue tumor > 5 cm or deep-seated, fast-growing soft-tissue tumor, palpable bone tumor, or deep persisting bone pain). The remaining 79 sarcomas were found accidentally. “Size over 5 cm” for soft-tissue tumors, and “deep persisting bone pain” for bone tumors had the highest sensitivity and positive predictive value. Of the 79 sarcoma patients who were referred without alarm symptoms, 7 were found accidentally on imaging, 5 were referred with suspected recurrence of a sarcoma, 64 were referred with a confirmed histological diagnosis, and 3 were referred for other reasons.Interpretation —Defined alarm symptoms are predictive of sarcoma, but one-third of the patients were found accidentally. Further studies on presenting symptoms in primary care are needed to assess the true value of alarm symptoms.

Background and purposeThe 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 methodsWe 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.ResultsThe 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.InterpretationPre-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.