Active surveillance of diffuse-type tenosynovial giant cell tumors: A retrospective, multicenter cohort study.

BACKGROUND: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a mono-articular, soft-tissue tumor. Although it can behave locally aggressively, D-TGCT is a non-malignant disease. This is the first study describing the natural course of D-TGCT and evaluating active surveillance as possible treatment strategy. METHODS: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected. RESULTS: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment. CONCLUSION: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted.

One-Stage Synovectomies Result in Improved Short-Term Outcomes Compared to Two-Stage Synovectomies of Diffuse-Type Tenosynovial Giant Cell Tumor (D-TGCT) of the Knee: A Multicenter, Retrospective, Cohort Study.

Diffuse-type tenosynovial giant cell tumors' (D-TGCTs) intra- and extra-articular expansion about the knee often necessitates an anterior and posterior surgical approach to facilitate an extensive synovectomy. There is no consensus on whether two-sided synovectomies should be performed in one or two stages. This retrospective study included 191 D-TGCT patients from nine sarcoma centers worldwide to compare the postoperative short-term outcomes between both treatments. Secondary outcomes were rates of radiological progression and subsequent treatments. Between 2000 and 2020, 117 patients underwent one-stage and 74 patients underwent two-stage synovectomies. The maximum range of motion achieved within one year postoperatively was similar (flexion 123-120°, p = 0.109; extension 0°, p = 0.093). Patients undergoing two-stage synovectomies stayed longer in the hospital (6 vs. 4 days, p < 0.0001). Complications occurred more often after two-stage synovectomies, although this was not statistically different (36% vs. 24%, p = 0.095). Patients treated with two-stage synovectomies exhibited more radiological progression and required subsequent treatments more often than patients treated with one-stage synovectomies (52% vs. 37%, p = 0.036) (54% vs. 34%, p = 0.007). In conclusion, D-TGCT of the knee requiring two-side synovectomies should be treated by one-stage synovectomies if feasible, since patients achieve a similar range of motion, do not have more complications, but stay for a shorter time in the hospital.

The route to diagnosis of sarcoma patients: Results from an interview study in the Netherlands and the United Kingdom.

INTRODUCTION: Sarcomas are rare tumours. Early diagnosis is challenging, but important for local control and potentially survival and quality of life(QoL). We investigated (1)the route to diagnosis (RtD) experienced by sarcoma patients, including factors contributing to the length of the RtD from patients' perspective; (2)the impact of the RtD on QoL and care satisfaction; and (3)differences in aims 1-2 between English and Dutch patients. METHODS: Fifteen sarcoma patients from The Royal Marsden Hospital, United Kingdom, and Radboud University Medical Centre, The Netherlands, were interviewed, exploring RtD experiences. Interviews were analysed according to qualitative content analysis. RESULTS: The main themes were: patient interval, diagnostic interval, reflection on the RtD and recommendations for improvement. Patient interval was long if symptoms were attributed as benign, did not interfere with daily life or were expected to cease. An incorrect working diagnosis, ineffective process of additional investigations, long referral times and lack of a lead clinician lengthened the diagnostic interval. Long waiting times, false reassurance and inadequate information provision led to dissatisfaction and a high emotional burden. Factors for improvement included increasing awareness of patients and healthcare providers, empowering patients, and having a lead clinician. CONCLUSION: The RtD of sarcoma patients is complex. Increasing awareness of patients and healthcare providers may contribute to shorten the RtD.

IGF signaling pathway analysis of osteosarcomas reveals the prognostic value of pAKT localization.

AIM: The aim of this study was to examine the expression of the IGF signaling pathway components in osteosarcoma samples before and after chemotherapy with special emphasis on their prognostic value. MATERIALS & METHODS: Tumor material and follow-up data of 58 osteosarcoma patients were analyzed. Immunohistochemical staining was carried out to identify proteins related to the IGF pathway. Changes in protein expression during treatment, correlations between proteins and subsequent influence on survival were tested. RESULTS: Proteins of the IGF signaling system are widely expressed in osteosarcoma samples. We demonstrate a change in expression of intracellular pathway proteins after chemotherapy. Remarkably, cytoplasmic pAKT, but not nuclear pAKT, is associated with poor survival. CONCLUSION: IGF pathway proteins seem to be widely activated in osteosarcoma, but their expression changes after chemotherapy. This has implications for the timing of both measuring target expression and pathway interference. Our observations on the prognostic value of cytoplasmic pAKT warrant further investigation while considering the introduction of AKT inhibitors for osteosarcoma treatment.