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Background: The improved outcome of extremity soft tissue sarcoma patients surgically treated until 2007 at the authors' institution was previously reported. This study updates the analysis at a later follow-up and extends the patients' cohort to assess changes in outcomes over time for extremity and superficial trunk soft tissue sarcoma (ESTSTS) treated at a single referral center., Methods: All consecutive patients with primary localized adult-type ESTSTS surgically treated at the authors' institution between 1987 and 2017 were included and divided into group 1 (1987-2002) and group 2 (2003-2017) according to primary surgery year. Crude cumulative incidence (CCI) of sarcoma-specific mortality (SSM), local recurrence (LR), and distant metastases (DM) were calculated in a competing-risks framework. DM-free survival (DMFS) and post-DM survival were also assessed., Results: The study identified 2382 patients. The median follow-up was 104 months (range, 63-127 months), and the post-DM follow-up was 76 months (range, 37-126 months). Since 2003, an increased adoption of preoperative treatments was observed: the use of chemotherapy, radiotherapy and combined chemoradiotherapy went from 10.5% to 23.7%, from 1.7% to 17.8%, and from 1% to 11.8% respectively. This change in treatment strategies was associated to an improvement in CCI-SSM (27.8% vs 19.5%; P < 0.001), CCI-LR (14.1 vs 7.5%; P < 0.001), DMFS (57.9% vs 65.8%; P = 0.004), and post-DM (12.2% vs 20.1%; P = 0.012), but not in CCI-DM., Conclusions: Increased adoption of preoperative treatments and greater availability of medical agents in the recent years were associated to better outcomes. New treatments are eagerly awaited for further improvement of outcome for ESTSTS patients because no major changes have been observed since 2003., (© 2022. Society of Surgical Oncology.)

Purpose: The need for systematic reexcision in patients who underwent unplanned excision (UE) for extremity and superficial trunk soft tissue sarcoma (ESTSTS) has been questioned. We investigated the outcome of patients who underwent reexcision for ESTSTS compared with primarily resected at our institution and the prognostic impact of microscopic residual disease (MR) in the reexcision specimen., Methods: Primary ESTSTS patients surgically treated at our institution between 1997 and 2017 were divided in three groups: primarily resected (A), reexcised after macroscopically complete UE (B), and incomplete UE (C). Weighted overall survival (OS), crude cumulative incidence of local relapse (CCI-LR), and distant metastasis (CCI-DM) were calculated and compared. In group B, multivariable models were performed to assess factors associated with the outcomes., Results: A total of 1962 patients were identified: 1076, 697 and 189 in groups A, B, and C, respectively. Overall median follow-up was 85 months. Seven-year weighted-OS was 73.8%, 84.1%, and 80.7% (p < 0.001) for groups A, B, and C respectively. Seven-year CCI-LR and DM were 5.0% and 25.3%, 12.1% and 15.8%, and 13.6% and 29.4% (both p < 0.001) for groups A, B, and C, respectively. At multivariable analysis, the presence MR was associated with LR (p < 0.001) but not with OS nor CCI-DM., Conclusions: UE and the presence of MR at pathology in reexcision specimen are associated to a higher risk of LR but not to a higher risk of DM or lower OS. After macroscopic complete UE, postponing reexcision until a LR occurs may be considered on an individualized basis.

Previous data have indicated that organ-specific and non-organ-specific autoimmune diseases may occur in the same patient. We report here our study on the type and prevalence of endocrine autoimmune diseases in undifferentiated connective tissue disease (UCTD). A retrospective analysis revealed five out of 75 UCTD cases (6.6%) with cytology-verified autoimmune thyroiditis (associated with insulin-dependent diabetes mellitus in one case). Other UCTD patients had Graves' disease (one case), non-toxic multinodular goitre (two cases) and central hypothyroidism (one case). In a prospective study, thyroid function was evaluated in 15 consecutive UCTD patients with neither clinical nor laboratory signs of thyroid involvement. Basal and post-TRH stimulation TSH levels were significantly higher in UCTD patients than in healthy subjects.

Connective tissue diseases (CTDs) are chronic multisystemic inflammatory disorders whose indicative signs or symptoms have a high sensitivity but poor specificity in predicting the evolution into a given CTD. We have analysed 84 consecutive patients initially diagnosed as having an early undifferentiated CTD (early UCTD) with the aim of verifying the evolution into one definite CTD and of evaluating the predictive value of clinical and laboratory parameters. During a 5-year study period, 33 patients developed signs of a full-blown CTD; the highest probability of evolution was in the first 48 months after the onset. Multivariate analysis allowed us to select those variables correlating with evolution into a particular CTD, such as sclerodactyly and oesophageal dysfunction for systemic sclerosis, xerostomia and anti-nuclear antibodies (SS-A pattern) for Sjögren's syndrome, and fever and anti-DNA antibodies for systemic lupus erythematosus. Furthermore, we assessed the prevalence of various clinical and laboratory manifestations, complications and prognosis of those patients diagnosed after a 5-year disease duration period as having a UCTD. In our series, major organs such as the kidney or heart seem to be spared, whereas we detected a relatively high prevalence of endocrine disease of autoimmune origin.