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Spindle cell rhabdomyosarcoma represents a rare neoplasm characterized by monomorphic spindle cells with a fascicular architecture and variable skeletal muscle differentiation. Following incidental identification of a ZFP64::NCOA3 gene fusion in an unclassified spindle cell sarcoma resembling adult-type fibrosarcoma, we performed a retrospective archival review and identified four additional cases with a similar histology and identical gene fusion. All tumors arose in adult males (28-71 years). The neoplasms were found in the deep soft tissues, two were gluteal, and one each arose in the thigh, abdominal wall, and chest wall. Morphologically, the tumors were characterized by spindle cells with a distinctive herringbone pattern and variable collagenous to myxoid stroma. The nuclei were relatively monomorphic with variable mitotic activity. Three tumors had immunoreactivity for MyoD1, and four contained variable expression of desmin and smooth muscle actin. All cases tested for myogenin, CD34, S100, pankeratin, and epithelial membrane antigen were negative. Targeted RNA sequencing revealed a ZFP64::NCOA3 fusion product in all five tumors. Three patients developed distant metastases, and two ultimately succumbed to their disease within 2 years of initial diagnosis. This study suggests ZFP64::NCOA3 fusions define a novel subtype of rhabdomyosarcoma with a spindle cell morphology and aggressive clinical behavior. The potential for morphologic and immunohistochemical overlap with several other sarcoma types underscores the value of molecular testing as a diagnostic adjunct to ensure accurate classification and management of these neoplasms.

Non‐osteogenic, non‐Ewing soft‐tissue sarcoma (NONE‐STS) of bone is a rare presentation of primary bone cancers. Optimal treatments and outcomes for this heterogenous group are poorly described. We evaluated the factors associated with long‐term outcomes in patients with this disease. Patients with localized NONE‐STS of bone treated at the Toronto Sarcoma Program from 1987 to 2017 were identified. Clinical characteristics, treatment, and survival information were collected. Kaplan‐Meier (log‐rank) survival estimates from the time of definitive surgery, with uni‐/multivariate analyses (Cox) of sarcoma‐specific survival were performed. A total of 106 patients (60.4% male; median age 46 years) with NONE‐STS of bone were identified. Common histologies included undifferentiated pleomorphic sarcoma [UPS]/malignant fibrous histiocytoma [MFH] (UPS/MFH, 41.5%), leiomyosarcoma (LMS, 20.8%), and fibrosarcoma (FS, 11.3%). Tumors were often high grade (59.4%) and involved the extremities (88.7%), with most receiving chemotherapy (67.9%) with cisplatin/doxorubicin‐based regimens (73.6%). In the full cohort, 10‐year DFS (45.7%, [95%CI: 35.7‐55.8%]), OS (53.4%, [95%CI: 41.7‐62.2%]), and SSS (63.9%, [95%CI: 53.9‐72.5%]) were moderate. Histology specific, 10‐year SSS was 70.7% [95%CI: 56.1‐85.5%] for UPS/MFH, 51.8% [95%CI: 29.8‐73.8%] for LMS, and 72.2% [95%CI: 45.1‐99.2%] for FS. Only UPS/MFH (n = 4) showed sarcoma‐related death >10 years. Multivariate analysis identified axial location (HR = 35.5, [95%CI: 3.4‐369.6]), high grade (HR = 16.9, [95%CI: 1.6‐185.1]), and disease relapse (HR = 485.1, [95%CI: 36.3‐6482.6]) as risk factors for death (p, In patients with Non‐osteogenic, non‐Ewing soft‐tissue sarcoma (NONE‐STS) of bone, long‐term survival is similar to that of osteosarcoma. Patients receiving chemotherapy who demonstrated ≥85% necrosis on pathologic specimens had improved survival, whereas those with UPS/MFH histology experienced sarcoma‐related death beyond 10 years.

Background Conventional primary pelvic chondrosarcoma often presents as a low- or intermediate-grade tumor in older patients. Although this is the most common variant of pelvic chondrosarcoma, studies examining treatment outcomes are lacking. The purpose of this study was to evaluate patients with these tumors to determine their outcomes of treatment. Methods Seventy-three patients (grade I [n = 19, 26%] and grade II [n = 54, 74%]) were reviewed including 55 (75%) males and 18 (25%) females, with a mean age of 51 (range, 17-81) years and follow-up of 9 ± 5 years. Results The 10-year disease-specific survival was 71%. Grade II disease (hazard ratio [HR], 6.74; P = .04) and age ≥50 years (HR, 3.97; P = .02) was associated with death due to disease. The 10-year local recurrence- and metastatic-free survival were 79% and 72%. Of the patients with a local recurrence (n = 11), 7 (64%) recurred at a higher histological grade. Patient age ≥50 years was associated with local recurrence (HR, 10.03; P = .02) and metastatic disease (HR, 4.20; P = .02). Conclusion Advancing patient age was an independent risk factor for worse survival and disease recurrence. Tumors often recurred locally at a higher grade and as such wide local excision remains the treatment of choice for these tumors.

BACKGROUND Local recurrence (LR) of sacral chordoma is a difficult problem and the mortality risk associated with LR remains poorly described. The purpose of this study was to evaluate the risk of mortality in patients with LR and determine if patient age is associated with mortality. METHODS A total of 218 patients (144 male, 69 female; mean age 59 ± 15 years) with sacrococcygeal chordomas were reviewed. Cumulative incidence functions and competing risks for death due to disease and nondisease mortality were employed to analyze mortality trends following LR. RESULTS The 10-year overall survival (OS) was 55%. Patients with LR had 44% 10-year OS, similar to patients without (59%; P = .38). The 10-year OS between those less than 55 compared with ≥55 years were similar (69% vs 48%; P = .52). The 10-year death due to disease was worse in patients with LR compared with those without (44% vs 84%; P

Background Soft-tissue sarcomas (STSs) are a heterogeneous group of malignant tumors that can be difficult to treat. This is particularly true after incomplete or unplanned excisions and especially for patients with American Joint Committee on Cancer stage III tumors, who are at high risk for relapse. Numerous studies have shown that an inadequate sarcoma excision is associated with a worse prognosis. However, other reports have suggested an improved prognosis for patients with an initial unplanned excision and subsequent re-excision in comparison with patients who undergo planned primary surgery. The purpose of this study was to determine the impact of an unplanned excision on treatment and subsequent oncologic and functional outcomes for patients with stage III extremity STS. Methods From the prospectively collected database at a tertiary-referral sarcoma center, all patients with stage III STS of the extremities treated between 1989 and 2010 were identified. Patient records were reviewed to identify patient demographics, tumor details, treatments, complications, and functional and oncologic outcomes. Results Five hundred patients with stage III STSs of the extremities were identified, and 94 of these patients (18.8%) were referred after inadequate excisions had been performed elsewhere. All 94 patients with unplanned excisions underwent re-excision in an attempt to achieve clear margins, and 83% of these patients had residual tumor in the re-excision specimen. In the re-excision group, the rates of plastic reconstruction (eg, skin grafts and rotational or free flaps) and amputation were significantly higher in comparison with the rates for patients who underwent a primary planned resection (P = .023 and P = .03, respectively). The rates of local recurrence, metastasis-free survival, and overall survival were not significantly different between the 2 groups, nor were the functional outcomes. Conclusions Unplanned excision of stage III STS leads to an unfavorable clinical course and necessitates more extensive surgery. As a result of aggressive re-excision and multidisciplinary treatment, a negative effect on oncologic outcomes cannot be confirmed.

There are limited data regarding the molecular characterization of undifferentiated pleomorphic sarcomas (UPS; formerly malignant fibrous histiocytoma). This study aimed to investigate the utility of next generation sequencing (NGS) in UPS to identify subsets of patients who harbour actionable mutations. Patients diagnosed with UPS underwent pathological re-evaluation by a pathologist specializing in sarcoma. Tumor DNA was isolated from archived fresh frozen tissue samples and genotyped using NGS with the Illumina MiSeq TruSeq Amplicon Cancer Panel (48 genes, 212 amplicons). In total, 95 patients initially classified with UPS were identified. Following pathology re-review the histological subtypes were reclassified to include: Myxofibrosarcoma (MFS, N = 44); UPS(N = 18); and Others (N = 27; including undifferentiated spindle cell sarcoma (N = 15) and dedifferentiated liposarcoma (N = 6)). Seven cases were excluded from further analysis for other reasons. Baseline demographics of the finalized cohort (N = 88) showed a median age of 66 years (32-95), primarily with stage I-III disease (92%) and high-grade (86%) lesions. Somatic mutations were identified in 31 cases (35%)(Total mutations = 36: solitary mutation(n = 27); two mutations( =n = 3); three mutations(n = 1)). The most commonly identified mutations were in TP53 (n = 24), ATM (n = 3) and PIK3CA (n = 2). Three of 43 patients with MFS and one of 18 patients with UPS had clinically relevant mutations, mainly related to biomarkers of prediction of response; however few had targetable driver mutations. Somatic mutation status did not influence disease free or overall survival. Based on the small number of clinically relevant mutations, these data do not support the routine use of targeted NGS panels outside of research protocols in UPS.

Background and Objectives The primary objective of this study was to estimate the change in health-related quality of life (HRQL) 1 year following treatment for extremity soft tissue sarcoma (STS), measured by the EQ-5D. Secondary objectives included determining clinical variables associated with HRQL at 1 year, estimating the proportion with a clinically important difference (CID) in HRQL, and evaluating variability within EQ-5D domains. Methods Patients over the age of 16 years, treated for a localized extremity STS, were included. The EQ-5D change score from pre-treatment to 1-year follow-up was determined. The association of clinical variables with EQ-5D scores was estimated using a linear regression model. The proportion of patients with a CID in HRQL score was determined. A vector analysis of the EQ-5D domains was undertaken. Results The mean EQ-5D change score was 0.02. Age, sex, disease status, and initial EQ-5D score were associated with EQ-5D score at 1 year. There was a CID improvement in 32% and a deterioration in 24%. The anxiety and depression domain demonstrated the most change between baseline and 1 year after treatment. Conclusion Most patients maintain a high level of HRQL following treatment for extremity STS. J. Surg. Oncol. 2016;114:821–827. © 2016 2016 Wiley Periodicals, Inc.

Background and Objectives To describe the surgical treatment of patients with a pathologic fracture through a benign tumor of the proximal femur to determine if there is a difference in local recurrence, complications or functional outcome compared to patients with tumors in the same location without pathologic fractures. Methods From 1989–2010, of 97 patients, 29 presented with a pathologic fracture (PF) through a proximal femoral benign bone tumor and 68 presented without a pathologic fracture (NPF). Outcomes of the two groups were compared in terms of surgical management, postoperative complications, local recurrence and functional scores. Results Fibrous dysplasia, giant cell tumor of bone and chondroblastoma were the most common subtypes. Most patients were managed with joint preservation in both PF (86.2%) and NPF (98.5%) groups (P = 0.03). Local recurrence risk was similar for patients in the PF (10.3%) and NPF (8.8%) groups. Mean follow-up was 105.7 months (P = 0.8). Functional outcome scores were high in both groups and not statistically significantly different. Conclusions The majority of pathologic fractures through a benign bone tumor of the proximal femur can be successfully treated with curettage, burring, bone grafting and internal fixation without increasing the risk of local recurrence or negatively impacting functional outcome. J. Surg. Oncol. © 2015 Wiley Periodicals, Inc.

BACKGROUND The objectives of this study were to evaluate the risk of local recurrence and survival after soft tissue sarcoma (STS) resection with positive margins and to evaluate the safety of sparing adjacent critical structures. METHODS One hundred sixty-nine patients with localized STS who had positive resection margins were identified from a prospective database. Patients who had positive margins were stratified into 3 groups, each representing a specific clinical scenario: critical structure positive margin (eg major nerve, vessel, or bone), tumor bed resection positive margin, and unexpected positive margin. The rates of local recurrence-free survival (LRFS) and cause-specific survival (CSS) were calculated and compared with relevant control patients who had negative margins after STS resection. RESULTS After planned close dissection to preserve critical structures, the 5-year LRFS and CSS rates both depended on the quality of the surgical margins (97% and 80.3%, respectively, for those with negative margins vs 85.4% and 59.4%, respectively, for those with positive margins; P = .015 and P = .05, respectively). Negative margins achieved through resection of critical structures because of tumor invasion or encasement only slightly improved the 5-year rates of LRFS (91.2%) and CSS (63.6%; P = .8 and P = .9, respectively). The lowest 5-year LRFS and CSS rates were 63.4% and 59.2%, respectively, after an unexpected positive margin during primary surgery. CONCLUSIONS After patients undergo resection of STS with positive margins, oncologic outcomes can be predicted based on the clinical context. Sparing adjacent critical structures in this setting is safe and contributes to improved functional outcomes. Cancer 2014;120:2866–2875. © 2014 American Cancer Society.

Management of soft tissue sarcoma involves multimodality treatment, including surgery and radiotherapy. Pathologic fracture of the femur after such treatment in the thigh is one serious, late complication and nonunion rates of 80-90% are reported. We hypothesize that the combination of radiotherapy and periosteal stripping (during tumor resection) leads to greater impairment of the fracture repair process than either intervention alone. Female Wistar retired breeder rats were randomized into four treatment groups (control, radiotherapy, surgery, and combination of radiotherapy and surgery) and three end-points (21, 28, and 35 days post-fracture). Designated animals first underwent radiotherapy, followed by surgical stripping of the periosteum 3 weeks later and femoral fracture with fixation after another 3 weeks. Animals were sacrificed and fractures examined using microCT and histomorphometry. Simple transverse or short oblique femoral fractures were produced. By 35 days, control animals formed unions, periosteum-stripped animals formed hypertrophic non-unions and irradiated animals formed atrophic non-unions. Histomorphometry revealed an absence of chondroid and osteoid production in animals undergoing radiotherapy. The relative contribution of periosteal stripping to occurrence of non-union was statistically insignificant. Radiation prior to fracture reliably resulted in atrophic non-union in our model. The contribution of periosteal stripping was negligible.

BACKGROUND: This study sought to determine if preoperative image-guided intensity-modulated radiotherapy (IG-IMRT) can reduce morbidity, including wound complications, by minimizing dose to uninvolved tissues in adults with lower extremity soft tissue sarcoma. METHODS: The primary endpoint was the development of an acute wound complication (WC). IG-IMRT was used to conform volumes to avoid normal tissues (skin flaps for wound closure, bone, or other uninvolved soft tissues). From July 2005 to June 2009, 70 adults were enrolled; 59 were evaluable for the primary endpoint. Median tumor size was 9.5 cm; 55 tumors (93%) were high-grade and 58 (98%) were deep to fascia. RESULTS: Eighteen (30.5%) patients developed WCs. This was not statistically significantly different from the result of the National Cancer Institute of Canada SR2 trial (P = .2); however, primary closure technique was possible more often (55 of 59 patients [93.2%] versus 50 of 70 patients [71.4%]; P = .002), and secondary operations for WCs were somewhat reduced (6 of 18 patients [33%] versus 13 of 30 patients [43%]; P = .55). Moderate edema, skin, subcutaneous, and joint toxicity was present in 6 (11.1%), 1 (1.9%), 5 (9.3%), and 3 (5.6%) patients, respectively, but there were no bone fractures. Four local recurrences (6.8%, none near the flaps) occurred with median follow-up of 49 months. CONCLUSIONS: The 30.5% incidence of WCs was numerically lower than the 43% risk derived from the National Cancer Institute of Canada SR2 trial, but did not reach statistical significance. Preoperative IG-IMRT significantly diminished the need for tissue transfer. RT chronic morbidities and the need for subsequent secondary operations for WCs were lowered, although not significantly, whereas good limb function was maintained. Cancer 2013. © 2013 American Cancer Society.

BACKGROUND: The treatment of diffuse tenosynovial giant cell tumor (TGCT) requires extensive surgical resection of the hypertrophic synovium and multiple soft tissue masses yet still may result in high rates of local failure. The authors of this report examined their experience in treating patients with advanced/multiply recurrent TGCT with a combination of surgery and external-beam radiotherapy. METHODS: Fifty patients who were treated for TGCT with radiotherapy and surgery from 1972 to 2006 were identified. Patient demographics, radiotherapy treatment parameters, surgical treatment, and oncologic and functional outcomes were evaluated. All patients had pathologic review at presentation and required at least 1 year follow-up. RESULTS: Forty-nine patients had diffuse TGCT with both intra-articular and extra-articular disease (1 had malignant TGCT). Twenty-eight patients (56%) were referred after at least 1 local recurrence. Thirty patients (60%) underwent at least 2 operations before radiotherapy. The mean dose of radiation delivered was 39.8 gray. At a mean follow-up of 94 months (range, 19-330 months), 47 patients (94%) had not developed a recurrence or had stable disease/signal characteristics on serial cross-sectional imaging (for those patients who had gross residual disease at the time of radiotherapy). Two patients required subsequent total hip arthroplasty because of progressive osteoarthritis, and there were 4 cases of avascular necrosis (only 1 post-treatment). Forty-one patients had good/excellent function. CONCLUSIONS: For patients with extensive or multiple local relapses or when surgery alone would result in a large burden of residual disease or major functional loss, the addition of moderate-dose adjuvant radiotherapy provided excellent local control while maintaining good function with low treatment-related morbidity. Cancer 2012. © 2012 American Cancer Society.

BACKGROUND: Radiation-induced soft tissue sarcomas (RI-STS) are rare, and it is believed that they are associated with a poor prognosis.The authors of this report compared the clinical and functional outcomes of adults who had extremity RI-STS with the outcomes of adults with sporadic STS. METHODS: Forty-four patients who were diagnosed with RI-STS from 1989 to 2009 were identified from 4 prospectively collected databases. Patient demographics, surgical and adjuvant treatment parameters, and oncologic and functional outcomes were evaluated. RESULTS: The median latent period from irradiation of the primary condition to RI-STS diagnosis was 16 years. The median radiotherapy dose used for the index condition was 45 gray. The median age at RI-STS diagnosis was 56 years. The most common primary diagnoses were breast cancer (36.4%) and lymphoma (34.1%). The most common RI-STS histologies were malignant fibrous histiocytoma (36.4%) and angiosarcoma (18.2%). Forty-two patients underwent surgery, 13 patients received adjuvant radiotherapy, and 8 patients received adjuvant chemotherapy. Systemic metastases occurred in 50% of treated patients (n = 21), and 26% (n = 11) developed local recurrence, the risk of which was lower among patients who received reirradiation (P = .043). The 5-year disease-free interval (DFI) and overall survival (OS) rates for patients with RI-STS who presented without metastasis were 36% and 44%, respectively. Patients who had International Union Against Cancer TNM stage III RI-STS had a significantly worse DFI compared with patients who had stage III sporadic STS (multivariate analysis, P = .051). Eighteen patients with RI-STS underwent functional assessment after surgery, and their results were comparable to those of patients with sporadic STS. CONCLUSIONS: Despite aggressive surgical treatment, patients who have RI-STS remain at greater risk of both local and systemic recurrence compared with patients who have sporadic STS, but they can anticipate similar functional outcomes. Reirradiation can be relatively safe and effective if used properly. Cancer 2011. © 2011 American Cancer Society.

BACKGROUND: A study was undertaken to evaluate results of surgery and radiotherapy (RT) for high-risk extracranial chondrosarcomas. METHODS: Between 1986 and 2006, 60 patients underwent surgery and RT for extracranial high-risk chondrosarcoma. Preoperative RT (median, 50 gray [Gy]) and postoperative RT (median, 60 Gy) were used in 40% and 60% patients, respectively. Sites included pelvis/lower extremity (48%), chest wall (22%), spine/paraspinal (17%), and head and neck (13%). Overall, median tumor size was 7 cm (range, 1-22 cm), and tumor grade was I, II, and III in 22%, 64%, and 14% of cases, respectively. RESULTS: Pathologically clear surgical margins (R0) were present in 50%, microscopic positive margins (R1) in 28%, and gross positive margins (R2) in 13%, half of whom had clinically detectable residual disease; surgical margin was unknown in 8%. Median follow-up was 75 months (range, 5-230 months). The crude local control rate was 90%. Patients with R0, R1, and R2 resections had local control of 100%, 94%, and 42%, respectively. Of the 8 cases that had R2 resection, 3 experienced uncontrolled progression, but 5 patients had stable disease with long-term follow-up. The 10-year overall survival, progression-free survival, and cause-specific survival were 86%, 80.5%, and 89.4%, respectively. Younger age and grade III tumors were associated with worse progression-free survival (P = .03 and .0003, respectively). CONCLUSIONS: Although surgery with complete resection is paramount in management of chondrosarcoma, RT is a useful adjuvant treatment and appears to offer excellent and durable local control where wide surgical resection is difficult to accomplish. Cancer 2011. © 2011 American Cancer Society.

BACKGROUND: The objective of this study was to assess patient, tumor, and treatment factors that affected overall survival in a group of patients who underwent surgery for soft tissue sarcoma (STS) and presented with American Joint Commission on Cancer stage IV disease. METHODS: A retrospective review was undertaken of a single institution's database from the years 1986 to 2006 in all patients who met the following inclusion criteria: 1) surgical management of the primary tumor was undertaken, and 2) metastatic disease was present at the time of initial presentation. In total, 112 patients were identified who met the inclusion criteria. RESULTS: The 5-year survival rate for the entire group was 17%. In univariate analysis, the variables that were identified as statistically significant for predicting improved overall survival were resection of metastatic disease (P = .003)

BACKGROUND: As Ewing sarcoma (EWS) can affect children and adults, these patients can be treated at either a pediatric or an adult institution. This study investigated whether differences in therapeutic strategy undertaken in pediatric and adult specialty sarcoma centers correlated with clinical outcome. METHODS: Data from patients with localized EWS treated between 1990 and 2005 at tertiary care pediatric and adult institutions were reviewed. RESULTS: Fifty-three patients (24 adult and 29 pediatric) were treated. Pediatric patients received a median of 16 cycles of chemotherapy comprised of doxorubicin, vincristine, cyclophosphamide, ifosfamide, and etoposide. Adult patients received a median of 10 cycles of treatment, and a significantly lower total cumulative dose of ifosfamide and cyclophosphamide (P < .0001). There was no difference noted with regard to the total dose of doxorubicin, or in the type of local therapy offered (surgery or radiotherapy, vs both). However, local therapy occurred earlier in pediatric patients compared with adults (3.7 months vs 7.4 months; P = .0003). The 3-year event-free survival (EFS) rate in pediatric and adult patients was 70% ± 9% and 43% ± 13% (P = 0.1), respectively. The 3-year overall survival rate was 81% ± 7.7% and 59% ± 12% (P = .02) for pediatric and adult patients, respectively. Factors found to be significantly associated with EFS on univariate analysis included pelvic site, cyclophosphamide dose, and time to local therapy. On multivariate analysis, only pelvic disease (hazard ratio [HR] 4.26; P = .018) and time to local therapy (HR, 1.19; P = .002) were found to be significant. CONCLUSIONS: Adults with localized EWS have an inferior outcome compared with pediatric patients. This difference may be related to lower doses of alkylating agents and the timing of local therapy. Cancer 2010. © 2010 American Cancer Society.

BACKGROUND: Myxoid liposarcoma has been reported to be more radiosensitive compared with other soft tissue sarcomas (STS). The authors report the results of multidisciplinary treatment of extremity myxoid liposarcoma compared with a contemporary cohort of other STS subtypes with an emphasis on the role of radiotherapy (RT) in improving local control. METHODS: Between 1989 and 2004, 691 patients were identified from a prospective STS database who underwent combined management for localized extremity STS and were followed for a minimum of 12 months or until death. All patients underwent surgery together with pre or postoperative RT, depending on their presenting characteristics and resection margins. Demographics and outcomes were compared between patients with myxoid liposarcoma and other STS subtypes (other-STS). RESULTS: Of 691 patients, 88 patients had myxoid liposarcoma and 603 had other STS subtypes (other-STS). Median age was 48 and 60 years for the myxoid liposarcoma and other-STS groups, respectively. Median follow-up was 86 and 61 months, respectively. For myxoid liposarcoma and other-STS groups, preoperative RT was used in 57% versus 61% of patients and postoperative RT in 43% versus 39%, respectively. The 5-year local recurrence-free survival was 97.7% for patients with myxoid liposarcoma compared with 89.6% for patients with other-STS tumors (P = .008). High-grade tumors were present in 7% and 59% of myxoid liposarcoma and other-STS patients, respectively (P = .0003). Two myxoid liposarcoma patients with local recurrence had positive resection margins, whereas only 33% of patients in the other-STS group who developed a local recurrence had positive resection margins. No patients with myxoid liposarcoma required amputation as primary management, whereas 8 (1.3%) required amputation as primary management in the other-STS group. Systemic disease control was superior in myxoid liposarcoma compared with other-STS patients, with 5-year overall and metastasis-free survival rates of 93.9% versus 76.4% (P = .0008) and 89.1% versus 66.0% (P = .0001) respectively. Of 12 myxoid liposarcoma patients with distant metastases, 7 appeared in nonpulmonary sites. In comparison, 205 (34%) patients with other-STS tumors developed systemic disease but 78% had pulmonary metastases. CONCLUSIONS: Multidisciplinary management of extremity STS achieves high rates of local control. Myxoid liposarcoma is associated with higher rates of local control compared with other STS subtypes, after combined surgery and radiation, suggesting a particular radiosensitivity that can be exploited to improve oncologic outcome in appropriate cases. Cancer 2009. © 2009 American Cancer Society.

Background and Objectives Patients' expectations regarding their surgical recovery can significantly influence health outcomes. This study examines the relationship between pre-treatment outcome expectations and post-operative function and health-related quality of life (HRQoL) in patients with extremity soft tissue sarcoma (ESTS). Additionally, we evaluate predictors of patients' outcome expectations. Methods Data were prospectively collected on 157 ESTS patients (62% male, mean age 56 years) treated with limb-preservation surgery between January 2001 and February 2005. Associations between patients' outcome expectations and 1-year function and HRQoL outcomes were evaluated using multivariable regression analyses. Factors predicting patient expectations were investigated using multinomial logistic regression. Results Patients expecting a difficult recovery and patients with uncertain expectations had worse functional outcomes than patients anticipating an easy recovery. Education, dispositional optimism, tumor location, AJCC stage, and baseline function/HRQoL were significant predictors of patient expectations. For example, patients with primary school education more frequently reported uncertain expectations regarding length of recovery (OR = 20; 95% CI, 3.7–108.6) and complications (OR = 12; 95% CI, 2.7–58.0) than patients with post-secondary education. Conclusions Patient expectations significantly influence functional outcome in ESTS. Patients at risk for uncertain expectations may benefit from additional, individualized education so as to optimize their treatment outcomes. J. Surg. Oncol. 2009;100:375–381. © 2009 Wiley-Liss, Inc.

Background and Objectives Prior studies have shown that sarcoma growth can be stimulated by insulin-like growth factor-I (IGF-I). To extend this line of research, we carried out in vivo growth studies of primary human sarcoma in immunosuppressed control and IGF-I–deficient mice. Methods Human sarcoma specimens (one osteosarcoma and seven soft tissue sarcomas) were harvested in the operating room and implanted in immunosuppressed mice. Second-generation sarcomas were transplanted to control (GH replete lit/+ mice) and to experimental (GH/IGF-I–deficient lit/lit) animals. When tumors reached 1,000 mm3 in one group, average tumor size was compared in the two groups. IGF-I receptor expression was measured by RT-PCR and IGF-I receptor binding sites were assayed by radiolabeled IGF-I. Results Five of eight sarcomas demonstrated reduced growth in the GH/IGF-I–deficient lit/lit animals. In four of the five sarcomas that demonstrated growth inhibition, IGF-R was elevated relative to placenta or a positive control cell line (MCF-7, which is known to be responsive to IGF-I in vitro and in vivo). In three of the five sarcomas that demonstrated growth suppression, IGF-R was elevated twofold after implantation in the experimental IGF-I–deficient animals. Conclusions The GH–IGF axis may be an important stimulator of tumor growth in sarcomas. These experiments suggest that IGF suppression may inhibit sarcoma growth in vivo. J. Surg. Oncol. 2002;81:75–79. © 2002 Wiley-Liss, Inc.