Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment.

PURPOSE: Synovial sarcoma (SS) is a common soft tissue sarcoma (STS) with a propensity for young adults and notable sensitivity to chemotherapy (CT). This study provides a current clinicopathologic, staging, and prognostic assessment for SS. The problems with the current American Joint Committee for Cancer (AJCC) Staging System in relation to SS are discussed. METHODS: Review of a prospective database supplemented by retrospective data. RESULTS: One hundred fifty patients were assessed; median age was 30 years and median follow-up was 52 months. Overall actuarial 5-year survival rate was 57%. Size trend, but not a cutoff of less than 5 cm versus > or = 5 cm, was a prognostic indicator (P <.001). The current AJCC/International Union Against Cancer Staging System differentiated prognosis less well than the recently proposed Royal Marsden Hospital Staging System. Age greater than 20 years at diagnosis implied worse prognosis. A local recurrence event was associated with a worse survival (P <.001). Therapeutic CT was administered to 55 patients. Eleven of 19 patients had an objective response to a combination of ifosfamide and doxorubicin. Four cases had complete response after CT. Twenty-one patients had pulmonary metastasectomy, with an actuarial 5-year survival rate of 23%. CONCLUSION: SS tends to affect young people. In this subtype of STS, size trend is the most significant influence on stage and hence survival; however, smaller SSs have an unexpectedly poor prognosis. Adequate local control may affect survival. SS is often chemosensitive, and given its poor prognosis, multicenter trials of adjuvant therapy are warranted.     

Malignant fibrous histiocytoma and other fibrohistiocytic tumors in pediatric patients: the St. Jude Children's Research Hospital experience

BACKGROUND: Malignant fibrous histiocytoma (MFH) is a controversial entity. In the current study, the authors reviewed their institutional experience with these tumors to characterize their clinical features in pediatric patients and assess the impact of surgical resection on outcome. METHODS: The records of the 28 patients who were diagnosed with MFH or MFH variants of soft tissue between January 1971 and December 2000 were reviewed and the tumors were reclassified according to the World Health Organization guidelines. RESULTS: Seventeen patients had MFH; 10 patients had angiomatoid fibrous histiocytoma (FH), and 1 patient had a plexiform fibrohistiocytic tumor. The median age of patients at the time of diagnosis was 7.3 years. The most common primary tumor site was the extremity (n = 14). Metastatic disease (to the lung) was present in only three patients, each of whom had MFH. Of the 17 MFH tumors, 13 were high grade, 8 were invasive, and 6 measured > 5 cm. All angiomatoid FH tumors and the plexiform fibrohistiocytic tumor were noninvasive, and 10 measured < or = 5 cm. Surgical treatment was comprised of wide local excision with clear margins (n = 18), amputation (n = 3), excision with positive or indeterminate surgical margins (n = 4), partial resection (n = 2), or biopsy only (n = 1). Primary reexcision was performed for 21 patients. The 5-year survival and event-free survival (EFS) estimates for patients with MFH were 76.5% +/- 11.2% and 70.6% +/- 12.1%, respectively; the 5-year survival and EFS estimates were 100% +/- 0% for patients with angiomatoid FH or plexiform fibrohistiocytic tumor. Compared with partial resection or excision, wide local excision or amputation was found to have a positive impact on the probability of EFS in patients with localized disease (P = 0.008). All four patients with metastatic or unresectable MFH had died by the time of last follow-up. CONCLUSIONS: MFH should be distinguished from angiomatoid FH and plexiform fibrohistiocytic tumors, both of which are less aggressive. Wide local excision is the treatment of choice, regardless of the histology or grade of the tumor. Patients with metastatic or unresectable MFH appear to have a poor outcome and would benefit from more effective therapies.     

Histopathology peer review of high-grade soft tissue sarcoma: the Scandinavian Sarcoma Group experience

From 1981 to 1986, a total of 240 patients with a primary soft tissue sarcoma with malignancy grade III or IV were entered into an adjuvant chemotherapy multicenter trial conducted by the Scandinavian Sarcoma Group (SSG). Histopathologic peer review of all the specimens was performed by an expert pathology committee. The most common soft tissue sarcoma after review was malignant fibrous histiocytoma (MFH) (40%), followed by synovial sarcoma (15%), leiomyosarcoma (9%), liposarcoma (8%), and malignant Schwannoma (6%). In 25% of the cases the histologic type of sarcoma was reclassified, and in 40% of the cases the malignancy grade was changed. By survival analyses, the reclassification of malignancy grade seemed to be valid. Also, grading highly malignant soft tissue sarcoma in two grades (III and IV) increased the prognostic information. Of 164 tumors from the centers with the most reported cases (five centers with 25 to 51 tumors each), eight tumors were found to be ineligible for the adjuvant study (5%); of 76 tumors from 13 centers with few tumors (one to 16 tumors per center), 12 tumors were ineligible (16%). We conclude that histologic peer review is important in studies of soft tissue sarcoma.     

A single-team experience of limb sparing approach in adults with high-grade malignant fibrous histiocytoma

Malignant fibrous histiocytoma (MFH) is the most common subtype of soft-tissue sarcoma (STS). When located in a limb, MFH, is currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT). During 8 years, 42 adult patients with high-grade limb MFH were approached by LSS and RT. Our results reflect a single-team experience and point to several important conclusions. High grade MFH, treated by conservative approach, lead to a 10-year relapse-free survival of 62% and a 10-year overall survival rate of 80%. Recurrences of MFH tend to occur during the first 2 years. Relapse-free survival was affected mainly by location in the lower limb vs. the upper limb, irrespective of the tumor size. Patients who had their diagnostic biopsies in another medical center had a greater tendency to local and systemic relapse. It seems that the most important clues for disease-free survival are the team experience and cooperation. All other factors are tumor-biology dependent, and thus far are beyond our control.     

A comparison of staging systems for localized extremity soft tissue sarcoma

BACKGROUND: Staging systems for soft tissue sarcoma (STS) are important to identify patients with similar systemic risk who might benefit from specific treatments. This study compared four commonly used staging systems for predicting systemic outcomes of patients with localized extremity STS, as proposed by the fourth and fifth editions of the American Joint Committee on Cancer/International Union Against Cancer (AJCC/UICC) staging system, the Memorial Sloan-Kettering Cancer Center (MSK) system, and the Surgical Staging System (SSS) of the Musculoskeletal Tumor Society. METHODS: Three hundred consecutive adult patients with newly diagnosed nonmetastatic STS of the lower extremity were treated at Memorial Sloan-Kettering Cancer Center between 1982 and 1989. Metastasis free survival was the end point of the study. The prognostic value of the four staging systems and their components were examined in univariate and multivariate analyses. The Akaike information criterion (AIC) was used to identify the system that best predicted the risk of systemic recurrence. RESULTS: Compartment status, depth, grade, and size were all independent predictors of outcome within their respective staging systems. However, when compared with one another, only depth, grade, and size retained their prognostic significance. Of the four models, the AIC predicted that the MSK was the best predictor of systemic relapse, followed by the fifth edition of the AJCC/UICC staging system. CONCLUSIONS: Staging systems such as the MSK system or the fifth edition of the AJCC/UICC system, which include tumor depth, grade, and size as prognostic factors, are the most predictive of systemic relapse in patients presenting with localized extremity STS. Both of these systems identify the same group of patients at the highest risk who would be the most suitable for adjuvant chemotherapy trials.     

Malignant fibrous histiocytoma: an institutional review

Background: A thorough understanding of malignant fibrous histiocytoma (MFH), the most common subtype of soft tissue sarcoma, will lead to improved histologic-specific protocols. Methods: 126 patients with histologically confirmed MFH were analyzed. The median follow-up was 42 months (range 1-233 months). Results: Overall survival was 58% at 5 years and 38% at 10 years. Grade significantly influenced prognosis, with 10-year survival of 90%, 60%, and 20% for low, intermediate, and high grade tumors, respectively (p = 0.0007). Distant metastases at initial presentation (p = 0.0002) and size of the primary tumor (p = 0.0007) influenced outcome. Neither anatomic site nor depth of the primary tumor were significant prognostic factors. Positive microscopic margins were associated with a decreased disease-free survival (p = 0.006). Conclusions: Tumor grade, size, and distant metastases at initial presentation remain the most important prognostic factors for MFH. Resection with negative microscopic margins decreased the incidence of local recurrence.     

Brachytherapy for pediatric soft-tissue sarcoma

PURPOSE: To review the use of brachytherapy (BRT) to treat soft-tissue sarcoma (STS) in pediatric patients at St. Jude Children's Research Hospital. METHODS AND MATERIALS: Thirty-one patients, median age 11 years (range 1-21 years) with Pediatric Oncology Group (POG) Grade 2-3 soft-tissue sarcoma (excluding rhabdomyosarcoma and Ewing's sarcoma) were treated with BRT initially (n = 27) or at the time of recurrence (n = 4) using I-125 or Ir-192 in a temporary (n = 29) or permanent implant (n = 2). Twelve patients were treated with BRT alone and the remaining 19 were treated with a combination of BRT and external beam irradiation (EBRT). The majority of patients had involved margins of resection (n = 20) and tumors less than 5 cm (n = 17). RESULTS: Twenty-seven patients were treated with BRT at the time of presentation. Among the 10 patients treated with BRT alone, one patient developed metastases (4 months) and died of metastatic disease (12 months after presentation); there were no local or regional failures among the remaining 9 patients. Among the 17 patients treated with a combination of BRT and EBRT, there was one local (17 months), two regional (both at 8 months), and 3 distant failures (12, 15, 66 months). The median survival for the surviving 25 patients was 34 months. Wound dehiscence, fibrosis/telangectasia, pigment changes, and cellulitis were the most common side effects. CONCLUSIONS: BRT is an excellent treatment option for pediatric patients with STS. Disease control may be achieved with a high rate of success when BRT is used alone or in combination with EBRT. BRT should be considered for patients with STS who require radiation therapy with the objective of reducing the dose to normal tissues and shortening the overall treatment time. Limb preservation, functional outcome, and toxicity assessment require careful assessment in a prospective study.     

796例软组织肉瘤分析

目的:了解软组织肉瘤各病种发病情况及相对频率,并调查软组织肉瘤发病趋势。方法:对本院1993年1月至2003年12月所有收治的原发软组织肉瘤796例发病情况进行回顾性分析,从而初步得到各病种发病情况和相对频率,并推测其发病趋势。结果:依据病理分型发病率最高者为恶性纤维组织细胞瘤、滑膜肉瘤、脂肪肉瘤、横纹肌肉瘤等,分别占所收治例数的31.5%,16.8%,16.8%和16.2%。软组织肉瘤可见于各个年龄阶段,可发生于全身任何部位,但各亚型均有各自的特点。结论:软组织肉瘤发病率较低。恶性纤维组织细胞瘤在软组织肉瘤中发病率最高。软组织肉瘤的发病率呈逐年上升趋势。     

The clinical and functional outcome for patients with radiation-induced soft tissue sarcoma

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.     

Soft tissue sarcoma diagnosed subsequent to lymphoma is associated with prior radiotherapy and decreased survival

BACKGROUND:

Cancer survivors are at increased risk for second malignancies, and vigilance is thus required. The authors sought to determine whether there was an association among lymphoma, sarcoma, and the associated treatments for these diseases.

METHODS:

From the authors' prospective soft tissue sarcoma (STS) database of 8240 patients, they identified 112 patients with STS and lymphoma treated from 1982 to 2009 who had complete follow‐up data. They examined the importance of the initial diagnosis in patients with both STS and lymphoma, in addition to determining the role of radiation therapy, a known inducer of sarcoma.

RESULTS:

Review of their sarcoma, gastric, urology, breast, and gynecology databases revealed that lymphoma (95%) or leukemia (5%) occurred in 1.6% of STS patients in comparison to 0.5% of patients in the other databases (P < .01; odds ratio, 3.1; 95% confidence interval, 2.6‐3.8). Patients diagnosed with STS only were more likely to die of disease at 10 years compared with those with STS and lymphoma (P = .006), but this difference was not significant when patients presenting with recurrence or metastases were excluded. Among patients with lymphoma and STS, lymphoma was the first diagnosis in 71% of patients. Median survival after STS diagnosis was shorter when lymphoma was the initial diagnosis (67 vs 170 months, P = .002), and these patients were more likely to have radiation‐associated STS (44% vs 3%, P < .001).

CONCLUSIONS:

There was a 3‐fold higher incidence of lymphoma in STS patients compared with other solid tumors. The poor prognosis of those diagnosed with both STS and lymphoma was most likely a consequence of prior irradiation. Cancer 2011. © 2011 American Cancer Society.     

Configuration of Soft-tissue Sarcoma on MRI Correlates with Grade of Malignancy

BackgroundThe aim of the study was to assess whether the configuration of primary soft-tissue sarcoma (STS) on MRI correlates with the grade of malignancy.Patients and methods71 patients with histologically proven STS were included. Primary STS were examined for configuration, borders, and volume on MRI. The tumors were divided into high-grade (G3), intermediate-grade (G2) and low-grade (G1) STS according to the grading system of the French Federation of Cancer Centers Sarcoma Group (FNCLCC).Results30 high-grade, 22 intermediate-grade and 19 low-grade primary STS lesions were identified. High- and intermediate-grade (G3/2) STS significantly most often appeared as polycyclic/multilobulated tumors (p < 0.001 and p = 0.002, respectively). Low-grade (G1) STS mainly showed an ovoid/nodular or streaky configuration (p = 0.008), and well-defined borders. The appearance of high-, intermediate- and low-grade STS with an ovoid/nodular configuration were mainly the same on MRI. All streaky G3/2 sarcoma and 17 of 20 patients with polycyclic/multilobulated G3 sarcoma showed infiltrative borders. High-grade streaky and polycyclic/multilobulated STS are larger in volume, compared to intermediate- and low-grade STS.ConclusionsConfiguration of STS on MRI can indicate the grade of malignancy. Higher-grade (G2/3) STS most often show a polycyclic/multilobulated configuration, while low-grade STS are mainly ovoid/nodular or streaky. Infiltrative behavior might suggest higher-grade STS in streaky and polycyclic/multilobulated STS.Key words: soft-tissue sarcoma, MRI, configuration, FNCLCC, malignancy, prediction     

Incidence and survival of soft tissue sarcoma in England between 2013 and 2017, an analysis from the National Cancer Registration and Analysis Service

There is a paucity of population-based data detailing the incidence and survival of patients with soft tissue sarcoma (STS), in part due to the heterogeneity of disease and changes to classification. Here, the incidence and survival of all STS subtypes registered in England between 2013 and 2017 were analysed using cancer registry data held by the National Cancer Registration and Analysis Service. Age-standardised incidence rates were calculated per 1 000 000 using the 2013 European Standard Population. Net survival was computed using Brenner's alternative method, with the Ederer II estimator. Age-specific overall survival was assessed using Kaplan-Meier. The influence of age, sex, socioeconomic deprivation and diagnostic routes on survival was assessed using Cox proportional hazards modelling. In total, 19 717 patients were diagnosed with STS, an average of 3943 patients per year and representing approximately 0.8% of malignancies. The most common histological diagnoses were Gastrointestinal Stromal Tumours (GIST), leiomyosarcoma and undifferentiated sarcoma, accounting for 20.2%, 13.3% and 12.7% of all sarcomas, respectively. Five-year net survival for all malignant STS was 65.0%; and was lowest for patients with vascular tumours at 39%. Patients from most deprived cohorts had 23% greater chance of dying within 5 years than patients in least deprived areas. This population-based study has allowed us for the first time to define the incidence and survival rates of prevalent STS subtypes in England such as GIST, liposarcoma and leiomyosarcoma, as well as rare entities and groups with inferior outcome. This data is invaluable for service provision, benchmarking and addressing inequality.     

Pathologic staging of renal cell carcinoma: significance of tumor classification with the 1997 TNM staging system

BACKGROUND: The TNM staging system for renal cell carcinoma was revised by the American Joint Committee on Cancer (AJCC) and the International Union Against Cancer (UICC) in 1997. The 1997 TNM staging system for renal cell carcinoma reclassifies tumors using criteria for size and for extent of renal vein/vena cava involvement that are different from the criteria used in the 1987 staging system. The current study investigated the prognostic significance of tumor classification and other factors using the new staging system. METHODS: Records from 1547 renal cell carcinoma patients (1039 males and 508 females; mean age, 63.4 years; mean follow-up, 7.1 years) who underwent surgical resection between 1970 and 1998 were analyzed retrospectively. Tumors were staged using the 1987 and 1997 TNM criteria, and Kaplan-Meier estimates of survival and disease recurrence were compared for both staging systems. The Peto-Peto log rank test and the generalized Wilcoxon test were used to assess univariate significance of prognostic factors on survival. Cox proportional hazards regression analysis was then completed to assess the significance of the revised staging system. RESULTS: Tumor classification using the 1987 TNM staging system (P = 0.0001) and the 1997 TNM staging system (P = 0.0001) was a significant predictor of cause specific survival. Using 1997 TNM staging criteria, 641 patients were reclassified from the T2 classification to the T1 classification, 114 patients were reclassified from the T3c classification to the T3b classification, 11 patients were reclassified from the T4b classification to the T3c classification, and 3 patients were reclassified from the T4b classification to the T3b classification. Patients with reclassified tumors had outcomes similar to patients with tumors that remained in the same tumor classification. Patient stratification was improved using the new staging system. Prognostic discrimination for cause specific survival at 10 years was noted for the 1987 and 1997 TNM classifications (T1, 97% vs. 91%; T2, 84% vs. 70%; T3a, 53% vs. 53%; T3b, 48% vs. 42%; and T3c, 29% vs. 43%). CONCLUSIONS: The revised classification of renal cell carcinoma was a significant predictor of cause specific survival for the cohort of patients described in this report. Using the new system, the stratification of patients was improved. Patients who had their tumors reclassified as a result of the new staging system had outcomes similar to those of patients who had tumors that remained in the same classification. Based on an analysis of this cohort, tumor classification is valid, and the T1 subclassification is warranted. However, additional revision may be required to optimize staging.     

Retroperitoneal liposarcomas: follow-up analysis of dedifferentiation after clinicopathologic reexamination of 86 liposarcomas and malignant fibrous histiocytomas

BACKGROUND: Dedifferentiated liposarcoma (DDL) juxtapose components of well-differentiated liposarcoma (WDL) and nonlipogenic sarcoma. Malignant fibrous histiocytoma (MFH) is no longer considered a homogeneous entity, but rather as the common morphologic appearance of various subtypes of sarcomas. The objectives of the current retrospective study were: 1( to analyze the relation between DDLs and tumors previously diagnosed as MFHs; 2) to trace the evolution of liposarcomas, and 3) to assess the consequences of dedifferentiation. METHODS: Between 1974 and 2001, 86 patients with retroperitoneal liposarcoma (61 patients) or MFH (25 patients) underwent surgery at Institut Bergonie in Bordeaux, France. Histologic review was performed and tumors reclassified as WDL or DDL were retained for further clinicohistologic study. Subsequently, initial presentation and all recurrences were analyzed. RESULTS: The 61 liposarcomas consisted of 21 WDLs and 35 DDLs; 17 MFHs were reclassified as DDL. In all, approximately half of the retroperitoneal liposarcomas and MFHs were found to be DDLs. The DDLs presented with a smaller size (20 cm vs.30 cm; P = .05) but a lower rate of complete resection (72% vs.90%; P = .015) and remission (72% vs. 100%; P = .0015). Dedifferentiated recurrence was evidenced in 7 WDLs. Ten DDLs presented with metastatic evolution. DDLs demonstrated a tendency toward lower rates of 5-year overall survival (55% vs. 82%; P = .075). CONCLUSIONS: Most occurrences of retroperitoneal liposarcomas and MFHs are in fact DDLs and dedifferentiated recurrence of WDLs is frequent. Retroperitoneal DDLs present a lower rate of complete remission than WDLs and a risk of metastatic recurrence. Therefore, extensive histologic analysis of WDLs is required to identify an undifferentiated component and avoid misdiagnosis of DDL.     

Cohort analysis of patients with localized, high-risk, extremity soft tissue sarcoma treated at two cancer centers: chemotherapy-associated outcomes.

PURPOSE: Patients with American Joint Committee on Cancer stage III soft tissue sarcoma (STS) have high risks of distant recurrence and death. The role of chemotherapy for these patients remains controversial despite several randomized trials and a meta-analysis. METHODS: We reviewed the treatments and outcomes of 674 consecutive adult patients presenting with primary stage III extremity STS between 1984 and 1999. Pre- or postoperative doxorubicin-based chemotherapy was used in a nonrandomized fashion in approximately half of this high-risk population. The objective of this review was to evaluate the impact of chemotherapy while accounting for known prognostic variables. RESULTS: Among 674 patients, 338 (50%) were treated with local therapy only, and 336 (50%) were treated with local therapy plus chemotherapy. The median follow-up for survivors was 6.1 years. Five-year local and distant recurrence-free interval probabilities were 83% and 56%, respectively, for the two groups combined. The 5-year disease-specific survival (DSS) rate was 61%. Cox regression analyses showed a time-varying effect associated with chemotherapy. During the first year, the hazard ratio associated with DSS for patients treated with chemotherapy versus no chemotherapy was 0.37 (95% CI, 0.20 to 0.69; P = .002). Thereafter, this hazard ratio was 1.36 (95% CI, 1.02 to 1.81; P = .04). CONCLUSION: It seems that the clinical benefits associated with doxorubicin-based chemotherapy in patients with high-risk extremity STS are not sustained beyond 1 year. These results suggest that caution should be used in the interpretation of randomized clinical trials of adjuvant chemotherapy that seem to demonstrate clinical benefits with relatively short-term follow-up.     

Pediatric sarcoma in Central America

BACKGROUND:

Children with cancer in middle‐income countries have inferior outcomes compared with similar children in high‐income countries. The magnitude and drivers of this survival gap are not well understood. In the current report, the authors sought to describe patterns of clinical presentation, magnitude of treatment abandonment, and survival in children with sarcoma in Central America.

METHODS:

A retrospective review was conducted of hospital‐based registries from national pediatric oncology referral centers. Patients with newly diagnosed osteosarcoma, Ewing sarcoma, rhabdomyosarcoma (RMS), and soft tissue sarcoma (STS) between January 1, 2000 and December 31, 2009 were included. Survival analyses were performed first using standard definitions of overall survival (OS) and event‐free survival (EFS) and then with abandonment included as an event (abandonment‐sensitive OS and abandonment‐sensitive EFS).

RESULTS:

In total, 785 new cases of pediatric sarcoma were reported (264 diagnoses of osteosarcoma, 175 diagnoses of Ewing sarcoma, 240 diagnoses of RMS, and 106 diagnoses of STS). The rate of metastatic disease at presentation was high (osteosarcoma, 38%; Ewing sarcoma, 39%; RMS, 29%; and STS, 21%). The treatment abandonment rate also was high, particularly among patients with extremity bone sarcomas (osteosarcoma, 30%; Ewing sarcoma, 15%; RMS, 25%; and STS, 15%). Of 559 patients who experienced a first event, 59% had either recurrent or progressive disease. The 4‐year OS rate (±standard error) was 40% ± 3%, and the EFS rate was 30% ± 2%; however, these rates decreased further to 31% ± 2% and 24% ± 2%, respectively, when abandonment was taken into account.

CONCLUSIONS:

The current results indicated that high rates of metastases and treatment abandonment and difficulty with upfront treatment effectiveness are important contributors to the poor survival of children with pediatric sarcomas in Central America. Initiatives for early diagnosis, psychosocial support, quality improvement, and multidisciplinary care are warranted to improve outcomes. Cancer 2013. © 2012 American Cancer Society.     

Immunohistochemical Loss of the DNA Mismatch Repair Proteins MSH2 and MSH6 in Malignant Fibrous Histiocytomas

Purpose: Soft tissue sarcomas (STS) account for less than 1% of all malignancies and constitute a heterogeneous tumor entity in which malignant fibrous histiocytomas (MFH) represent one-third and are characterized by a lack of type-specific differentiation. A defective mismatch repair (MMR) system cause the familial cancer syndrome hereditary non-polyposis colorectal cancer (HNPCC), and since occasional MFH have been described in HNPCC patients we assessed the contribution of defective MMR to the development of MFH.Methods: MMR status was characterized in a series of 209 histopathologically reviewed MFH. Tissue microarray sections from the tumors were immunohistochemically stained for the MMR proteins MLH1, MSH2 and MSH6, and cases with aberrant staining were further characterized for microsatellite instability.Results and Discussion: Two of the 209 STS-a storiform-pleomorphic MFH and a myxofibrosarcoma-showed concomitant loss of MSH2 and MSH6, but retained staining for MLH1 on both cases. The myxoid tumor also had a microsatellite unstable phenotype. These findings, together with previous observations of defective MMR in pleomorphic STS, indicate that these tumors may be part of the HNPCC-associated tumor spectrum and demonstrate that MMR defects occur in a small subset of STS.     

Appraising the current role of chemotherapy for the treatment of sarcoma

Sarcomas are a heterogeneous group of relatively rare mesenchymal neoplasms. They can be grouped into two general categories: soft tissue sarcoma (STS) and primary bone sarcoma, which are treated differently. Because sarcomas are relatively rare and complex with a wide variety of different histopathologic subtypes, evaluation by multidisciplinary teams who have expertise in the field is recommended. Treatment guidelines for the use of chemotherapy in patients with STS and bone sarcoma have been published by the National Comprehensive Cancer Network. The role of adjuvant chemotherapy in resected STS remains controversial. Although chemotherapy improves disease-free survival, the long-term overall survival benefit remains unproven. Chemotherapy is typically used as palliative treatment for most subtypes of metastatic STS. In contrast, chemotherapy has a proven role in the treatment of primary bone tumors and Ewing sarcoma, but it has not demonstrated efficacy in the treatment of chondrosarcoma. The standard chemotherapy regimens used in sarcoma are associated with significant toxicity, including long-term complications. Less intense and less toxic regimens are the focus of ongoing clinical research. Newer cytotoxic agents with an improved safety profile, such as trabectedin and palifosfamide, are currently in development. Future research needs to focus on identification of subpopulations of patients that are most likely to benefit from chemotherapy.     

Incidence and time trends of soft tissue sarcomas in German children 1985-2004 - a report from the population-based German Childhood Cancer Registry

The incidence of soft tissue sarcoma (STS) in Europe is increasing, but it is unclear whether this increase can also be seen in Germany. We analysed the heterogeneous group of STS recorded to the German Childhood Cancer Registry (GCCR) between 1985 and 2004 with respect to incidence data. Age-specific, age-standardised and cumulative incidence rates were calculated. Additionally, the average annual percent change (AAPC), derived from a Poisson regression model, was estimated, using time in years as the explanatory, continuous variable. Two thousand sixty-one children were diagnosed at a median age of 72 months. Most common are rhabdomyosarcomas (RMS) (n=1202) and fibrosarcomas (n=174). The age-standardised incidence rate (ASR) is 0.9 per 100,000/year for all STS, 0.70 for rhabdomyosarcoma-like (n=1588) and 0.18 for non-rhabdomyosarcoma-like (n=411) STS. AAPC is +0.4% (95%-confidence interval [-0.4;+1.2%]) for STS. In Germany, the increase in incidence rate is less evident than in other European countries.     

Treatment results and prognostic factors in patients with malignant fibrous histiocytoma

The objective of this study is to investigate the treatment results, prognostic factors, the role of postoperative radiotherapy, and the usefulness of American Joint Committee on Cancer (AJCC) Staging System (2002) for soft tissue sarcomas in malignant fibrous histiocytoma (MFH). Between November 1987 and December 2000, 76 patients with localized MFH underwent surgery as the primary treatment modality with or without radiotherapy in our institution and were reviewed retrospectively. Patients with regional nodal disease, distant metastases or retroperitoneal disease were excluded from our study. All patients had at least 27 months of follow-up. The 5-year overall survival rate, local control rate, and distant metastasis-free rate were 74%, 62%, and 87%, respectively. In multivariate analysis, AJCC 2002 staging system was the only independent prognostic factor for overall survival rates (p=0.0017). Postoperative radiotherapy was the only significant factor for local control rates (p=0.0024). In conclusion, staging system is a prognostic predictor for overall survival rates and postoperative radiotherapy can improve local control. However, the optimal adjuvant treatment strategy for MFH should still be further explored.