Staging Of Soft Tissue Sarcomas Research Articles

Experimental models of tumor growth in soft tissue sarcomas

Soft tissue sarcomas are rare tumors (about 1 % of all malignant neoplasms) and include more than 70 histological subtypes, the pathogenetic features of which remain unclear. This is largely due to both quantity and volume of clinical material and high heterogeneity of the disease. Given the rarity and heterogeneity of each individual subtype of soft tissue sarcoma, there is an urgent need to develop universal model systems to understand the molecular changes that determine tumor biology. Such systems include CDX models (cell line-derived xenograft), created from cell lines, PDX (patient-derived xenograft), obtained from primary tumor/metastasis cells, both a whole fragment of surgical material and from a cell suspension; humanized animals containing various human immune cells, and GEM (genetically engineered mouse) models, which are created through transfection of genetic changes characteristic of different subtypes of soft tissue sarcomas. To create these systems, not only widely available mouse models are used, but also other animals, such as fish (Danio rerio) , rats, pigs, and dogs. Another important goal of using animal models is to screen the effectiveness of modern drugs. To date, treatment of various subtypes of soft tissue sarcomas is based on standard protocols of chemotherapy (doxorubicin, epirubicin, dacarbazine, ifosfamide) and surgical resection. In the case of inoperable forms or late stages of soft tissue sarcomas, animal models are a potential tool in predicting the effectiveness of therapy and personalized selection of treatment regimens. In this regard, studies of the mechanisms of targeted action on specific molecules and the use of humanized animals for the development of new approaches to immunotherapy are of particular relevance. The current review discusses animal model systems of the three most common types of soft tissue sarcomas: liposarcomas, undifferentiated pleomorphic and synovial sarcomas, as well as the use of these models to find new therapeutic solutions. Conclusion. Currently, PDX and GEM models are widely used to identify molecules and signaling pathways involved in the development of sarcomas, identify tumor-initiating cells, and assess the chemoresistance of known drugs and new drugs at the level of the entire tumor ecosystem. However, the key problems of animal models of soft tissue sarcomas remain changes in their composition and phenotype compared to the original tumor, poor survival of surgical material, and lack of cellular immunity in immunocompetent models, high cost, and the length of time it takes to create and maintain the model. A solution to one of the problems may be the use of humanized animals with PDX, which implies the presence in the model of immune, stromal and tumor components that are as close as possible to the human body.

A closer look at the staging of soft tissue sarcomas in an era of personalized medicine

We read with enthusiasm the editorials by Rick Hass and William Tseng in response to our recently published manuscript, “ A novel, simplified, externally validated staging system for truncal/extremity soft tissue sarcomas: An analysis of the US Sarcoma Collaborative database ” (1). These editorials, along with a considerable array of recent publications, review articles, and other editorials over the past few years, represent a re-invigorated interest for this rare, and previously somewhat ignored, malignancy amongst the oncology community.

Undifferentiated pleomorphic sarcoma: Factors predictive of adverse outcomes

Undifferentiated pleomorphic sarcoma (UPS) encompasses rare neoplasms that can arise either in the dermis or in the subfascial soft tissue. The behavior of UPS ranges from indolent to aggressive, but data predicting outcomes are limited. Identify predictors of poor outcomes by analyzing a large collection of UPS cases. We evaluated all available cases of UPS (including those termed atypical fibroxanthoma, malignant fibrous histiocytoma, pleomorphic dermal sarcoma, and subfascial UPS) across 3 tertiary care centers. Among the 319 patients, 45 experienced recurrence, 33 experienced metastasis, and 96 died of any cause. Risk factors for recurrence were clinical tumor size larger than 5cm and invasion beyond subcutaneous fat. Risk factors for distant metastases were tumor site, tumor size larger than 2cm, invasion beyond subcutaneous fat, and lymphovascular invasion. Risk factors for overall mortality were age, immunosuppression, tumor size larger than 2cm, and lymphovascular invasion. History of skin cancer was associated with a lower risk of recurrence and metastasis. This was a retrospective study. Using the unbiased approach of pooling all UPS cases regardless of terminology, we identified clinical and histologic factors predicting poor outcomes. We propose subcategorization of UPS (into superficial versus deep UPS), which is consistent with the American Joint Committee on Cancer staging of soft-tissue sarcoma.

Performance Analysis of the American Joint Committee on Cancer 8th Edition Staging System for Retroperitoneal Sarcoma and Development of a New Staging Algorithm for Sarcoma-Specific Survival.

The American Joint Committee on Cancer (AJCC) recently published the 8th edition of the AJCC Cancer Staging Manual. Major changes were made to the staging algorithm for retroperitoneal sarcoma; however, whether these changes improve staging system performance is questionable. This retrospective cohort analysis of 3703 adult patients with retroperitoneal sarcoma in the Surveillance, Epidemiology, and End Results (SEER) database compares a novel staging system incorporating histologic subtype of sarcoma with current and prior AJCC soft tissue sarcoma staging systems using multiple statistical techniques. The effect of tumor size on sarcoma-specific survival was also assessed by flexible, non-linear Cox proportional hazard regression using restricted cubic splines and fractional polynomials. The relationship between the covariate-adjusted log hazard for disease-specific survival and tumor size is non-linear. Although the new AJCC T classification approximates this hazard fairly well, the overall prognostic impact of tumor size is limited after accounting for other predictive factors. Predictive accuracy and concordance indices of the AJCC 8th edition staging system for retroperitoneal sarcoma are significantly lower than the prior 7th edition. A proposed staging system incorporating histologic grade, tumor size, and histologic subtype is superior to both the AJCC 7th and 8th editions in predicting sarcoma-specific survival. AJCC committees should not revise tumor staging algorithms unless the changes actually improve the staging system. A proposed staging scheme incorporating data regarding histologic subtype of sarcoma performs significantly better than both the 7th and 8th AJCC staging systems.

Sarcoma tumor size (T) staging: Are radiology or pathology measurements more appropriate?

e22522 Background: In the AJCC version 7 TNMG staging of soft tissue sarcomas (STS), the longest dimension (1D) of the primary tumor at pathology analysis is the gold standard for tumor size (T) staging. However, measurements may differ between scans and actual tissue measurement, due to tissue elasticity, deformation, and/or formalin fixation. Thus, tumor size may change compared to preoperative imaging. Should STS T stage be defined by imaging or by direct tumor measurement? We examined the variability of the measurements between radiology and pathology data, examining 1D, cross sectional area (2D), and tumor volume (3D) to assign a T stage. Methods: We reviewed all patients (pts) with extremity STS who had surgery at Mt Sinai Hospital New York (2010-2015). MRI or CT and resected gross tumor measurements were available for 79 pts. After eliminating 10 samples with grossly irregular shapes and 11 samples with incomplete data, 58 tumors had complete 3D measurements. We calculated Pearson correlation coefficients for paired variables (radiology vs pathology size in 1D, 2D and 3D). Results: Imaging measures correlated well with direct tumor measurements. Pearson correlation coefficients for 1D, 2D and 3D measurements were 0.93, 0.72 and 0.78, respectively (all p < 0.01). The SEM (radiology/pathology size) = 0.13, i.e. 13% for 1D measurements. Thus, T stage could be incorrectly assigned in up to 13% of samples near 5 cm in size; this proportion decreases the further the tumor is from the 5 cm cutoff. Conclusions: As shown previously in the rationale for RECIST, 1D measures provide the smallest variance between radiology and pathology. The situation is made more complex in AJCC version 8, with four T categories. It remains unclear how to stage the 10-15% of primary STS with irregular shapes. These data support the use of nomograms for risk assessment rather than using bins created by the AJCC tumor staging system. 3D tumor volumes, if used at all, may play a greater role when assessing therapy responses or contending with tumors of irregular shape, rather than for routine STS staging.

Elevated preoperative peripheral blood neutrophil-to-lymphocyte ratio to predict clinical outcome in patients with localized soft tissue sarcoma.

11049 Background: Recent studies suggest that markers of systemic inflammation such as blood neutrophil-to-lymphocyte ratio (NLR) may be prognostic for various cancers, though its clinical utility has not been widely accepted. This study aims to investigate its clinical relevance in patients (pts) with soft tissue sarcoma (STS). Methods: Five hundred and twenty-nine pts with localized STS who had available pre-operative blood counts at the time of diagnosis were retrospectively examined. An optimal cutoff for high NLR ( > 2.5) in predicting overall survival (OS) and relapse-free survival (RFS) in pts who underwent curative surgery (n = 473) was determined using receiver operating curve analyses. Cutoffs for platelet-lymphocyte ratios (PLR, > 180) and lymphocyte-monocyte ratios (LMR, < 3.6) were similarly obtained. Survival analysis was performed using the Kaplan-Meier method and multivariate Cox proportional models. Median follow-up was 40 months. Results: A high NLR was present in 311 (58.8%) pts, which was significantly associated with tumor grade ( p< 0.0001), depth ( p= 0.003) and size > 5 cm ( p= 0.0242), but not with age at diagnosis, sex or ethnicity. High NLR was associated with both worse OS (HR 1.78; 95%CI 1.28-2.47; p= 0.0005) and RFS (HR 1.54; 95%CI 1.17-2.03; p= 0.0019), as were age at diagnosis, tumor grade, size, PLR and LMR. In multivariate models adjusted for clinicopathological predictors of survival, only NLR, in addition to tumor grade and size, were independently associated with worse OS (HR 1.52; 95%CI 1.09-2.11; p= 0.0131) and RFS (HR 1.42; 95%CI 1.08-1.85; p= 0.0114). Analysis of survival according to American Joint Committee on Cancer (AJCC) stages subdivided as NLR-high and NLR-low revealed a significant worse prognosis for NLR-high subgroups ( p< 0.0001), with a 2.2-fold and 1.5-fold higher risk of death within stages II (HR 2.20; 95%CI 1.20-4.01; p= 0.0103) and III (HR 1.55; 95%CI 1.01-2.37; p= 0.0459), respectively. Conclusions: High NLR is an independent marker of poor prognosis among pts with localized STS. Inclusion of NLR as a classifier into the AJCC staging of STS may improve estimation of survival.

Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology.

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy.

Whole-body magnetic resonance imaging in myxoid liposarcoma: A useful adjunct for the detection of extra-pulmonary metastatic disease

Myxoid liposarcomas (MLS) are a subgroup of soft-tissue sarcomas which have a propensity for extra-pulmonary metastases. Conventional radiological staging of soft-tissue sarcomas consists of chest radiographs (CXR) and thoracic computed tomography (CT) for possible chest metastases, supplemented by magnetic resonance imaging (MRI) for local disease. The optimal radiological modality to detect extra-pulmonary metastases for systemic staging has not been proven. We reviewed the efficacy of Whole-Body MRI (WBMRI) for this purpose.33 WBMRI and simultaneous CT scans were performed in 28 patients suffering from MLS between 2007 and 2015. 38 metastases were identified in seven patients via WBMRI. Osseous lesions predominated (spine, pelvis, chest-wall and long bones), followed by soft-tissue and abdominal lesions. Of the 29 soft-tissue or osseous metastases that were within the field-of-view of the simultaneous CT scans, five soft-tissue and zero osseous metastases were identified using CT. Metastatic disease was detected in three patients solely using WBMRI, which directly influenced their management.WBMRI is a useful adjunct in the detection of extra-pulmonary metastatic disease, which directly alters patient management. WBMRI has demonstrated an ability to identify more sites of metastatic disease compared to CT. WBMRI should be used in two situations. Firstly, at diagnosis where ablative treatment will be required e.g. amputation, when the diagnosis of occult metastasis would change treatment planning. Secondly, at diagnosis of relapse to confirm if it is a solitary site of relapse prior to consideration of metastectomy.

Angiosarcoma of the head and the neck: Clinical analysis of 21 cases

Objective: To study the clinical manifestations of the head and neck angiosarcoma and the relationship of the staging with clinical treatment and prognosis. Methods: The clinical data of 21 patients with head and neck angiosarcoma treated in our hospital between January 1993 and January 2008 were retrospectively analyzed. By studying the medical records of all patients and reviewing the related literatures, we discussed the clinical manifestations of these patients and the relationship of staging with clinical treatment and prognosis of head and neck angiosarcoma based on soft tissue sarcoma staging of AJCC2002'6th. SPSS 17.0 software was used for statistical analysis. We calculated the survival rate using Kaplan-Meier method and compared the survival curves using log rank test. Results: Among these 21 cases, 10 were treated with non-combined treatment, and the other 11 cases were treated with combined treatment. There were 6 stage Ⅰ cases, 3 stage Ⅱ cases, 1 stage Ⅲ case, and 11 stage Ⅳ cases. The 1-, 3-, 5- and 10-year total survival rates were 72.2%, 41.3%, 27.5% and 13.8%, respectively. The average time of recurrence or metastasis was 4 months. Twelve cases had local recurrence (accounting for 57.1%), and 11 cases had distant metastasis (accounting for 52.4%). The metastatic sites included lymph node in 5 cases, lung in 4 cases, liver in 1 case, and bone in 1 case. Different treatment modalities (x~2=8.31, P=0.004) and tumor staging (x~2=9.74, P= 0.002) were prognostic factors. Conclusion: There are no unified treatment strategies for angiosarcoma of the head and the neck. The overall prognosis is still not ideal. Biological treatment has been applied to clinical practice. Preoperative combined treatment has positive results. Tumor staging is an important factor affecting the prognosis. Surgery-based combined treatment can improve patient survival.

Soft tissue sarcoma of the head & neck: nomogram validation and analysis of staging systems.

Soft tissue sarcomas of the head and neck (STSHN) comprise a rare group of malignancies. Our objective is to determine the utility of soft tissue sarcoma staging systems within the head and neck, and to validate an individualized soft tissue sarcoma nomogram within head and neck primary sites. Previously-untreated patients with STSHN diagnosed and treated between 1982 and 2012 were eligible (n = 319, median follow-up 46 months). Clinical variables were extracted from a prospectively-maintained database. The performance of AJCC/UICC and MSK staging systems and a soft tissue sarcoma-specific nomogram were assessed. Four-year overall survival (OS), disease specific survival (DSS), and recurrence-free survival (RFS) were 72%, 76%, and 71%, respectively. AJCC/UICC and MSK staging systems accurately stratified outcomes (OS, DSS, and RFS; P < 0.001 for all comparisons). The nomogram stratified outcomes by quartile (P < 0.001), and predicted risk of death at 4, 8 and 12 years (P < 0.001). Concordance indices for overall survival for the AJCC/UICC system, MSK system, and the nomogram were 0.71, 0.70, and 0.78, respectively. Oncologic outcomes among groups of patients with STSHN can be accurately predicted using both the AJCC/UICC and MSK staging systems. A soft tissue sarcoma-specific nomogram provides reliable, individualized prognostic information for patients with STSHN.

The role of 18F-FDG PET/CT in diagnosis and staging of musculoskeletal soft tissue sarcomas

Adequate resection of soft tissue tumors reduces the risk of local recurrence and improves survival. Lymphatic dissemination of these tumors may occur and is related to worst prognosis. Proper staging thus improves patient management. Therefore, 18F-FDG PET/CT has increasingly been incorporated in the management of patients with soft tissue sarcomas, from grading, prognostic evaluation, diagnosis, staging and subsequent patient management. Formerly, soft tissue malignancies, after diagnosis, were staged with only chest radiographs and chest CT. Initial studies using solely dedicated PET scanners revealed a low sensitivity to detect metastases, especially in the detection of lung nodules. However, with the advent of 18F-FDG PET/CT hybrid imaging, there is growing evidence of the added benefit in performing this whole-body imaging modality in the work-up of these patients. In this review, we will discuss the incremental value of 18F-FDG PET/CT in the diagnosis and staging of soft tissue sarcomas, the patterns of uptake according to the different histological types as well as alert the nuclear medicine physician of the false-positive and false-negative situations.

Lumping, Splitting, and Making Sense: Implications of Soft Tissue Sarcoma Staging for Prognosis, Therapy, and Research

Alex B. Haynes, MD, MPH and Raphael E. Pollock, MD, PhDDepartment of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TXThe concept of staging cancer originated in the earlytwentieth century as a way of understanding the extent andseverity of disease and thereby estimating prognosis. Theseearly efforts at classifying cancer were anatomically basedand as such this has provided the foundation for modernstaging. The tumor, node, metastasis classification systemof staging was first introduced in the 1940s as a stan-dardized method of anatomic staging and used assessmentof primary tumor extent, lymph node involvement, anddistant disease. Through an objective assessment of thesethree parameters, patients were able to be grouped in arough estimation of the severity of the disease. Thisapproach was adopted by the American Joint Committeeon Cancer (AJCC) and provides the underpinning of themodern staging system for solid tumors. Refinements havebeen made for individual malignancies whereby otherpertinent factors such as serum markers and histologicgrade have been included in staging.As therapeutic strategies have improved and becomemore complex, stage has played an important role intreatment decisions, in comparisons of results acrossinstitutions and across time, and in assessment of progno-sis. The decision to include elements of multimodal care isoften based on stage, such as in adjuvant therapy formalignant melanoma or neoadjuvant approaches to rectalcancer. Additionally, standardized staging is important inclassifying patients for clinical research so that findingsabout treatment and outcome can be applied to theappropriate population of patients. Finally, staging plays animportant role in cancer epidemiology. Measuring anddefining the rate of early and late stage presentation canhelp to better direct prevention, screening, and diagnosis,as well as the application of the correct therapy at thecorrect point in time.Staging of soft tissue sarcoma presents some uniquedifficulties, which Maki et al. illustrate.

Toward Better Soft Tissue Sarcoma Staging: Building on American Joint Committee on Cancer Staging Systems Versions 6 and 7

Based on review of patient data in case conferences over time, we hypothesized that clinically relevant data are omitted in routine soft tissue sarcoma staging. We examined subsets of a prospectively collected single institution soft tissue sarcoma database with respect to criteria of the AJCC versions 6 (2002) and 7 (2010) staging systems and examined their clinical outcomes. Relapse-free survival decreases with increasing primary tumor size in four categories, versus two categories used in AJCC 6 and 7 staging. Disease-specific survival decreases over three categories. Conversely, omission of tumor depth as a prognostic factor in version 7 appears supported, since tumor depth is not an independent risk factor for disease-specific survival by multivariate analysis. Patients with nodal disease and no other metastases fare better than patients with other metastases, but have inferior outcomes compared with patients with large high-grade tumors without nodal metastasis. Multivariate analysis identified size, site, grade, age, nodal metastatic disease, and other metastatic disease as independent risk factors for disease-specific survival. Versions 6 and 7 criteria are tacit regarding anatomic site and histology for tumors with identical FNCLCC grade. Improved patient risk assessment may be achieved by staging using a larger number of size categories. Staging system refinements come at the cost of a larger number of staging categories. Histology or site-specific staging systems, nomograms or Bayesian belief networks may provide more accurate means to assess clinical outcomes.

Radical Surgery with a Therapeutic Lymphadenectomy in a Rare Case of MPNST with Synchronous Regional and Non Regional Lymph Node Metastasis

Sir, Lymph node metastases from adult soft tissue sarcomas (STS) are rare. They are frequently associated with certain histological subtypes which include angiosarcoma, embryonal rhabdomyosarcoma and epithelioid sarcomas. Therapeutic lymphadenectomy is considered appropriate treatment for isolated regional lymph nodes metastasis (RLNM) from STS and can potentially improve long-term survival [1]. The role of surgery in the presence of non regional lymph node metastasis is not clearly defined in literature. A 19-year-old girl presented to us with a recurrent painless swelling in the right supraclavicular region for 4 months duration. She supposedly underwent a surgical excision for a similar swelling a year prior, elsewhere. Clinical examination confirmed the presence of a well circumscribed, multi-nodular swelling measuring 4 × 3cm in the right supraclavicular fossa, with involvement of the overlying skin; which had healed by secondary intention following the first surgery (Fig. 1). There were multiple significant discrete lymph nodes in bilateral cervical and right axillary regions. (Along levels II, III and IV on the right side and levels III and IV on the left side) Histopathology of the trucut biopsy from the supraclavicular swelling with immunohistochemistry correlation suggested a diagnosis of high grade malignant peripheral nerve sheath tumor (MPNST) (Figs. 2 and 3a–d). A PET-CT scan done for staging, showed metabolically active uptakes in the recurrent tumor in the supraclavicular fossa and in the multiple bilateral cervical and right axillary lymph nodes (Fig. 4). Fig. 1 Clinical photograph at presentation Fig. 2 a. H&Ex20—Showing tumor arranged in sheets and vague fascicles composed of round, oval to spindle shaped cells having pleomorphic vesicular nuclei and prominent nucleoli, with peritheliomatous arrangement at certain areas. b IHCX20—Tumor ... Fig. 3 a H&Ex20—Showing tumor with epithelioid and spindle cell areas. b IHCX20—Tumor cells showing immumopositivity to vimentin. c IHCX20—Tumor cells showing immumopositivity to S-100p. d IHCX20—70 % of the tumor ... Fig. 4 PET-CT scan showing metabolically active uptakes in the recurrent tumor in the supraclavicular fossa and in the multiple bilateral cervical and right axillary lymph nodes The patient was taken up for a radical surgery; en-bloc excision of the recurrent tumor along with the overlying skin was done, safeguarding the truck of the brachial plexus. A bilateral selective neck dissection involving levels II to V and a complete right axillary clearance was done. The resultant skin and soft tissue defect in the right supraclavicular region was reconstructed using a right latissimus dorsi myocutaneous flap (Fig. 5a, b). The final histopathology confirmed the preoperative diagnosis of MPNST. 6 out of the 14 right sided cervical lymph nodes, 4 out of the 12 left sided cervical lymph nodes and 9 out of the 15 right sided axillary lymph nodes showed metastatic tumor deposits. The patient completed 60 Grey of adjuvant external beam radiotherapy to the tumor bed and is disease free for the past one and half years following surgery. Fig. 5 a Intra-operative photograph following the en-bloc resection of the tumor and the therapeutic cervical and axillary lymph node dissections. b Immediate post operative photograph following latissimus dorsi flap reconstruction MPNST is currently the recommended term for all malignancies that arise from the peripheral nervous system or that show nerve sheath differentiation [2]. It is the sixth most common type and accounts for about 3–10 % of all STS [3]. Roughly half of all MPNSTs are sporadic; the remaining tumors are found in patients who are diagnosed with Neurofibromatosis Type I. These tumors commonly arise in the buttocks, thigh, brachial plexus, upper arm and para-spinal regions. Clinically, these tumors are aggressive, locally invasive, and highly metastatic. The most common site of distant metastasis is the lung, followed by bone and pleura. RLNM are uncommon, occurring in <10 % of patients, and are mainly seen in conjunction with widespread metastasis. There is currently no standardized treatment for MPNST other than radical surgery even in the presence of RLNM [4]. Patients who present with isolated RLNM have an improved survival compared with patients who present with regional and distant metastasis at diagnosis. Although routine use of FDG PET imaging as part of the initial staging of soft-tissue sarcomas is not recommended [5], it is reported to be useful in certain special circumstances as in our patient wherein the likelihood of distant metastasis was high. The absence of distant metastasis in the PET-CT prompted us to undertake a potentially curative surgery in our patient despite the presence of non regional lymph node metastasis; this case thus expands our knowledge on the biology of MPNST.

How well do we communicate risk? An evaluation of AJCC version 6 and 7 staging systems for soft tissue sarcomas.

10001 Background: Cancer staging systems allow physicians to communicate risks of a particular clinical situation with one another and with patients. Few data have been presented to support the update of a commonly employed staging system for soft tissue sarcoma (STS). We examined American Joint Committee on Cancer (AJCC) versions 6 (2002) and 7 (2010) staging systems for patients with primary STS using a single institution clinically annotated prospective database. Methods: Subsets of the prospectively collected Memorial Sloan-Kettering Cancer Center STS database of 8647 patients from 1982 to 2010 were examined with respect to criteria of the AJCC versions 6 and 7 staging systems. Results: Tumor size does not appear to be adequately addressed in version 6 or 7. Relapse-free survival was statistically worse for increasing size of primary STS &lt;5, 5-10, 10-15, and &gt;15 cm; in comparison, overall survival decreased over three size categories (&lt;5, 5-10, &gt;10 cm). Tumor depth, a statistically significant factor in patient outcomes that is included in version 6, is functionally omitted in version 7. Patients with node involvement without other metastases fare statistically worse than patients with large, high grade tumors without nodal metastasis, as shown previously. Version 6 and 7 criteria do not address effects of primary anatomic site and histology, even for tumors with same FNCLCC grade. Sarcoma subtypes defined after publication of FNCLCC criteria are difficult to incorporate into existing guidelines. Conclusions: Improved prognostication of STS outcomes is better achieved by staging according to anatomic primary site, depth, and a larger number of size categories. Histology-specific nomograms improve prognostic accuracy, such as those for liposarcoma, GIST, or rhabdomyosarcoma. Staging accuracy increases at the cost of portability and simplicity. The increasing difficulties with use of a single STS staging system highlight the potential benefit of newer techniques, such as patient-specific nomograms or Bayesian networks. Staging systems will require significant modifications to incorporate increasingly sophisticated molecular diagnostics.

New FIGO staging for uterine sarcoma identifies a population of early stage patients with minimal risk for recurrence

Objective: Uterine leiomyosarcoma (LMS) has been traditionally staged by modified 1988 FIGO criteria for endometrial adenocarcinoma. Staging may also be performed utilizing the AJCC soft tissue sarcoma staging system. In 2009, FIGO staging for uterine LMS was established. The aim of this study was to compare the 3 systems in order to determine their accuracy in predicting disease specific survival.

FDG PET/CT in Initial Staging of Adult Soft-Tissue Sarcoma

Soft-tissue sarcomas spread predominantly to the lung and it is unclear how often FDG-PET scans will detect metastases not already obvious by chest CT scan or clinical examination. Adult limb and body wall soft-tissue sarcoma cases were identified retrospectively. Ewing's sarcoma, rhabdomyosarcoma, GIST, desmoid tumors, visceral tumors, bone tumors, and retroperitoneal sarcomas were excluded as were patients imaged for followup, response assessment, or recurrence. All patients had a diagnostic chest CT scan. 109 patients met these criteria, 87% of which had intermediate or high-grade tumors. The most common pathological diagnoses were leiomyosarcoma (17%), liposarcoma (17%), and undifferentiated or pleomorphic sarcoma (16%). 98% of previously unresected primary tumors were FDG avid. PET scans were negative for distant disease in 91/109 cases. The negative predictive value was 89%. Fourteen PET scans were positive. Of these, 6 patients were already known to have metastases, 3 were false positives, and 5 represented new findings of metastasis (positive predictive value 79%). In total, 5 patients were upstaged by FDG-PET (4.5%). Although PET scans may be of use in specific circumstances, routine use of FDG PET imaging as part of the initial staging of soft-tissue sarcomas was unlikely to alter management in our series.

Magnetic resonance imaging and positron emission tomography-computed tomography evaluation of soft tissue sarcoma with surgical and histopathological correlation

Purpose:The aim of this study was to evaluate the role of positron emission tomography-computed tomography (PET-CT) and magnetic resonance imaging (MRI) in characterization and pre-operative staging of soft-tissue sarcoma (STS) and correlating with operative and histopathological findings.Materials and Methods:Twenty patients (age range 16-72 years [mean 44.4 years]) with resectable and STS were included in this prospective study. Pre-operative MRI was carried out in all patients with acquisition of T1W, T2W, and short tau inversion recovery (STIR) sequences in appropriate planes. Contrast enhanced MRI was performed in four patients. Whole body PET-CT was performed in 13 patients. Demographic data, clinical features, pre-operative imaging analysis, operative, and histopathological findings were analyzed using SPSS software version 11.5.Results:The most common histologic type was malignant fibrous histiocytoma (MFH) (30%). Of 18 STSs 20 were high-grade. Agreement existed between MR and operative size. MRI had 100% negative predictive value (NPV) in predicting neurovascular bundle involvement. However, positive predictive value (PPV) was 33%. MRI had PPV of 20% while PET-CT had 50% PPV in detecting lymph node involvement. Overall staging accuracy of MRI was 75% when correlated with surgical and histopathological findings. Combined PET-CT and MRI staging, in 13 patients, was better (92.31%) when compared with staging with MRI (84.62%). Specific diagnosis on image characteristics was correctly suggested in 35% patients.Conclusions:MRI is the robust modality in local staging of STSs and PET-CT adds greater accuracy to overall staging in combination with MRI.

Sarcomas of Soft Tissue and Bone

Sarcomas originate from bone or soft tissue. The most common bone sarcomas are osteosarcomas, Ewing sarcomas, and chondrosarcomas. Soft tissue sarcomas develop in fibrous tissue, fat, muscle, blood vessels, and nerves. Historically, soft tissue sarcomas of the trunk and extremities were reported separately from those of visceral organs (e.g., gastrointestinal and gynecologic sarcomas). This chapter discusses the classification, epidemiology, diagnosis, staging, and treatment of sarcomas of bone and cartilage, and classic soft tissue sarcomas. Management of Kaposi sarcoma, gastrointestinal stromal tumors (GISTs), mesothelioma, and rhabdomyosarcoma is also described. Figures include images of patients with osteosarcoma, liposarcoma, uterine leiomyosarcoma, GIST, and osteosarcoma in a patient with Paget disease of bone. Tables list epidemiologic features of sarcomas, a summary of sarcomas by histology, familial syndromes associated with increased risk of sarcoma, survival rates in sarcoma patients, staging of soft tissue sarcomas, and results of a meta-analysis of doxorubicin-based adjuvant chemotherapy for localized resectable soft tissue sarcoma. This chapter contains 126 references.

Sarcomas of the Soft Tissue and Bone

Sarcomas originate from bone or soft tissue. The most common bone sarcomas are osteosarcomas, Ewing sarcomas, and chondrosarcomas. Soft tissue sarcomas develop in fibrous tissue, fat, muscle, blood vessels, and nerves. Historically, soft tissue sarcomas of the trunk and extremities were reported separately from those of visceral organs (e.g., gastrointestinal and gynecologic sarcomas). This chapter discusses the classification, epidemiology, diagnosis, staging, and treatment of sarcomas of bone and cartilage, and classic soft tissue sarcomas. Management of Kaposi sarcoma, gastrointestinal stromal tumors (GISTs), mesothelioma, and rhabdomyosarcoma is also described. Figures include images of patients with osteosarcoma, liposarcoma, uterine leiomyosarcoma, GIST, and osteosarcoma in a patient with Paget disease of bone. Tables list epidemiologic features of sarcomas, a summary of sarcomas by histology, familial syndromes associated with increased risk of sarcoma, survival rates in sarcoma patients, staging of soft tissue sarcomas, and results of a meta-analysis of doxorubicin-based adjuvant chemotherapy for localized resectable soft tissue sarcoma. This chapter contains 126 references.