Robert M. Tamurian

Predicting Survival for Well-Differentiated Liposarcoma: The Importance of Tumor Location

BACKGROUND Although well-differentiated liposarcoma (WD Lipo) is a low grade neoplasm with a negligible risk of metastatic disease, it can be locally aggressive. We hypothesized that survival for WD Lipo varies significantly based on tumor location. METHODS We identified 1266 patients with WD Lipo in the Surveillance, Epidemiology, and End Results database from 1988-2004. After excluding patients diagnosed by autopsy only, those lacking histologic confirmation, those lacking data on tumor location, and those with metastatic disease or unknown staging information, we arrived at a final study cohort of 1130 patients. Clinical, pathologic, and treatment variables were analyzed for their association with overall survival (OS) and disease-specific survival (DSS) using Kaplan-Meier analysis and Cox proportional hazards multivariate models. RESULTS Mean age was 61 y (± 14.6), 72.2% were white, and 60.4% were male. Eighty-one percent of patients were treated with surgical therapy alone, 4.6% were treated with radiotherapy (RT) alone, and 12.9% were treated with both surgery and RT. Extremity location was most common (41.6%), followed by trunk (29%), retroperitoneal/intra-abdominal (RIA, 21.6%), thorax (4.2%), and head/neck (3.6%). With a median follow-up of 45 mo, median OS was 115 mo (95% confidence interval [CI] 92-138 mo) for RIA tumors compared to not reached for other tumor locations (P = 0.002). On multivariate analysis, increasing age and RIA location both predicted worse OS and DSS while tumor size, race, sex, receipt of RT, and Surveillance, Epidemiology, and End Results (SEER) stage did not. Tumor size became a significant predictor of worse DSS, but not OS, only when site, SEER stage, and extent of resection were removed from the multivariate model. Non-RIA locations, including extremity, experienced statistically similar OS, but 5-y DSS for trunk location was intermediate [92.3%, (95% CI 88.5%-96.1%) compared with 98.0% (95% CI, 96.2%-99.8%) for extremity and 86.6 (95% CI 81.1%-92.1%) for RIA, P < 0.001]. CONCLUSIONS Among patients with WD Lipo, RIA location is associated with significantly worse outcomes independent of tumor size. Future studies should focus on the anatomic and biologic reasons for these differences.

Lack of survival benefit following adjuvant radiation in patients with retroperitoneal sarcoma: A SEER analysis

BACKGROUND The benefit of radiation therapy (RT) among patients with retroperitoneal sarcoma (RPS) is controversial. We performed a retrospective analysis of the effect of RT on survival among RPS patients using a nationwide cancer registry. METHODS Utilizing data from the Surveillance, Epidemiology, and End Results (SEER) database, we identified 2308 cases of RPS from 1988 to 2004. We excluded 773 cases for age < 18, identification by autopsy only, absence of histologic confirmation, presence of metastatic disease, or lack of surgical intervention. Overall survival (OS) and disease-specific survival (DSS) were estimated using the Kaplan-Meier method. Multivariate analysis was performed using a Cox proportional hazards model, adjusting for significant covariables. RESULTS Among 1535 patients who met entry criteria, RT was administered to 373 patients (24.3%). The majority of RT (n = 300, 80.4%) was administered postoperatively. Median OS was 60 and 60 mo, respectively, for patients receiving and not receiving RT (P = 0.59). Median DSS was 86 and 117 mo, respectively, for patients receiving and not receiving RT (P = 0.84). On multivariate analysis, younger age, female gender, low and intermediate histologic grade, liposarcoma histology, tumor size 5-10 cm, and completeness of resection all independently predicted better OS and DSS, while RT did not (HR for OS with RT 0.92, 95% CI 0.78-1.09 and HR for DSS with RT 0.96, 95% CI 0.78-1.17). On subgroup analysis by histology, patients with malignant fibrous histiocytoma (MFH) receiving RT demonstrated statistically improved OS (P = 0.002) and DSS (P = 0.01), respectively. CONCLUSIONS With the possible exception of MFH, postoperative RT offers no survival benefit in RPS. Further studies are necessary to determine if the selective application of RT is indicated.

Giant cell tumor of bone.

EDUCATIONAL OBJECTIVES As a result of reading this article, physicians should be able to: 1. Identify at-risk populations for giant cell tumor of bone. 2. Recognize the biology that drives giant cell tumor of bone. 3. Describe modern surgical and adjuvant techniques to effectively treat giant cell tumor of bone. 4. Recognize the complications associated with radiation therapy, poor resection, and adjuvant treatments. Giant cell tumor of bone (GCT) is a benign, locally aggressive bone tumor. Giant cell tumor of bone primarily affects the young adult patient population. The natural history of GCT is progressive bone destruction leading to joint deformity and disability. Surgery is the primary mode of treatment, but GCT has a tendency to recur locally despite a range of adjuvant surgical options. Pulmonary metastasis has been described. However, systemic spread of GCT rarely becomes progressive, leading to death. This review presents the clinicopathologic features of GCT and a historical perspective that highlights the current rationale and controversies regarding the treatment of GCT.

Contiguous organ resection is safe in patients with retroperitoneal sarcoma: An ACS-NSQIP analysis.

The practice of aggressive contiguous organ resection (COR) of retroperitoneal sarcoma (RPS) is controversial. We examined rates of 30‐day morbidity and mortality following resection of RPS utilizing data from the American College of Surgeons National Surgical Quality Improvement Program (ACS‐NSQIP) database.

Influence of Physician Specialty on Treatment Recommendations in the Multidisciplinary Management of Soft Tissue Sarcoma of the Extremities

IMPORTANCE Although prospective randomized data are available to guide the multidisciplinary management of soft tissue sarcoma (STS) of the extremities, controversy exists regarding adjuvant chemotherapy and radiation therapy. OBJECTIVE To determine if clinical specialty introduces bias in recommendations for multimodality treatment of STS. DESIGN Electronic survey. SETTING Database of active members of the American Society of Clinical Oncology, the Society of Surgical Oncology, and the Connective Tissue Oncology Society. PARTICIPANTS Members of specialty oncology societies with an active interest in STS. EXPOSURE Physician specialty. MAIN OUTCOMES AND MEASURES Survey responses regarding the multidisciplinary management of STS were scored on a 5-point Likert scale and analyzed using analysis of variance. RESULTS The questionnaire was completed by 320 of 490 potential respondents (65%), including medical (18%), radiation (8%), orthopedic (22%), and surgical oncologists (45%). Respondents concurred on the use of radiation therapy for margins positive for tumor, for high-grade tumors, for improvement in local control, for tumors larger than 10 cm, and for tumors in close proximity to a neurovascular bundle. Respondents diverged on the use of radiation therapy for tumors 5 to 10 cm in size, for low-grade tumors, for radiation-associated STS, and for survival benefit. Only radiation oncologists felt that radiation therapy was underutilized as a treatment modality (mean [SEM] Likert scale score, 2.44 [0.12]; P < .001). There was agreement on the use of chemotherapy for synovial sarcoma, for high-grade tumors, for tumors larger than 10 cm, for patients younger than 50 years of age, and for survival benefit. Medical oncologists were more likely to recommend chemotherapy for margins positive for tumor (mean [SEM] score, 3.12 [0.12]; P = .03) and for improvement in local control (mean [SEM] score, 2.91 [0.12] P = .08). Surgical oncologists placed the least emphasis on chemotherapy in the overall treatment plan (mean [SEM] score, 2.60 [0.07]; P = .001). CONCLUSIONS AND RELEVANCE Specialty bias exists in adjuvant treatment recommendations for STS. This highlights the importance of multidisciplinary STS tumor boards and interdisciplinary care to facilitate consensus decision making for individual patients.

Torsional Properties of Distal Femoral Cortical Defects

The optimal management of pathologic long bone lesions remains a challenge in orthopedic surgery. The goal of the current study was to investigate the effect of defect depth on the torsional properties of the distal femur. A laterally placed distal metaphyseal cylindrical defect was milled in the cortex of the distal femur in 20 composite models. The proximal extent of the defects was constant. By decreasing the radius of the cylinder that intersected this predefined cord, 4 different radii defining 4 different depths of resection of the distal femur were created for testing: 17%, 33%, 50%, and 67% cortical defects, when normalized to the width of the femur at the level of resection. Each femur was mounted into a hydraulic axial/torsion materials testing machine and each specimen underwent torsional stiffness testing and torsional failure in external rotation. The specimens with less than a 33% cortical loss consistently demonstrated a superiorly oriented spiral fracture pattern, while the specimens with greater than a 50% cortical loss consistently demonstrated an inferiorly oriented transverse fracture pattern. The cortical defects were all statistically (P<.05) less stiff in torsion as the defect grew larger. There was a strong linear correlation between the mean torsional stiffness and cortical defect size (r(2)=0.977). This observation is supported by finite element analysis. The amount of femur remaining is crucial to stability. This biomechanical analysis predicts a critical loss of torsional integrity when a cortical defect approaches 50% of the width of the femur.

Lateral elbow pain in a 21-year-old pregnant female

The present case report highlights the diagnosis and modern surgical techniques involved when treating an aggressive benign bone tumour in a skeletally mature patient. It also stresses the importance of a broad differential when examining a patient. An increase in circulating growth hormones during pregnancy could play a role in the rapid growth of particular tumours. For example, certain transforming growth factor (TGF)-β receptors have been identified in cells of aneurysmal bone cysts and a rat model has demonstrated that TGF-β isoforms are regulated differently in pregnancy, and also could have an important role in apoptosis and cell survival. The occurrence of aneurysmal bone cysts during pregnancy suggests a possible link between the pregnancy and tumour growth.