Metastasectomy for oligometastatic bone disease of the appendicular skeleton: A concise review.

As the life expectancy of patients with carcinomas improves, there is an associated increase in the risk of development of metastatic bone disease. Patients with solitary or oligometastatic bone disease of the appendicular skeleton may benefit from metastasectomy and reconstruction to improve overall survival. Specifically, patients with renal cell carcinoma and a solitary metastatic bone lesion will have long-term survival benefit from metastasectomy and reconstruction. There remains controversy in the literature about metastasectomy for solitary metastatic bone disease in other common carcinomas, such as breast and thyroid.

Giant Cell Tumor of the Distal Radius: A Review.

Giant cell tumor of the distal radius presents a significant challenge in management due to high risk of recurrence and potential loss of function. Shared decision-making guides management, particularly for more advanced lesions. Intralesional curettage can optimize wrist function but at the cost of a higher recurrence risk. Wide resection decreases local recurrence but has higher complication rates regardless of reconstruction method. No functional difference exists between motion-preserving procedures and arthrodesis; therefore, patients should be clearly informed of the risks and benefits of each treatment option.

Conventional Cartilaginous Tumors: Evaluation and Treatment.

¼: Enchondromas are benign cartilaginous lesions that rarely require surgical intervention. ¼: Atypical cartilaginous tumors (ACTs), also referred to as grade-1 chondrosarcomas, may be managed without any intervention or with extended intralesional curettage and bone-void filling. ¼: High-grade chondrosarcomas, or grade-2 and 3 chondrosarcomas, should be managed aggressively with wide resection. ¼: Chemotherapy and radiation do not currently play a role in the treatment of chondrosarcomas. ¼: Differentiating an enchondroma from an ACT and an ACT from a high-grade chondrosarcoma can be difficult and requires clinical experience, radiographic and advanced imaging, and possibly a biopsy. Ultimately, a multidisciplinary team that includes a musculoskeletal oncologist, a radiologist, and a pathologist is needed to make the most appropriate diagnosis and treatment plan for each patient.

A Rare Case of Progressive Malignant Triton Tumor With Rare Somatic Mutation in TSC2 Gene.

BACKGROUND: Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumor with additional rhabdomyolysis differentiation that shows rapid progression and poor clinical outcomes. CASE REPORT: We report the case of an adult male with a metastatic MTT. Despite extensive counseling, the patient initially refused recommended treatment. Upon disease progression, the patient was admitted to our institution and multiple distant organ metastases were found. The patient underwent an above-knee amputation followed by palliative chemotherapy. The patient died a few months later due to rapid disease progression. CONCLUSION: To our knowledge, this is the first report of a case of MTT with multiple splenic metastases. We also describe the first finding of a frame-shift mutation in the tuberous sclerosis complex 2 (TSC2) gene in a patient with MTT. Because of limited clinical experience and the lack of clinical trials, the effects of chemotherapy and radiation therapy for MTT remain controversial. However, given the aggressive nature of these tumors and the tendency for early recurrence and metastasis, prompt diagnosis and early surgical treatment are crucial for the best outcomes.

The Educational Impact of a Fellowship-trained Orthopaedic Oncologist.

INTRODUCTION: Musculoskeletal oncology is a subspecialty of orthopaedics with few fellowship-training locations. Although orthopaedic oncologists comprise a minority within the field of orthopaedic surgery, most work at academic centers and serve in leadership roles with notable impact on patients and the training of residents. This article investigates the objective impact orthopaedic oncologists have regarding resident operative case volume and performance on in-training examinations. METHODS: The William Beaumont Army Medical Center and Texas Tech University Health Sciences Center of El Paso combined orthopaedic residency program's case logs and Orthopaedic In-Training Examination (OITE) scores between 2013 and 2018 were reviewed. This provided 3 academic years of data before and after an orthopaedic oncology faculty member arrived in 2016. The case volume and OITE examination performance before and after the addition of the orthopaedic oncology faculty member were compared. RESULTS: After the addition of an orthopaedic oncology faculty member, a significant increase was observed in the program's OITE overall correctly answered questions (171.30 versus 181.03, P = 0.004) and oncology subsection percentile (56th to the 66th percentile, P = 0.038). An increase was also observed in resident oncology case volume from 29 oncology cases per year to 138 cases on average (P = 0.022). DISCUSSION: The addition of a fellowship-trained orthopaedic oncologist results in increased exposure to orthopaedic oncology cases and improved resident performance on the OITE. This may correlate to improved American Board of Orthopaedic Surgeons Part I pass rates and improved overall resident satisfaction.

Long-term outcome of iliosacral resection without reconstruction for primary bone tumours.

AIMS: Iliac wing (Type I) and iliosacral (Type I/IV) pelvic resections for a primary bone tumour create a large segmental defect in the pelvic ring. The management of this defect is controversial as the surgeon may choose to reconstruct it or not. When no reconstruction is undertaken, the residual ilium collapses back onto the remaining sacrum forming an iliosacral pseudarthrosis. The aim of this study was to evaluate the long-term oncological outcome, complications, and functional outcome after pelvic resection without reconstruction. METHODS: Between 1989 and 2015, 32 patients underwent a Type I or Type I/IV pelvic resection without reconstruction for a primary bone tumour. There were 21 men and 11 women with a mean age of 35 years (15 to 85). The most common diagnosis was chondrosarcoma (50%, n = 16). Local recurrence-free, metastasis-free, and overall survival were assessed using the Kaplan-Meier method. Patient function was evaluated using the Musculoskeletal Tumour Society (MSTS) and Toronto Extremity Salvage Score (TESS). RESULTS: At a mean follow-up of 159 months (1 to 207), 23 patients were alive without disease, one was alive with lung metastases, one was alive following local recurrence, four were dead of disease, and three had died from other causes. The overall ten-year survival was 77%. There was only one (3%) local recurrence, which occurred at 26 months. There were 18 complications in 17 patients; 13 wound healing complications/infections, three fractures, one pulmonary embolism, and one dislocation of the hip. Most complications occurred early. The mean functional scores were 21.1 (SD 8.1) for MSTS-87, 67.3 (SD 23.9) for MSTS-93 and 76.2 (SD 20.6) for TESS. CONCLUSION: Patients requiring Type I or Type I/IV pelvic resections can expect a good oncological outcome and a high rate of local control. Complications are generally acute in nature and are easily manageable. These patients achieved a good functional outcome without the need for bony reconstruction. Cite this article: Bone Joint J 2020;102-B(6):779-787.

Giant cell tumor of the talus: A case report.

Giant cell tumor is a benign primary bone neoplasm which most often occurs in a periarticular location. Involvement of the bones of the foot and ankle is rare, and there have been a limited number of previous case reports involving the talus. Here we report a case of giant cell tumor of the talus, which was initially radiographically occult in a 43-year-old female, with emphasis on MRI imaging characteristics. The patient underwent surgical excision and curettage. Histological examination revealed the presence of spindle cells admixed with giant cells, confirming GCT. We further provide an overview of the radiological findings of GCT. Giant cell tumor is a benign bone neoplasm of mesenchymal origin, identified by multinucleated giant cells [1]. GCT is locally aggressive and can destroy adjacent bone and articulations. The most commonly affected bones are the distal femur, proximal tibia, and distal radius, with an epiphyseal predominance in 90% of cases [2]. Presentations are mostly mono-ostotic, however multicentricity may occur in younger patients [3]. Very few cases have been reported in the bones of the feet, an incidence of 1%-2% have been previously reported [4]. GCT is seen between ages 20 and 40 years, with a 56% predominance in females [3]. Although benign, 1%-9% cases may "metastasize" to the lungs. The initial treatment is surgical removal, either en bloc, or more commonly intralesional curettage and the use of adjuvants. Even after resection, GCT has a high recurrence rate [2]. The trigger for GCT is currently unknown. However, a majority of cases have cytogenetic abnormalities of telomeric associations (tas). Involvement of the RANK pathway is also believed to contribute to the pathogenesis of GCT [2].

Pilomatrix Carcinoma of the Antecubitum.

We present the case of a 46-year-old male with atypically large left elbow pilomatrix carcinoma present for 10 years with emergent excision after developing life-threatening hemorrhage. Pilomatrix carcinoma is a dermal-based malignant tumor typically of the head and neck region. Histopathology shows islands of basaloid cells, shadow cells, and fibromyxoid fibroma. Reoccurrence is seen in 23% of cases on an average of six months after primary excision. The current standard of care is wide excision with close follow-up.

Foot plantar soft tissue malignant myoepithelioma tumor: Case report and review of the literature.

The spectrum of myoepithelial tumors usually occur in the salivary glands, and occasionally in the skin, breast, upper aero-digestive tract, and soft tissues. The myoepithelial tumors have no sex predominance and usually present within a wide range of age of distribution around the third and fifth decades. We describe a 12 year old male patient with primary malignant myoepithelial tumor in the foot plantar soft tissues. Including this tumor with unusual location, and age of presentation is essential in the differential diagnosis for soft tissue tumors in the pediatric population.

Diabetic Control Predicts Surgical Site Infection Risk in Orthopaedic Trauma Patients.

OBJECTIVES: (1) To determine the incidence of surgical site infections (SSIs) in diabetic orthopaedic trauma patients and (2) to establish a protocol for managing diabetes mellitus (DM) in orthopaedic trauma patients. DESIGN: Retrospective cohort study. SETTING: Level 1 Trauma Center. PATIENTS: All diabetic orthopaedic trauma patients who underwent surgical intervention with at least 1 month follow-up. Patients were classified as poorly controlled or controlled diabetic patients based on admission hemoglobin A1c and blood glucose (BG) levels. INTERVENTIONS: Orthopaedic surgical intervention in accordance with fracture type and a standardized diabetes management protocol with internal medicine comanagement. MAIN OUTCOME MEASUREMENT: SSI incidence. RESULTS: There were 260 patients during the study period. Two hundred two (77.7%) were included in the final analysis. Seventy-five (37.1%) patients met the criteria for poorly controlled DM. The overall rate of SSI was 20.8%; 32.0% for poorly controlled diabetic patients, and 14.2% for controlled diabetic patients (P < 0.01). The admission blood glucose level (BG, P = 0.05), but not discharge BG, was associated with SSI incidence. CONCLUSIONS: Trauma patients with poorly controlled DM have a higher rate of SSIs than patients with controlled DM. Perioperative diabetes control does not seem to decrease infection rates in these patients. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Analysis of Margin Classification Systems for Assessing the Risk of Local Recurrence After Soft Tissue Sarcoma Resection.

Purpose To compare the ability of margin classification systems to determine local recurrence (LR) risk after soft tissue sarcoma (STS) resection. Methods Two thousand two hundred seventeen patients with nonmetastatic extremity and truncal STS treated with surgical resection and multidisciplinary consideration of perioperative radiotherapy were retrospectively reviewed. Margins were coded by residual tumor (R) classification (in which microscopic tumor at inked margin defines R1), the R+1mm classification (in which microscopic tumor within 1 mm of ink defines R1), and the Toronto Margin Context Classification (TMCC; in which positive margins are separated into planned close but positive at critical structures, positive after whoops re-excision, and inadvertent positive margins). Multivariate competing risk regression models were created. Results By R classification, LR rates at 10-year follow-up were 8%, 21%, and 44% in R0, R1, and R2, respectively. R+1mm classification resulted in increased R1 margins (726 v 278, P < .001), but led to decreased LR for R1 margins without changing R0 LR; for R0, the 10-year LR rate was 8% (range, 7% to 10%); for R1, the 10-year LR rate was 12% (10% to 15%) . The TMCC also showed various LR rates among its tiers ( P < .001). LR rates for positive margins on critical structures were not different from R0 at 10 years (11% v 8%, P = .18), whereas inadvertent positive margins had high LR (5-year, 28% [95% CI, 19% to 37%]; 10-year, 35% [95% CI, 25% to 46%]; P < .001). Conclusion The R classification identified three distinct risk levels for LR in STS. An R+1mm classification reduced LR differences between R1 and R0, suggesting that a negative but < 1-mm margin may be adequate with multidisciplinary treatment. The TMCC provides additional stratification of positive margins that may aid in surgical planning and patient education.

Intermetatarsal bursa primary synovial chondromatosis: case report and review of the literature.

Primary synovial chondromatosis is a benign neoplastic process, occurring mostly in large joints, more rarely in tendon sheaths, and extremely uncommonly in bursae. We describe a patient with primary synovial chondromatosis arising in the fourth intermetatarsal bursa. Knowledge of the bursal anatomy of the forefoot, and of characteristic imaging findings and the pathogenesis of synovial chondromatosis, is essential in including this uncommon entity in the differential when occurring in unusual locations.

An Analysis of Tumor- and Surgery-Related Factors that Contribute to Inadvertent Positive Margins Following Soft Tissue Sarcoma Resection.

BACKGROUND: The risk of local recurrence (LR) after soft tissue sarcoma (STS) resection is higher in the setting of inadvertent positive margins (IPMs). This study assessed whether both tumor- and surgery-related factors contribute to IPMs, and whether tumor- versus surgery-related IPMs differ in LR or overall survival (OS). METHODS: Retrospective review of a tertiary center database identified patients with IPMs following STS resection between 1989 and 2014. Of 2234 resected STSs, 309 (13%) had positive margins; 89 (4%) were IPMs. Mean follow-up was 52 months, mean tumor size was 9.2 cm, and 55% were high grade. Cases were categorized as surgery-related (67, 75%) or tumor-related (22, 25%). RESULTS: There was a significant difference in positive margin location, with the deep margin commonly involved in surgery-related IPMs (55% vs. 9%; p < 0.001). Tissue type also differed (p = 0.01), with surgery-related IPMs frequently in muscle (33%), while tumor-related IPMs favored subcutaneous tissues (41%). STSs with surgery-related IPMs were larger (p = 0.01). Histologic subtypes differed (p = 0.02), with myxofibrosarcoma and undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma predominating in tumor-related IPMs (82%). The cumulative probability of LR after IPMs, with death as a competing risk, was 28% (95% confidence interval [CI] 18-35) at 5 years and 37% (95% CI 24-45) at 10 years. Mortality was 28% (95% CI 18-38) at 5 years and 38% (26-50) at 10 years. There was no difference in LR (p = 0.91) or OS (p = 0.44) between surgery- and tumor-related IPMS. CONCLUSIONS: IPMs after STS resection results in substantial LR risk. While demonstrating distinct surgery- and tumor-related contributions, there was no between-group difference in LR or OS. These results may aid in avoiding IPMs. LEVEL OF EVIDENCE: Therapeutic Level III, retrospective comparative study.

Modeling synovial sarcoma metastasis in the mouse: PI3'-lipid signaling and inflammation.

Solid tumor metastasis is a complex biology, impinged upon by a variety of dysregulated signaling pathways. PI3'-lipid signaling has been associated with metastasis and inflammation in many cancers, but the relationship between tumor cell-intrinsic PI3'-lipid signaling and inflammatory cell recruitment has remained enigmatic. Elevated PI3'-lipid signaling associates with progression of synovial sarcoma, a deadly soft tissue malignancy initiated by a t(X;18) chromosomal translocation that generates an SS18-SSX fusion oncoprotein. Here, we show in genetically engineered mouse models of locally induced expression of SS18-SSX1 or SS18-SSX2 that Pten silencing dramatically accelerated and enhanced sarcomagenesis without compromising synovial sarcoma characteristics. PTEN deficiency increased tumor angiogenesis, promoted inflammatory gene expression, and enabled highly penetrant spontaneous pulmonary metastasis. PTEN-deficient sarcomas revealed infiltrating myeloid-derived hematopoietic cells, particularly macrophages and neutrophils, recruited via PI3'-lipid-induced CSF1 expression in tumor cells. Moreover, in a large panel of human synovial sarcomas, enhanced PI3'-lipid signaling also correlated with increased inflammatory cell recruitment and CSF1R signal transduction in both macrophages and endothelial cells. Thus, both in the mouse model and in human synovial sarcomas, PI3'-lipid signaling drives CSF1 expression and associates with increased infiltration of the monocyte/macrophage lineage as well as neutrophils.

MicroRNAs in osteosarcomagenesis.

The etiology of osteosarcoma (OS) remains enigmatic. Particular clinical and molecular patterns, observed with high frequency in OS, suggest that it results from some yet-to-be-discovered central driver. How else can biology generate such an aggressive, metastatic, genetically and chromosomally unstable malignancy with virtually no apparent precursor neoplasms that are partway along a disease path toward OS? With this conundrum as a backdrop, the discovery of every new native molecule with power to impact a cell's biology is usually quickly followed by a search to see if this type of molecule contains the key to unlock OS biology.