PHF1::TFE3-positive fibromyxoid sarcoma? Report of 2 cases and review of 13 cases of PHF1::TFE3-positive ossifying fibromyxoid tumor in the literature.

OBJECTIVES: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain histogenesis. Most OFMTs have benign behavior, and many harbor gene fusions involving the PHD finger protein 1 (PHF1), such as EP400::PHF1, MEAF6::PHF1, EPC1::PHF1, and PHF1::TFE3. The PHF1::TFE3 fusion is unique because PHF1 is at 5' instead of residing at 3' in the other fusions. In this study, we describe 2 cases of OFMT harboring PHF1::TFE3 fusions and review 13 published cases. METHODS: Two cases of PHF1::TFE3-positive OFMT were investigated using RNA Next-Generation Sequencing and immunohistochemistry. RESULTS: Most (12/15) of the PHF1::TFE3 OFMTs we studied were located at proximal and distal extremities, with a multinodular growth pattern. Only 1 case (1/10) had a shell of bone at the periphery. Areas morphologically similar to sclerosing epithelioid fibrosarcoma or low-grade fibromyxoid sarcoma were found in 8 of 12 (66.7%) cases. Eleven cases (11/15 [73.3%]) were regarded as malignant based on more than 2/50 high-power field mitotic figures, increased cellularity, or the presence of necrosis. Among the 9 cases with follow-up data, 2 patients died of disease (with metastases), 1 patient is alive with metastases, and 1 patient had multiple local recurrences. CONCLUSIONS: Because PHF1 is located at 3' in all the PHF1 fusions in OFMTs except PHF1::TFE3, the different driver molecular alterations suggest that OFMTs with 3'-PHF1 fusions and OFMTs with PHF1::TFE3 are different tumors. Immunohistochemistry confirmed TFE3 expression in all PHF1::TFE3 OFMTs. Because PHF1::TFE3-positive OFMTs have increased mitotic figures and tumor cellularity, with a high rate of metastasis, using the name PHF1::TFE3 positive fibromyxoid sarcoma may be appropriate.

Vertical or Horizontal (Bikini) Incision for Direct Anterior Total Hip Arthroplasty: Outcomes of Early (<90 day) Revision.

BACKGROUND: The horizontal 'bikini' incision for direct anterior approach (DAA) total hip arthroplasty has gained popularity due to its early wound healing characteristics; however, the nonextensile nature of this approach may pose problems in treating early complications. This study sought to characterize the outcomes of early revision (<90 days) in patients who underwent anterior hip arthroplasty utilizing either a traditional longitudinal incision or a horizontal (bikini) incision. METHODS: This retrospective study identified patients who underwent DAA primary total hip arthroplasty with a subsequent DAA revision within 90 days. Patients were divided into two cohorts based on the orientation of their incision: either 'longitudinal incision' (in accordance with the Smith Peterson interval) or 'horizontal bikini incision' (in accordance with the hip flexion crease). RESULTS: There were 74 patients who underwent DAA revision arthroplasty within 90 days of primary arthroplasty; 65 had a longitudinal incision, and 9 had a horizontal (bikini) incision. In the longitudinal incision group, 2 patients (3.1%) required plastic surgery closure, and 11 patients (16.9%) required additional operations. Of the 9 bikini incision patients, 6 patients required the assistance of plastic surgery closure, and 7 patients required multiple orthopaedic operations. CONCLUSIONS: Our study suggests that a horizontal bikini incision is less forgiving in the early postoperative period if a more extensile exposure is needed for revision surgery, as measured by the need for plastic surgery and additional returns to the operating room. In our cohort, the longitudinal incision allowed for the management of early surgical complications with less morbidity.

Synchronous Low-Grade Central Osteosarcoma and Ewing Sarcoma: A Rare Case Report.

A 23-year-old female patient presented with radicular back pain, perineal numbness, and urinary retention. The patient was diagnosed with cauda equina syndrome and magnetic resonance imaging (MRI) of the spine revealed an enhancing osseous lumbar lesion causing severe central stenosis. A core needle biopsy of the lumbar spine showed microscopic features compatible with a small round blue cell tumor. CD99 and FLI1 were positive in the tumor cells. Next-generation sequencing demonstrated a EWSR1::FLI1 fusion. Given these findings, the spine lesion was diagnosed as Ewing sarcoma. The patient underwent surgical decompression of L2. On further workup, an MRI revealed an ill-defined enhancing mass of the right distal femur. This area was biopsied, demonstrating a fibro-osseous lesion with osteoblast proliferation containing nuclear atypia, low mitotic activity, and SATB2 positivity, diagnosed as low-grade central osteosarcoma (LGCOS). The patient underwent resection, which showed a classic LGCOS by histomorphology. Although fluorescence in-situ hybridization study for MDM2 gene amplification was negative, the overall findings are most consistent with LGCOS. These neoplasms are considered to be synchronous due to the presentation of each entity within 6 months. Considering the aggregate yearly incidence of Ewing sarcoma (approximately 1 case per 750 000 per year) and LGCOS (approximately 1 case per 10 million per year), the aggregate yearly probability of developing both of these genetically unrelated tumors in a single individual is 1 per 7.5 trillion per year, and it is likely such an event has never happened in the past.

Methylene Blue Infiltration As a Method for Distinguishing Superficial and Deep Infections.

BACKGROUND: There are no established objective methods to reliably differentiate between superficial and deep infection in the setting of total hip arthroplasty. We employed a technique of distinguishing superficial and deep infections by infiltrating methylene blue to a prosthetic hip during infection workup to determine its effectiveness and to characterize its utility in defining the joint cavity where deep debridement is required. METHODS: An analysis was conducted on 35 patients who preoperatively received an injection of methylene blue under radiological guidance to their total hip arthroplasty. Where established periprosthetic joint infection (PJI) criteria were not met, without signs of methylene blue beyond the deep fascia, the infection was considered superficial, and debridement remained superficial to the deep fascia. Where diagnosis of PJI was confirmed preoperatively or the presence of methylene blue in the wound cavity confirmed deep contamination, the prosthesis was addressed with methylene blue staining defining the joint cavity as a guide for debridement. RESULTS: There were 11 patients who had no methylene blue extrusion into the superficial compartment and did not meet PJI criteria. Eight patients failed to meet PJI criteria preoperatively, but had extrusion of methylene blue, confirming a deep infection intraoperatively. There were 16 patients who met PJI criteria preoperatively with methylene blue acting as a visual guide to the joint space. CONCLUSION: Infiltrating methylene blue in a prosthetic hip is useful in differentiating between superficial or deep infections where PJI workup is indeterminate. Where deep infection is present, its utility in defining the joint cavity may be advantageous.

Survivorship, complications, and outcomes following distal femoral arthroplasty for non-neoplastic indications.

AIMS: Endoprosthetic reconstruction with a distal femoral arthroplasty (DFA) can be used to treat distal femoral bone loss from oncological and non-oncological causes. This study reports the short-term implant survivorship, complications, and risk factors for patients who underwent DFA for non-neoplastic indications. METHODS: We performed a retrospective review of 75 patients from a single institution who underwent DFA for non-neoplastic indications, including aseptic loosening or mechanical failure of a previous prosthesis (n = 25), periprosthetic joint infection (PJI) (n = 23), and native or periprosthetic distal femur fracture or nonunion (n = 27). Patients with less than 24 months' follow-up were excluded. We collected patient demographic data, complications, and reoperations. Reoperation for implant failure was used to calculate implant survivorship. RESULTS: Overall one- and five-year implant survivorship was 87% and 76%, respectively. By indication for DFA, mechanical failure had one- and five-year implant survivorship of 92% and 68%, PJI of 91% and 72%, and distal femur fracture/nonunion of 78% and 70% (p = 0.618). A total of 37 patients (49%) experienced complications and 27 patients (36%) required one or more reoperation. PJI (n = 16, 21%), aseptic loosening (n = 9, 12%), and wound complications (n = 8, 11%) were the most common complications. Component revision (n = 10, 13.3%) and single-stage exchange for PJI (n = 9, 12.0 %) were the most common reoperations. Only younger age was significantly associated with increased complications (mean 67 years (SD 9.1)) with complication vs 71 years (SD 9.9) without complication; p = 0.048). CONCLUSION: DFA is a viable option for distal femoral bone loss from a range of non-oncological causes, demonstrating acceptable short-term survivorship but with high overall complication rates. Cite this article: Bone Jt Open 2022;3(3):173-181.

Worse Outcomes Are Associated With Proximal Femoral Replacement Following Periprosthetic Joint Infection.

BACKGROUND: Proximal femoral replacements (PFRs) are often used in the setting of severe bone loss. As osteolysis has become less common, PFR may be used to address other causes of bone loss such as infection or periprosthetic fracture. The aim of this study is to investigate the clinical outcomes of PFR for non-neoplastic conditions. METHODS: A retrospective review of 46 patients undergoing PFR at a single institution was performed. The electronic records were reviewed to extract relevant information including the reason for use of PFR, surgical variables, follow-up, and complications. Survivorship curves were generated and differences in survivorship were evaluated using the log-rank test. Radiographic evaluation was also performed. RESULTS: Using revision as an endpoint, the Kaplan-Meier analysis of the entire cohort demonstrated a survival rate of 74% at 1 year and 67% at 5 years. Patients with a preoperative diagnosis of periprosthetic joint infection demonstrated the lowest survivorship with a failure rate of 47%. Furthermore, a high dislocation rate at 17.4% (n = 8) was observed. The use of dual-mobility articulation was effective in reducing dislocation. CONCLUSION: PFR is a valuable reconstructive option for patients with massive proximal femoral bone loss. This study demonstrates that patients with periprosthetic joint infection who undergo PFR reconstruction are at very high risk of subsequent failure, most commonly from reinfection and instability. The use of a dual-mobility articulation in association with PFR appears to help mitigate risk of subsequent dislocation.

Distal Femoral Replacement and Periprosthetic Joint Infection After Non-Oncological Reconstruction: A Retrospective Analysis.

BACKGROUND: Distal femoral replacement (DFR) is commonly used to manage massive bone loss around the knee arising from aseptic loosening, periprosthetic joint infection (PJI), and distal femoral fractures. A number of studies report the outcome of DFR with considerable variation in long-term survivorship. This study investigated the outcome of DFR for patients with aseptic failures, fractures, and PJI. METHODS: A retrospective review of 182 patients who underwent DFR for non-oncological indications between 2002 and 2018 was conducted. Data collected included the following: indication, postoperative complications, reoperation, revision, and follow-up. Implant survivorship with Kaplan-Meier curves along with a log-rank test for different preoperative indications was performed. A Cox regression model was used to evaluate the risk of revision. RESULTS: The overall postoperative complication rate was very high at 36%. The most common complication was PJI (17%). The rate of reoperation for any cause was 29.7%, and the revision rate was 13.7%. The most common cause of re-revision was PJI (7.1%). Revision-free survivorship of the DFR implant was 91.6% at 1 year, 87.9% at 2 years, 82.5% at 5 years, and 73.4% at 10 years. Patients who had a prior-PJI had the lowest survivorship compared to patients undergoing DFR for management of periprosthetic fracture and mechanical loosening. Additionally, the prior-PJI group was at a fourfold increased risk of postoperative PJI compared to the aseptic group. CONCLUSION: DFR is a valuable reconstructive option for patients with massive bone loss around the knee. However, patients undergoing DFR are at high risk of complications, reoperations, and failure.

Mepivacaine versus Bupivacaine Spinal Anesthesia for Early Postoperative Ambulation.

BACKGROUND: Early ambulation after total hip arthroplasty predicts early discharge. Spinal anesthesia is preferred by many practices but can delay ambulation, especially with bupivacaine. Mepivacaine, an intermediate-acting local anesthetic, could enable earlier ambulation than bupivacaine. This study was designed to test the hypothesis that patients who received mepivacaine would ambulate earlier than those who received hyperbaric or isobaric bupivacaine for primary total hip arthroplasty. METHODS: This randomized controlled trial included American Society of Anesthesiologists Physical Status I to III patients undergoing primary total hip arthroplasty. The patients were randomized 1:1:1 to 52.5 mg of mepivacaine, 11.25 mg of hyperbaric bupivacaine, or 12.5 mg of isobaric bupivacaine for spinal anesthesia. The primary outcome was ambulation between 3 and 3.5 h. Secondary outcomes included return of motor and sensory function, postoperative pain, opioid consumption, transient neurologic symptoms, urinary retention, intraoperative hypotension, intraoperative muscle tension, same-day discharge, length of stay, and 30-day readmissions. RESULTS: Of 154 patients, 50 received mepivacaine, 53 received hyperbaric bupivacaine, and 51 received isobaric bupivacaine. Patient characteristics were similar among groups. For ambulation at 3 to 3.5 h, 35 of 50 (70.0%) of patients met this endpoint in the mepivacaine group, followed by 20 of 53 (37.7%) in the hyperbaric bupivacaine group, and 9 of 51 (17.6%) in the isobaric bupivacaine group (P < 0.001). Return of motor function occurred earlier with mepivacaine. Pain and opioid consumption were higher for mepivacaine patients in the early postoperative period only. For ambulatory status, 23 of 50 (46.0%) of mepivacaine, 13 of 53 (24.5%) of hyperbaric bupivacaine, and 11 of 51 (21.5%) of isobaric bupivacaine patients had same-day discharge (P = 0.014). Length of stay was shortest in mepivacaine patients. There were no differences in transient neurologic symptoms, urinary retention, hypotension, muscle tension, or dizziness. CONCLUSIONS: Mepivacaine patients ambulated earlier and were more likely to be discharged the same day than both hyperbaric bupivacaine and isobaric bupivacaine patients. Mepivacaine could be beneficial for outpatient total hip arthroplasty candidates if spinal is the preferred anesthesia type.

Efficacy of image-guided synovial biopsy.

OBJECTIVE: A variety of benign and neoplastic lesions can affect the synovium, including pigmented villonodular synovitis (PVNS) and synovial chondromatosis. Prior to surgical resection, accurate characterization of synovial lesions is necessary for appropriate treatment planning. Additionally, recent advances in potential medical therapies for PVNS could decrease or eliminate the need for surgery in some cases. Such treatment options demand accurate characterization of synovial lesions prior to treatment. METHODS AND MATERIALS: Institutional IRB approval was obtained. We identified 54 synovial biopsies performed at our institution using a comprehensive database search under ultrasound (US) or computed tomography (CT) guidance. Cases were reviewed for pre-procedure imaging, location, biopsy approach, biopsy results, post-procedure complications, and surgical pathology if synovectomy was performed. RESULT: A total of 54 image-guided synovial biopsies were performed, 36 using CT guidance and 18 using US guidance. Six different anatomic locations were biopsied (the hip, knee, shoulder, elbow, ankle, and temporomandibular joint). Synovial tissue was obtained in 89% of cases (48/54). CT-guided biopsies had a positive yield of 86% (31/36) and US-guided biopsies had a positive yield of 94% (17/18). Surgical pathology was obtained in 30 of the cases and image-guided biopsy concordance was 90% (27/30). Of the patients taken for synovectomy, biopsy concordance of suspected neoplastic lesions was 100% (23/23). In cases of suspected neoplasm, the concordance between image-guided biopsy and surgical pathology was 96% (22/23). There were no reported complications. CONCLUSION: Image-guided biopsy of synovial lesions is safe and effective for establishing a definitive diagnosis prior to surgical or other intervention.

Cutaneous Basal Cell Carcinoma with Bone Metastases: An Orthopaedic Case Report.

Basal cell carcinoma (BCC) is the most common skin cancer, and its incidence is increasing. Though metastatic BCC (mBCC) is uncommon, the literature demonstrates a 0.0028%-0.55% rate of metastasis. We report on a patient treated at our institution who was found to have mBCC with osseous metastases. To our knowledge, this is the first report of mBCC in the orthopaedic literature. Orthopaedic oncologists should consider mBCC in patients diagnosed with carcinoma of unknown origin, with a known history of BCC, or individuals with light skin pigmentation and age 50 or greater. This can help clinicians make the correct diagnosis and provide the appropriate treatment.

2018 International Consensus Meeting on Musculoskeletal Infection: Research Priorities from the General Assembly Questions.

Musculoskeletal infections (MSKI) remain the bane of orthopedic surgery, and result in grievous illness and inordinate costs that threaten healthcare systems. As prevention, diagnosis, and treatment has remained largely unchanged over the last 50 years, a 2nd International Consensus Meeting on Musculoskeletal Infection (ICM 2018, https://icmphilly.com) was completed. Questions pertaining to all areas of MSKI were extensively researched to prepare recommendations, which were discussed and voted on by the delegates using the Delphi methodology. The questions, including the General Assembly (GA) results, have been published (GA questions). However, as critical outcomes include: (i) incidence and cost data that substantiate the problems, and (ii) establishment of research priorities; an ICM 2018 research workgroup (RW) was assembled to accomplish these tasks. Here, we present the result of the RW consensus on the current and projected incidence of infection, and the costs per patient, for all orthopedic subspecialties, which range from 0.1% to 30%, and $17,000 to $150,000. The RW also identified the most important research questions. The Delphi methodology was utilized to initially derive four objective criteria to define a subset of the 164 GA questions that are high priority for future research. Thirty-eight questions (23% of all GA questions) achieved the requisite > 70% agreement vote, and are highlighted in this Consensus article within six thematic categories: acute versus chronic infection, host immunity, antibiotics, diagnosis, research caveats, and modifiable factors. Finally, the RW emphasizes that without appropriate funding to address these high priority research questions, a 3rd ICM on MSKI to address similar issues at greater cost is inevitable.

Risk Adjustment Is Necessary in Value-Based Payment Models for Arthroplasty for Oncology Patients.

BACKGROUND: Value-based payment models such as bundled payments have been introduced to reduce costs following total hip arthroplasty (THA). Concerns exist, however, about access to care for patients who utilize more resources. The purpose of this study is thus to compare resource utilization and outcomes of patients undergoing THA for malignancy with those undergoing THA for fracture or osteoarthritis. METHODS: We queried the American College of Surgeons National Surgical Quality Improvement Program database to identify all hip arthroplasties performed from 2013 to 2016 for a primary diagnosis of malignancy (n = 296), osteoarthritis (n = 96,480), and fracture (n = 13,406). The rates of readmissions, reoperations, comorbidities, mortality, and surgical characteristics were compared between the 3 cohorts. To control for confounding variables, a multivariate analysis was performed to identify independent risk factors for resource utilization and outcomes following THA. RESULTS: Patients undergoing THA for malignancy had a longer mean operative time (155.7 vs 82.9 vs 91.0 minutes, P < .001), longer length of stay (9.0 vs 7.2 vs 2.6 days, P < .001), and were more likely to be discharged to a rehabilitation facility (42.1% vs 61.8% vs 20.2%, P < .001) than patients with fracture or osteoarthritis. When controlling for demographics and comorbidities, patients undergoing THA for malignancy had a higher rate of readmission (adjusted odds ratio 3.39, P < .001) and reoperation (adjusted odds ratio 3.71, P < .001). CONCLUSION: Patients undergoing THA for malignancy utilize more resources in an episode-of-care and have worse outcomes. Risk adjustment is necessary for oncology patients in order to prevent access to care problems for these high-risk patients.