Subtrochanteric nonunion after intramedullary nailing due to pathological fracture: proximal femoral replacement as a salvage approach.

PURPOSE: To assess proximal femoral replacement as a treatment solution for nonunion of pathologic subtrochanteric fractures after cephalomedullary nailing in patients with pathological fracture and previously irradiated bone. METHODS: Retrospective review of five patients with pathological subtrochanteric femoral fractures that were treated with cephalomedullary nailing and developed a nonunion, which was revised with conversion to a proximal endoprosthetic replacement. RESULTS: All five patients had previously been treated with radiation. One patient had the latest follow-up at 2 months postoperatively. At that time, the patient was walking with a walker for assistance, with no evidence of hardware failure or loosening on imaging. The remaining four patients had the latest follow-up ranging from 9 to 20 months after surgery. At their latest follow-up, three of the four patients were ambulatory with no pain, using only a cane for assistance with longer distances. The other patient demonstrated pain in his affected thigh, utilizing a walker for assistance with ambulation at latest follow-up, but not requiring further surgical interventions. There were no hardware failures or implant loosening through the follow-up period. None of the patients required revision, and there were no postoperative complications observed at their last follow-up. CONCLUSIONS: In patients with pathological fractures in the subtrochanteric region that is treated with cephalomedullary nailing and developed a nonunion, treatment with conversion to a proximal femoral replacement with a mega prosthesis is a valuable treatment with good functional results and low risk for complications. LEVEL OF EVIDENCE: Therapeutic level IV.

Erratum to "COVID-19 vaccine-associated granulomatous mass mimicking a sarcoma: A case report" [Radiology Case Reports 17 (2022) 2775-2778].

[This corrects the article DOI: 10.1016/j.radcr.2022.05.035.].

COVID-19 vaccine-associated ganulomatous mass mimicking a sarcoma: a case report.

Several studies have previously documented the development of complications stemming from injection with one of the various COVID-19 vaccines. No study, however, has discussed the spontaneous development of a soft tissue mass shortly after a COVID-19 vaccine injection. We report on 66-year-old female with concerns of a growing shoulder mass, 2 weeks after receiving a COVID-19 vaccine booster. Initial work-up with X-ray and MRI was concerning for a soft tissue neoplasm, specifically a soft tissue sarcoma. Subsequent ultrasound guided biopsy demonstrated a benign granulomatous lesion. No further management was required as the lesion spontaneously resolved during a 3-month follow-up period.

Oncological Outcomes in Patients with Appendicular Myxofibrosarcomas: A Retrospective Study.

BACKGROUND: Myxofibrosarcoma (MFS) is notorious for its infiltrative growth pattern, making wide excisions difficult to achieve. Our objective was to assess the impact of surgical margins and other factors that affected rates of local recurrence (LR), distant metastasis (DM), and overall survival (OS) of individuals undergoing resection for MFS. METHODS: We retrospectively reviewed the medical records of 209 patients with appendicular soft tissue sarcomas between January 2012 and June 2018. Of these, 29 patients (14%) were diagnosed with myxofibrosarcoma. These patients underwent a total of 33 resections. The pathological analyses were conducted by an experienced musculoskeletal (MSK) pathologist. Demographics data, operative details, adjuvant therapy, and oncological outcomes were assessed. RESULTS: Of the 29 patients (33 resections), the overall LR rate was 24% (7/29) and the 2-year LR rate was 17% (5/29). Factors associated with negative oncological outcomes were as follows: tumor size ≤10 cm (2-year local recurrence-free rates (LRFRs), 65%; 95% CI, 44-86%; p=0.02) and positive surgical margins grouped with surgical margins ≤0.1 cm (hazard ratio (HR), 11.74; 95% CI, 1.41-97.74; p=0.02). Chemotherapy and radiotherapy together increased the 2-year LRFR (LRFR, 100%; 95% CI, 100%, p=0.001). Two-year DM and OS rates were 15% and 79%, respectively. Female gender was a predictor of distant metastasis. Local recurrence had a negative impact on overall survival. Intraoperative analysis of resection margin accuracy was 75% (12/16) when non-MSK pathologists were involved but 100% accurate (12/12) when analyzed by an MSK pathologist. CONCLUSION: Myxofibrosarcomas showed high LR rates after treatment. Close margins (≤0.1 cm) should be considered as a risk factor for LR, and LR is associated with negative overall survival. Neoadjuvant therapy in terms of combined chemotherapy and radiation therapy associates with decreased LR rates. If intraoperative assessment of margins is to be done, it should be performed by an experienced MSK pathologist.

Amyloid Arthropathy: A Review.

Amyloid arthropathy is a joint disease associated with systemic amyloidosis. Herein, we present a model case and review the clinicopathologic features and pathophysiology of this disorder. Amyloid arthropathy results from elevation of serum amyloidogenic proteins and their deposition as aggregates in synovial fluid and articular tissues. The most common proteins are beta-2-microglobulin in the context of long-term hemodialysis therapy and immunoglobulin light chains associated with plasma cell proliferations. We provide a comprehensive update on the pathogenesis, clinical manifestations, and pathologic features of amyloid arthropathy. We provide detailed insights on amyloid protein deposition and aggregation in joints and proper details for diagnosis.

Unplanned excision of soft tissue sarcoma: does it impact the accuracy of intra-operative pathologic assessment at time of re-excision?

PURPOSE: An "unplanned excision" refers to soft tissue sarcomas excised without planning imaging studies and a diagnostic biopsy, resulting in the presence of residual disease and usually necessitating a re-excision procedure. We aimed to assess the impact of previous unplanned excisions on the intra-operative pathologic assessment at the time of re-excision, in terms of need to perform repeat assessments and the accuracy to predict margin status of the final pathologic specimen. METHODS: Data was collected for all patients with extremity soft tissue sarcoma who had undergone wide local excision limb salvage surgery or amputation between 2012 and 2017. Intra-operative pathologic assessment with frozen sections was performed in all cases and was classified as negative, negative but close (< 1 mm), and positive. RESULTS: A total of 173 patients with extremity soft tissue sarcoma were included, 54 in the unplanned excision group and 119 in the planned excision group. The accuracy of intra-operative pathologic assessment to predict the margin status on final pathology was similar between groups (87% unplanned vs. 90.7% planned excisions). However, the need for repeat intra-operative pathologic assessment and subsequent resection due to microscopically positive margins was found to be higher within the unplanned excision group ((p = 0.04), OR = 3.2 (95% CI: 1.1-9.1, p = 0.048)). CONCLUSIONS: Intra-operative pathologic assessment of resection margins had a similar accuracy in planned and unplanned excisions; however, unplanned excisions showed a higher risk of re-resection during the same surgical setting.

Cancer stem cells as a therapeutic target in 3D tumor models of human chondrosarcoma: An encouraging future for proline rich polypeptide­1.

Chondrosarcoma is a malignant bone neoplasm that is refractory to chemotherapy and radiation. With no current biological treatments, mutilating surgical resection is the only effective treatment. Proline rich polypeptide 1 (PRP­1), which is a 15­amino acid inhibitor of mammalian target of rapamycin complex­1 (mTORC1), has been indicated to exert cytostatic and immunomodulatory properties in human chondrosarcoma cells in a monolayer. The aim of the present study was to evaluate the effects of PRP­1 on an in vitro 3D chondrosarcoma tumor model, known as spheroids, and on the cancer stem cells (CSCs) which form spheroids. JJ012 cells were cultured and treated with PRP­1. An ALDEFLUOR™ assay was conducted (with N,N­diethylaminobenzaldehyde as the negative control) to assess aldehyde dehydrogenase (ALDH) activity (a recognized CSC marker), and bulk JJ012, ALDHhigh and PRP­1 treated ALDHlow cells were sorted using flow cytometry. Colony formation and spheroid formation assays of cell fractions, including CSCs, were used to compare the PRP­1­treated groups with the control. CSCs were assessed for early apoptosis and cell death with a modified Annexin V/propidium iodide assay. Western blotting was used to identify mesenchymal stem cell markers (STRO1, CD44 and STAT3), and spheroid self­renewal assays were also conducted. A clonogenic dose­response assay demonstrated that 20 µg/ml PRP­1 was the most effective dose for reducing colony formation capacity. Furthermore, CSC spheroid growth was significantly reduced with increasing doses of PRP­1. Annexin V analysis demonstrated that PRP­1 induced CSC cell death, and that this was not attributed to apoptosis or necrosis. Western blot analysis confirmed the expression of mesenchymal markers, and the spheroid self­renewal assay confirmed the presence of self­renewing CSCs. The results of the present study demonstrate that PRP­1 eliminates anchorage independent CSC growth and spheroid formation, indicating that PRP­1 likely inhibits tumor formation in a murine model. Additionally, a decrease in non­CSC bulk tumor cells indicates an advantageous decline in tumor stromal cells. These findings confirm that PRP­1 inhibits CSC proliferation in a 3D tumor model which mimics the behavior of chondrosarcoma in vivo.

Soft Tissue Masses in Patients Who Underwent Cosmetic Fat Transfer Procedures for Gluteal Augmentation: A Report of 3 Cases.

CASES: Three patients were referred to our musculoskeletal oncology service after undergoing autologous fat grafting procedures. Two masses were suspected to be "soft tissue sarcomas," and one was due to a mass of unknown origin. These findings have not been reported in the orthopedic literature and may generate potential referrals for orthopedic oncologists. CONCLUSIONS: Awareness of potential complications of procedures from other surgical specialties and their radiographic characteristics is of utmost importance. The clinical and radiographic findings that could assist in distinguishing a mass related to an autologous fat transfer procedure from a soft tissue sarcoma are described.

Operative Reports in Orthopaedic Surgery: The Need for Assessment and Education.

BACKGROUND: The ability to complete an operative report is a vital skill for an orthopaedic surgeon. We hypothesized that most programs do not have formal operative report teaching, that resident operative reports at our institution are incomplete, and that a formal teaching program would improve operative reports. METHODS: A survey of residencies in the United States was conducted assessing the state of operative report education. In addition, resident operative reports were collected at our institution both pre and post a formal educational session. Scores were given for each report out of a possible 35 points. RESULTS: Total 54 institutions responded to the survey, of which 83% indicated that they had no formal resident operative report teaching. Within our institution, 100 resident operative dictations were assessed prior to instituting a formal education session, with a mean score of 24.5. The most commonly missed items in the report were preoperative antibiotics, deep venous thrombosis prophylaxis, and tourniquet time. The mean score of 100 resident operative dictations following the educational session improved to 31.8. CONCLUSION: Most residency programs do not conduct formal resident operative report teaching. Formal instruction on how to complete a comprehensive operative report resulted in a significant improvement in their quality.

Surgical treatment of low-grade chondrosarcoma involving the appendicular skeleton: long-term functional and oncological outcomes.

BACKGROUND: The traditional treatment for chondrosarcoma is wide local excision (WLE), as these tumors are resistant to chemotherapy and radiation treatment. While achieving negative margins has traditionally been the goal of chondrosarcoma resection, multiple studies have demonstrated good short-term results after intralesional procedures for low-grade chondrosarcomas (LGCS) with curettage and adjuvant treatments (phenol application, cauterization or cryotherapy) followed by either cementation or bone grafting. Due to the rarity of this diagnosis and the recent application of this surgical treatment modality to chondrosarcoma, most of the information regarding treatment outcomes is retrospective, with short or intermediate-term follow-up. The aim of this study was to assess the long-term results of patients with LGCS of bone treated with intralesional curettage (IC) treatment versus WLE. This retrospective analysis aims to characterize the oncologic outcomes (local recurrence, metastases) and functional outcomes in these two treatment groups at a single institution. METHODS: Using an institutional musculoskeletal oncologic database, we retrospectively reviewed medical records of all patients with LGCS of the appendicular skeleton that underwent surgical treatment between 1985 and 2007. Thirty-two patients (33 tumors) were identified with LGCS; 17 treated with IC and 15 with WLE. RESULTS: Seventeen patients (18 tumors) with a minimum clinical and radiologic follow-up of 10 years were included. Nine patients were treated with IC (four with no adjuvant, three with additional phenol, one with liquid nitrogen and one with H2O2) with either bone graft or cement augmentation, and nine others were treated with WLE and reconstruction with intercalary/osteoarticular allograft or megaprosthesis. The mean age at surgery was 41 years (range 14-66 years) with no difference (p = 0.51) between treatment cohorts. There was a mean follow-up of 13.5 years in the intralesional cohort (range 10-19 years) and 15.9 years in the WLE cohort (range 10-28 years, p = 0.36). Tumor size varied significantly between groups and was larger in patients treated with WLE (8.2 ± 3.1 cm versus 5.4 ± 1.2 cm, at the greatest dimension, p = 0.021). There were two local recurrences (LR), one in the intralesional group and one in the wide local excision group, occurring at 3.5 months and 2.9 years, respectively, and both required revision. No further LR could be detected with long-term follow-up. The MSTS score at final follow-up was significantly higher for patients managed with intralesional procedures (28.7 ± 1.7 versus 25.7 ± 3.4, p = 0.033). There were less complications requiring reoperation in the intralesional group compared with the wide local excision group, although this difference was not found to be statistically significant (one versus four patients, respectively; p = 0.3). CONCLUSION: This series of low-grade chondrosarcoma, surgically treated with an intralesional procedures, with 10-year follow-up, demonstrates excellent local control (88.9%). Complications were infrequent and minor and MSTS functional scores were excellent. Wide resection of LGCS was associated with lower MSTS score and more complications. In our series, the LR in both groups were detected within the first 3.5 years following the index procedure, and none were detected in the late surveillance period.

Benign Bone Tumors When They Coexist with Common Orthopaedic Conditions.

Benign and malignant bone tumors around the knee can be encountered with concomitant orthopaedic disease. As such, it is important to recognize these oncologic processes that may impact or alter the routine treatment of more common orthopaedic processes. Frequently, these rare oncologic conditions are discovered only through the evaluation of acute injuries as in the setting of fractures or sports injuries. Other times, they are encountered when evaluating chronic entities, such as arthroplasty. This article aims to provide general guidelines and management strategies for benign bone tumors when they coincide with common orthopaedic conditions around the knee.

Evaluating Treatment Strategies for Spinal Lesions in Multiple Myeloma: A Review of the Literature.

BACKGROUND: Vertebral disease is a major cause of morbidity in 70% of patients diagnosed with multiple myeloma (MM). Associated osteolytic lesions and vertebral fractures are well documented in causing debilitating pain, functional restrictions, spinal deformity, and cord compression. Currently, treatment modalities for refractory MM spinal pain include systemic therapy, radiotherapy, cementoplasty (vertebroplasty/kyphoplasty), and radio frequency ablation. Our objectives were to report on the efficacy of existing treatments for MM patients with refractory spinal pain, to determine if a standardized treatment algorithm has been described, and to set the foundation upon which future prospective studies can be designed. METHODS: A systematic search of the PubMed database was performed for studies relevant to the treatment of vertebral disease in MM patients. A multitude of search terms in various combinations were used, including but not limited to: "vertebroplasty," "kyphoplasty," "radiation," "multiple myeloma," "radiotherapy," and "radiosurgery." RESULTS: Our preliminary search resulted in 219 articles, which subsequently resulted in 19 papers following abstract, title, full-text, and bibliography review. These papers were then grouped by treatment modality: radiotherapy, cementoplasty, or combination therapy. Significant pain and functional score improvement across all treatment modalities was found in the majority of the literature. While complications of treatment occurred, few were noted to be clinically significant. CONCLUSIONS: Treatment options-radiotherapy and/or cementoplasty-for vertebral lesions and pathologic fractures in MM patients demonstrate significant radiographic and clinical improvement. However, there is no consensus in the literature as to the optimal treatment modality as a result of a limited number of studies reporting head-to-head comparisons. One study did find significantly improved pain and functional scores with preserved vertebral height in favor of kyphoplasty over radiotherapy. When not contraindicated, we advocate for some form of cementoplasty. Further prospective studies are required before implementation of a standardized treatment protocol. LEVEL OF EVIDENCE: 5.

Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Medial Cuneiform: A Case Report and Review of the Literature.

Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton. To the best of our knowledge, we report the first case of primary intraosseous MPNST involving a midtarsal bone (medial cuneiform). The patient was a 62-year-old female who presented with pain and tenderness but without swelling. Imaging revealed nonspecific findings, and the preoperative computed tomography-guided biopsy findings were consistent with MPNST. The patient was treated with neoadjuvant radiotherapy, followed by wide local excision and allograft reconstruction. At the final follow-up examination (24 months), the graft had been incorporated without evidence of local recurrence or distant disease. The patient with primary intraosseous MPNST of the medial cuneiform described in the present report presented with nonspecific clinical and radiologic findings. Thus, a high index of suspicion and histopathologic examination, including immunohistochemistry, are necessary for an accurate diagnosis.

Unplanned excision of soft-tissue sarcomas: current concepts for management and prognosis.

➤ Biopsy, staging, preoperative imaging and planning, as well as surgical treatment of soft-tissue sarcomas, are best carried out in specialized sarcoma centers, with the support of a multidisciplinary tumor board.➤ Tumor bed excision is recommended after most unplanned excisions, with a goal of obtaining complete tumor removal with an appropriately wide margin of resection.➤ The surgical resection area tends to be more extensive during tumor bed excision than during primary resection because of the need to resect potential areas of contamination, resulting in the need for more reconstructive procedures (flaps and skin grafts) and wider radiation fields.➤ Unplanned excisions are associated with an increased rate of local recurrence related to residual disease and positive margins after tumor bed excision, a deep location, and certain histologic subtypes, such as malignant peripheral nerve sheath tumor, myxofibrosarcoma, and dermatofibrosarcoma protuberans.➤ While adjuvant radiation therapy has not been found to mitigate the risk of local recurrence in unplanned excisions, it is generally utilized in the treatment of unplanned excisions as it is in the treatment of primary soft-tissue sarcomas.➤ Given the surgical and oncologic sequelae of unplanned excisions, prevention through the diffusion of concepts by means of provider education on how to approach soft-tissue masses, which can be potential soft-tissue sarcomas, is the best strategy.

Locally aggressive fibrous dysplasia mimicking malignancy: a report of four cases and review of the literature.

BACKGROUND: Fibrous dysplasia is a benign fibroosseous bone tumor that accounts for 5% to 10% of benign bone tumors. It can present as monostotic fibrous dysplasia (70% to 80%), polyostotic fibrous dysplasia (20% to 30%), McCune-Albright syndrome (2% to 3%), or Mazabraud's syndrome in rare cases. Bone lesions in fibrous dysplasia arise in the medullary canal and usually are confined to the bone. Cortical destruction and extension into soft tissue usually indicates malignant transformation or secondary aneurysmal bone cyst formation. Locally aggressive fibrous dysplasia with cortical destruction and extension into soft tissue in the absence of these two possibilities is extremely rare. It is important for the treating physician to distinguish this entity from more aggressive or malignant tumors to avoid overtreating the patient for a benign condition or inattention to a malignant tumor. CASE DESCRIPTIONS: We report four unusual cases of fibrous dysplasia with an aggressive radiographic appearance. They occurred in the rib (1), ilium (2), and distal femur (1). Two patients had pain and two had swelling. Radiologically, all were associated with cortical destruction and an associated soft tissue mass, and initially they were interpreted as potentially malignant. Three patients underwent biopsy and one patient did not have a biopsy. Histopathologic analysis by an experienced bone pathologist confirmed fibrous dysplasia in all patients. Two patients were treated surgically; one patient with zoledronic acid and one patient currently is being followed by observation alone. LITERATURE REVIEW: There are only a few reports in the literature that describe the locally aggressive variant of fibrous dysplasia that presents with pain and progressive swelling clinically and with cortical destruction and soft tissue extension on imaging which suggest malignancy. We could not find any article that describes the use of bisphosphonates in such lesions or the response to bisphosphonates clinically, on laboratory parameters or imaging. To our knowledge, this is the largest case report published regarding locally aggressive fibrous dysplasia arising outside the craniofacial skeleton. CLINICAL RELEVANCE: The locally aggressive variant of fibrous dysplasia may be confused with a malignant tumor or malignant degeneration of fibrous dysplasia. It is important to properly evaluate these lesions to ensure that a proper diagnosis is made, especially with respect to a malignant versus benign mass.

Five polyostotic conditions that general orthopedic surgeons should recognize (or should not miss).

General orthopedic surgeons frequently encounter patients with conditions affecting multiple bones. It is important to recognize common polyostotic diseases. This article describes five polyostotic conditions: Multipe Enchondromatosis (Ollier Disease and Maffucci syndrome), Multiple Hereditary Exostosis (Diaphyseal Aclasis), Fibrous Dysplasia (McCune-Albright syndrome and Mazabraud syndrome), Paget's Disease of bone (Osteitis Deformans), and Skeletal Metastases. This is a survey of the clinical, pathologic and radiographic features that assist in diagnosing these conditions. Also, an overview of the laboratory findings, treatment, follow-up, and prognosis is presented. Recognizing these diseases will aid in prompt and accurate diagnosis and appropriate referral and therapy.

Early complications of high-dose-rate brachytherapy in soft tissue sarcoma: a comparison with traditional external-beam radiotherapy.

BACKGROUND: Radiotherapy and surgery are routinely utilized to treat extremity soft tissue sarcoma. Multiple radiation modalities have been described, each with advantages and disadvantages, without one modality demonstrating clear superiority over the others. QUESTIONS/PURPOSES: We determined the overall initial complication rate in patients receiving surgery and radiotherapy, which specific complications were found when comparing different modalities, and whether combination therapy increased the overall rate of complications compared with surgery and single-modality radiotherapy. PATIENTS AND METHODS: We retrospectively reviewed the records of 190 patients who received external-beam radiotherapy (141 patients), high-dose-rate brachytherapy (37 patients), or both (12 patients). We evaluated 100 men and 90 women (mean age, 57 years; range, 18-94 years) for tumor size and subtype, comorbidities, stage, grade, margin of resection, type of adjuvant treatment, and complications. Minimum followup was 3 months (mean, 40 months; range, 3-155 months). RESULTS: The most frequent early complications in the high-dose-rate brachytherapy cohort were infection, cellulitis, and seroma and/or hematoma. In the external-beam radiotherapy cohort, chronic edema, fibrosis, and chronic radiation dermatitis were more frequently encountered. The total number of early complications and overall incidence of major complications requiring further surgery were similar among the three cohorts, but a larger number of patients in the high-dose-rate brachytherapy group required subsequent surgery for infection compared with the external-beam radiotherapy group. CONCLUSIONS: High-dose-rate brachytherapy decreases radiation exposure and allows shorter duration of treatment compared with traditional external-beam radiotherapy but has a higher perioperative wound complication rate. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.