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Cody C Wyles,1 Matthew T Houdek,2 Atta Behfar,3 Rafael J Sierra,21Mayo Medical School, 2Department of Orthopedic Surgery, 3Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USAAbstract: Osteoarthritis (OA) is a painful chronic condition with a significant impact on quality of life. The societal burden imposed by OA is increasing in parallel with the aging population; however, no therapies have demonstrated efficacy in preventing the progression of this degenerative joint disease. Current mainstays of therapy include activity modification, conservative pain management strategies, weight loss, and if necessary, replacement of the affected joint. Mesenchymal stem cells (MSCs) are a multipotent endogenous population of progenitors capable of differentiation to musculoskeletal tissues. MSCs have a well-documented immunomodulatory role, managing the inflammatory response primarily through paracrine signaling. Given these properties, MSCs have been proposed as a potential regenerative cell therapy source for patients with OA. Research efforts are focused on determining the ideal source for derivation, as MSCs are native to several tissues. Furthermore, optimizing the mode of delivery remains a challenge both for appropriate localization of MSCs and for directed guidance toward stemming the local inflammatory process and initiating a regenerative response. Scaffolds and matrices with growth factor adjuvants may prove critical in this effort. The purpose of this review is to summarize the current state of MSC-based therapeutics for OA and discuss potential barriers that must be overcome for successful implementation of cell-based therapy as a routine treatment strategy in orthopedics.Keywords: mesenchymal stem cell, osteoarthritis, treatment, regenerative medicine, cell therapy

Matthew T Houdek,1 Cody C Wyles,2 John R Martin,1 Rafael J Sierra11Department of Orthopedic Surgery, 2School of Medicine, Mayo Clinic, Rochester, MN, USAAbstract: Avascular necrosis (AVN) of the femoral head is a progressive disease that predominantly affects younger patients. Although the exact pathophysiology of AVN has yet to be elucidated, the disease is characterized by a vascular insult to the blood supply of the femoral head, which can lead to collapse of the femoral head and subsequent degenerative changes. If AVN is diagnosed in the early stages of the disease, it may be possible to attempt surgical procedures which preserve the hip joint, including decompression of the femoral head augmented with concentrated bone marrow. The use of autologous stem cells has shown promise in halting the progression of AVN of the femoral head, and subsequently preventing young patients from undergoing total hip arthroplasty. The purpose of this study was to review the current use of stem cells for the treatment of AVN of the femoral head.Keywords: avascular necrosis, femoral head, osteonecrosis, stem cells, concentrated bone marrow

Reconstruction of the abductor mechanism remains a primary challenge with contemporary proximal femoral replacement (PFR) surgery. Previously, techniques such as trochanteric preservation or direct repair to the implant have been described; however, these strategies are limited in their ability to tension the repair and reattach other muscles of the hip girdle. The aim of this study was to evaluate the outcomes of patients undergoing oncologic PFR using a novel technique of mesh augmentation for soft tissue repair., Methods: We reviewed 18 (mean age 64 years; 8 female: 10 male) consecutive patients undergoing PFR with Marlex mesh augmentation between 2018 and 2023 at a single institution. The most common indication was metastatic disease (n = 13). The mean follow-up in the 14 surviving patients was 27 months (range 12-34)., Results: All patients were ambulatory at final follow-up. There were no post-operative dislocations, infections, or wound issues. At the final follow-up, the mean total MSTS score was 77%., Conclusion: Mesh augmentation of PFRs allowed for adequate soft tissue tensioning and muscular attachment to the body of the implant. In our series, this technique was durable, with no dislocations and no mesh-related complications. In summary, mesh augmentation of PFRs may be considered during reconstruction for oncologic indications.

Background: Myxoid liposarcoma predominantly affects young and middle-aged individuals, and little is known regarding treatment tolerability and outcomes in older patients. This study aims to better understand this older patient population., Methods: This single institution retrospective study included patients aged 70 years and older with localized (non-metastatic) myxoid liposarcoma., Results: Sixteen patients were included. The median age was 75 years, and 9 (56%) were female. Fourteen (88%) were extremity tumors and two (12%) were trunk. The median tumor size was 10.4 cm (range, 3.6 to 28 cm). Five (31%) tumors had a round cell component. All patients had surgery. Fourteen (88%) had perioperative radiation, and three (19%) had perioperative chemotherapy. One patient had postoperative infection, and one patient had neutropenic fever from preoperative chemotherapy. The median follow up from surgery was 6.3 years. Eight (50%) patients died from MLPS. The median relapse-free survival and overall survival were 34 months and 75 months, respectively., Conclusions: Most older patients with localized MLPS received perioperative radiation therapy with surgery, and few serious toxicities were reported. Even with treatment, half of the patients relapsed.

Background : Synovial sarcoma is rare and may present as a small, slow-growing mass. These tumors are often mistaken as benign and are therefore prone to unplanned and/or non-oncologic excision. We sought to identify the rate of unplanned excision of synovial sarcoma and risk factors for recurrence and survival among this cohort. Methods : The medical records of 246 patients evaluated at a single institution for synovial sarcoma between 1997 and 2022 were retrospectively reviewed. Of these, 87 (35%) underwent unplanned, non-oncologic excision. The mean age of the cohort was 49 years. Primary tumors were located in the extremity (n = 63), abdomen (n = 6), thorax (n = 7), head/neck (n = 8), and paraspinal region (n = 3). The median maximum pre-treatment dimension of the primary tumor was 4.8 cm (IQR 7-2.4). Seventy-seven (86%) patients underwent re-excision of the tumor bed, 39 (45%) received chemotherapy, and 63 (72%) received radiation therapy. Results : Among patients who underwent unplanned excision, local recurrence-free survival (LRFS) was 98% at 1 year and 82% at 5 years. Metastasis-free survival (MFS) was 91% at 1 year and 72% at 5 years. Disease-specific survival (DSS) was 98% at 1 year and 72% at 5 years. When adjusting for tumor size, tumors which underwent unplanned excision did not have worse recurrence or survival compared to those which had planned excision ( p > 0.10). Size > 5 cm, monophasic subtype, and axial location were associated with increased risk of disease recurrence. Forty-six patients had residual tumor following re-excision, which was associated with worse MFS (HR 8.17, 95% CI [1.89, 35.2], p < 0.01) and DSS (HR 7.66, 95% CI [1.76, 33.4], p < 0.01). Patients who received radiotherapy had improved MFS (HR 6.4, 95% CI [1.42, 29.0], p = 0.02) and DSS (HR 5.86, 95% CI [1.27, 26.9], p = 0.02). Conclusions : One-third of patients presenting with synovial sarcoma were diagnosed after unplanned, non-oncologic excision. Patients with large, axial tumors had worse survival. Approximately half of patients who underwent unplanned excision had no residual tumor after pre-operative radiation. The use of radiation was associated with decreased rates of recurrence and improved disease-specific survival. Our results suggest that margin-negative re-resection and radiotherapy should be considered when feasible following unplanned excision of synovial sarcoma.

Background: Liposarcoma, one of the most prevalent sarcoma histologies, is recognized for its tendency for extra-pulmonary metastases. While oligometastatic cardiac disease is rarely reported, it poses a unique challenge as oligometastatic sarcomas are often managed with surgical resection., Case Report: We present a case of a 62-year-old man diagnosed with an oligometastatic myxoid liposarcoma (MLPS) to the heart 19 years after the primary tumor resection from the lower limb. The metastatic mass, situated in the pericardium adjacent and infiltrating the left ventricle, was not managed surgically but with a combination of chemotherapy and radiotherapy. The patient's disease remains stable to date, for more than 10 years., Literature Review: We conducted a review of the literature to determine the preferred management approach for solitary cardiac metastases of sarcomas. We also conducted an in-depth analysis focusing on reported cases of MLPS metastasizing to the heart, aiming to extract pertinent data regarding the patient characteristics and the corresponding management strategies., Conclusions: Although clinical diagnoses of solitary or oligometastatic cardiac metastases from sarcomas are infrequent, this case underscores the significance of aggressive management employing chemotherapy and radiotherapy for chemosensitive and radiosensitive sarcomas, especially when surgical removal is high-risk. Furthermore, it challenges the notion that surgery is the exclusive therapeutic option leading to long-term clinical benefit in patients with recurrent sarcomas.

Aims: Venous tumour thrombus (VTT) is a rare finding in osteosarcoma. Despite the high rate of VTT in osteosarcoma of the pelvis, there are very few descriptions of VTT associated with extrapelvic primary osteosarcoma. We therefore sought to describe the prevalence and presenting features of VTT in osteosarcoma of both the pelvis and the limbs., Methods: Records from a single institution were retrospectively reviewed for 308 patients with osteosarcoma of the pelvis or limb treated between January 2000 and December 2022. Primary lesions were located in an upper limb (n = 40), lower limb (n = 198), or pelvis (n = 70). Preoperative imaging and operative reports were reviewed to identify patients with thrombi in proximity to their primary lesion. Imaging and histopathology were used to determine presence of tumour within the thrombus., Results: Tumours abutted the blood vessels in 131 patients (43%) and encased the vessels in 30 (10%). Any form of venous thrombus was identified in 31 patients (10%). Overall, 21 of these thrombi were determined to be involved with the tumour based on imaging (n = 9) or histopathology (n = 12). The rate of VTT was 25% for pelvic osteosarcoma and 1.7% for limb osteosarcoma. The most common imaging features associated with histopathologically proven VTT were enhancement with contrast (n = 12; 100%), venous enlargement (n = 10; 83%), vessel encasement (n = 8; 66%), and visible intraluminal osteoid matrix (n = 6; 50%). Disease-specific survival (DSS) for patients with VTT was 95% at 12 months (95% CI 0.87 to 1.00), 50% at three years (95% CI 0.31 to 0.80), and 31% at five years (95% CI 0.14 to 0.71). VTT was associated with worse DSS (hazard ratio 2.3 (95% CI 1.11 to 4.84)., Conclusion: VTT is rare with osteosarcoma and occurs more commonly in the pelvis than the limbs. Imaging features suggestive of VTT include enhancement with contrast, venous dilation, and vessel encasement. VTT portends a worse prognosis for patients with osteosarcoma, with a similar survivability to metastatic disease., Competing Interests: None declared., (© 2024 The British Editorial Society of Bone & Joint Surgery.)

Background: Endoprostheses (EPC) are often utilized for reconstruction of the proximal humerus with either hemiarthroplasty (HA) or reverse arthroplasty (RA) constructs. RA constructs have improved outcomes in patients with primary lesions, but no studies have compared techniques in metastatic disease. The aim of this study is to compare functional outcomes and complications between HA and RA constructs in patients undergoing endoprosthetic reconstruction for proximal humerus metastases., Methods: We retrospectively reviewed our institutional arthroplasty database to identify 66 (56% male; 38 HA and 28 RA) patients with a proximal humerus reconstruction for a non-primary malignancy. The majority (88%) presented with pathologic fracture, and the most common diagnosis was renal cell carcinoma (48%)., Resultss: Patients with RA reconstructions had better postoperative forward elevation (74° vs. 32°, p < 0.01) and higher functional outcome scores. HA patients had more complications (odds ratio 13, p < 0.01), with instability being the most common complication., Conclusions: Patients with nonprimary malignancies of the proximal humerus had improved functional outcomes and fewer complications after undergoing reconstruction with a reverse EPC compared to a HA EPC. Preference for reverse EPC should be given in patients with good prognosis and ability to complete postoperative rehabilitation., (© 2024 Wiley Periodicals LLC.)

Background: Radiation-associated soft tissue sarcomas (RA-STS) are rare complications of patients receiving radiation therapy (RT) and are generally associated with a poor prognosis. Most of the literature surrounding RA-STS of the chest is centered on angiosarcoma. Therefore, we aim to document the management and outcome of patients with non-angiosarcoma RA-STS of the chest., Methods: We reviewed 17 patients (all female, median age 65 years) diagnosed with RA-STS. The most common primary malignancy was breast carcinoma (n = 15), with a median RT dose of 57.9 Gy. All patients underwent surgical resection; five patients (29%) received radiotherapy; and five patients (29%) received peri-operative chemotherapy., Results: The 5-year local recurrence and metastatic-free survival were 61% and 60%, while the 5-year disease-specific survival was 53%. Local recurrence was associated with death due to disease (HR 9.06, p = 0.01). Complications occurred in nine of patients, most commonly due to a wound complication (n = 7). At the most recent follow-up, the median Musculoskeletal Tumor Society Score was 63%., Conclusion: RA-STS involving the chest wall are aggressive tumors with a high risk of local relapse and death due to disease. Local recurrence was associated with death due to disease; as such, we recommend aggressive surgical management with evaluation for adjuvant therapies., (© 2024 Wiley Periodicals LLC.)

Background: Shoulder arthroplasty in the setting of severe proximal humerus bone loss can be challenging. The purpose of this study was to evaluate the outcomes of a modular segmental megaprosthesis when implanted in a reverse configuration for complex primary arthroplasty, reconstruction at the time of oncologic resection, and revision shoulder arthroplasty., Materials and Methods: A Joint Registry Database was queried to identify all shoulder arthroplasties performed at a single institution using the Comprehensive Segmental Revision System reverse shoulder arthroplasty (SRS-RSA; Zimmer Biomet). A retrospective review of electronic medical records and radiographs was performed to record demographic data, indication, outcomes, complications, and revision surgery., Results: Between February 2012 and October 2022, a total of 76 consecutive SRS-RSAs were implanted. An analysis of patients with minimum 12-month follow-up yielded 53 patients with a mean follow-up of 4.1 ± 2.43 years. Surgical complication rate in this cohort was observed in 41.5% (22 of 53) of cases. Overall, the revision rate at final follow-up was 26.4% (14 of 53), with a significant difference between the primary and revision cohorts. The number of prior surgeries was a significant risk factor for revision surgery, with a hazard ratio of 1.789 (95% confidence interval 1.314-2.436, P < .001). When analyzing aseptic humeral loosening rates across study cohorts, a significant difference was found between the primary arthroplasty (0%, n = 0) and the revision arthroplasty cohorts (22.2%, n = 6) (P = .04)., Discussion: Reverse shoulder arthroplasty using a modular segmental megaprosthesis remains a reasonable salvage option for shoulder reconstruction in the setting of proximal humeral bone loss. Because of the substantial bone loss and soft tissue deficiencies typically present in these cases, surgeons should educate patients on the relatively high complication rate, particularly when used in the setting of a previous failed arthroplasty., (Copyright © 2024 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.)

Chondrosarcoma is the second most common surgically treated primary bone sarcoma. Despite a large number of scientific papers in the literature, there is still significant controversy about diagnostics, treatment of the primary tumour, subtypes, and complications. Therefore, consensus on its day-to-day treatment decisions is needed. In January 2024, the Birmingham Orthopaedic Oncology Meeting (BOOM) attempted to gain global consensus from 300 delegates from over 50 countries. The meeting focused on these critical areas and aimed to generate consensus statements based on evidence amalgamation and expert opinion from diverse geographical regions. In parallel, periprosthetic joint infection (PJI) in oncological reconstructions poses unique challenges due to factors such as adjuvant treatments, large exposures, and the complexity of surgery. The meeting debated two-stage revisions, antibiotic prophylaxis, managing acute PJI in patients undergoing chemotherapy, and defining the best strategies for wound management and allograft reconstruction. The objectives of the meeting extended beyond resolving immediate controversies. It sought to foster global collaboration among specialists attending the meeting, and to encourage future research projects to address unsolved dilemmas. By highlighting areas of disagreement and promoting collaborative research endeavours, this initiative aims to enhance treatment standards and potentially improve outcomes for patients globally. This paper sets out some of the controversies and questions that were debated in the meeting., Competing Interests: A. Puri is a member of the editorial board of The Bone & Joint Journal. P. Ruggieri reports consulting fees from Exactech and Stryker, unrelated to this study. M. T. Houdek reports consulting fees from Link Orthopedics, unrelated to this study. E. Botello reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Zimmer Biomet, all of which are unrelated to this study. G. V. Morris reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Implantcast, all of which are unrelated to this study., (© 2024 Jeys et al.)

» The proximal humerus is a common location for primary bone tumors, and the goal of surgical care is to obtain a negative margin resection and subsequent reconstruction of the proximal humerus to allow for shoulder function.» The current evidence supports the use of reverse total shoulder arthroplasty over hemiarthroplasty when reconstructing the proximal humerus after resection of a bone sarcoma if the axillary nerve can be preserved.» There is a lack of high-quality data comparing allograft prosthetic composite (APC) with endoprosthetic reconstruction of the proximal humerus.» Reverse APC should be performed using an allograft with donor rotator cuff to allow for soft-tissue repair of the donor and host rotator cuff, leading to improvements in shoulder motion compared with an endoprosthesis., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSREV/B74)., (Copyright © 2024 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: Myxofibrosarcoma (MFS) is a subtype of soft tissue sarcoma with a highly infiltrative growth pattern that leads to a higher risk of inadvertent positive surgical margins and local relapse. Poorly defined tumor margins also pose a challenge for radiation therapy (RT) planning, in terms of treatment volumes and administration of pre- versus postoperative RT. This study aims to evaluate local control and patterns of recurrence in patients with MFS treated with neoadjuvant RT followed by definitive surgical excision., Methods and Materials: Multiple institutional databases were retrospectively searched for patients diagnosed with MFS between 2013 and 2021 who were exclusively treated with preoperative RT followed by definitive surgery at our institution. The endpoints of the study were defined as local tumor recurrence, distant metastasis, and death after the date of definitive surgery., Results: Forty-nine patients met the inclusion criteria and were included in the final study. The median age at diagnosis was 67 years, and 71% of patients were male. The tumor was superficially located in 63% of patients, and the mean tumor size at presentation was 7.8 cm. All patients received neoadjuvant RT and completed their planned course of treatment. Neoadjuvant chemotherapy was administered in 22% of patients. Inadvertent excision (IE) before definitive treatment was performed in 25 patients (51%), 84% of which had superficially located tumors. All margins were assessed using frozen section analysis at the time of definitive surgery, and 100% of patients had negative surgical margins, with 25% having no residual tumor. With a median follow-up of 4.7 years, the 5-year local control rate was 87%, and 5-year overall survival was 98%. Tumor depth was associated with distant metastasis ( P < .01)., Conclusions: Despite the infiltrative nature of MFS, preoperative RT followed by definitive surgical excision, especially in the setting of a reliable frozen section margin analysis, was associated with excellent local control., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Background and Objectives: Recent studies have reported acceptable outcomes after arthrodesis takedown and conversion to total hip arthroplasty (THA); however, there are no reports on outcomes after oncologic resection, which are inherently complex and may portend poorer outcomes. The purpose of this study was to examine the surgical and functional outcomes of patients who underwent prior hemipelvectomy for tumor resection and were later converted to THA., Methods: All patients who had prior iliofemoral arthrodesis after oncologic resection that were later converted to THA at a single institution were examined. Charts were reviewed for demographic information, operative information, functional outcomes, and complications/reoperations., Results: All three patients in this study were males who underwent internal hemipelvectomies for chondrosarcoma. Patients were converted to THA at a mean of 26 years after arthrodesis. Mean follow-up after conversion to THA was 7.4 years. During this follow-up period, two of the three patients required revision surgery. At last follow-up, the mean Harris Hip Score was 81 and the mean Mayo Hip Score was 67, and all patients were ambulatory without significant pain., Conclusions: Overall, patients who undergo iliofemoral arthrodesis after oncologic hemipelvectomy and are later converted to THA can expect to have a reasonable outcome, despite a high rate of complications and revision surgery., (© 2023 Wiley Periodicals LLC.)

Background: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a mono-articular, soft-tissue tumor. Although it can behave locally aggressively, D-TGCT is a non-malignant disease. This is the first study describing the natural course of D-TGCT and evaluating active surveillance as possible treatment strategy., Methods: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected., Results: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment., Conclusion: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: G. and M.A.J.v.d.S. report research funding from Daiichi-Sankyo to the department of Orthopedic Surgery of the Leiden University Medical Center outside the submitted work. E.S, E.P, R.L R., S.W.T, J.S.W., P.C.F., F.G.M.V, M.T.H., N.M.B., B.H.W.B.S., H.G. and L.v.d.H. declare no conflict of interest., (© 2024 Published by Elsevier Ltd.)

Background: Massive modular endoprostheses have become a primary means of reconstruction after oncologic resection of a lower extremity tumor. These implants are commonly made with cobalt-chromium alloys that can undergo wear and corrosion, releasing cobalt and chromium ions into the surrounding tissue and blood. However, there are few studies about the blood metal levels in these patients., Question/purpose: What is the whole blood cobalt and chromium ion level in patients with massive modular endoprostheses?, Methods: We performed a cross-sectional study of our total joints registry to identify patients with a history of an endoprosthetic reconstruction performed at our institution. Patients who were alive at the time of our review in addition to those undergoing an endoprosthetic reconstruction after an oncologic resection were included. Whole blood samples were obtained from 27 (14 male and 13 female) patients with a history of a lower extremity oncologic endoprosthesis. The median time from surgery to blood collection was 8 years (range 6 months to 32 years). Blood samples were collected and stored in metal-free ethylenediaminetetraacetic acid tubes. Samples were analyzed on an inductively coupled plasma mass spectrometer in an International Organization for Standardization seven-class clean room using polytetrafluoroethylene-coated instruments to reduce the risk of metal contamination. The analytical measuring range was 1 to 200 ng/mL for chromium and cobalt. Cobalt and chromium levels were considered elevated when the blood level was ≥ 1 ppb., Results: Cobalt levels were elevated in 59% (16 of 27) of patients, and chromium levels were elevated in 26% (seven of 27). In patients with elevated metal ion values, 15 of 17 patients had a reconstruction using a Stryker/Howmedica Global Modular Replacement System implant., Conclusion: Blood metal levels were elevated in patients who received reconstructions using modular oncology endoprostheses Future work is needed to establish appropriate follow-up routines and determine whether and when systemic complications occur because of elevated metal levels and how to potentially address these elevated levels when complications occur. Prospective and retrospective collaboration between multiple centers and specialty societies will be necessary to address these unknown questions in this potentially vulnerable patient group., Level of Evidence: Level IV, therapeutic study., Competing Interests: Each author certifies that there are no funding or commercial associations (consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article related to the author or any immediate family members. All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research® editors and board members are on file with the publication and can be viewed on request., (Copyright © 2023 by the Association of Bone and Joint Surgeons.)

Background: Multiple hereditary exostosis (MHE) is a rare autosomal dominant disorder characterized by multiple osteochondromas. There is a paucity of literature concerning total hip arthroplasty (THA) in patients with MHE. The aim of this study is to report long-term outcomes of THA in patients with MHE., Methods: Fourteen patients undergoing 15 THA's for the treatment of osteoarthritis in the presence of osteochondromas and proximal femoral deformity secondary to MHE were reviewed. Mean age at the time of surgery and follow-up was 56 and 12 years. Seven (47%) had uncemented femoral components. Eleven hips had coxa valga on preoperative imaging. Clinical outcomes were assessed with both Harris hip scores (HHS) and Musculoskeletal Tumor Society Scores (MSTS)., Results: Following surgery, there was an improvement in the HHS (48-82, p < 0.01) and MSTS scores (41-70%, p < 0.01). Complications occurred in 5 patients leading to reoperation in 3 patients, of which 2 patients underwent a revision procedure at 19 and 20-years postoperative. The 10-year revision free survival was 100%., Conclusions: THA in the setting of MHE reliably improves patient function. One in three patients will have a postoperative complication; however, the long-term incidence of revision is low., (© 2023. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Introduction: The humerus is a common site of metastases and primary tumors. For some patients with a segmental defect and/or diaphyseal cortical destruction a cemented intercalary device may provide a more reliable construct, however data on their use is limited., Methods: We reviewed 43 (28 male and 15 female) patients treated with an intercalary humeral spacer at a single tertiary referral center between 1989 and 2022. Humeral lesions were most commonly secondary to metastatic disease (n = 29, 68%), with 25 (58%) patients presenting with a pathologic fracture. Mean age and body mass index were 66 years and 27.9 kg/m 2 . First generation taper joint device were used in 22 patients and second-generation lap device in 21 patients., Results: Following reconstruction the 2-year overall survival was 30%. Mechanical complications occurred in 11 patients, most commonly aseptic loosening (n = 6, 14%). With death as a competing risk, the cumulative incidence of mechanical failure was 28% at 2-years postoperative. Following the procedure, mean Musculoskeletal Tumor Society scores was 70% and mean shoulder elevation was 87°., Conclusion: Reconstruction of the humeral diaphysis with an intercalary endoprosthesis provides restoration of function of the upper extremity, however, is associated with one in four patients having mechanical failure., (© 2023 Wiley Periodicals LLC.)

Aims: The sacroiliac joint (SIJ) is the only mechanical connection between the axial skeleton and lower limbs. Following iliosacral resection, there is debate on whether reconstruction of the joint is necessary. There is a paucity of data comparing the outcomes of patients undergoing reconstruction and those who are not formally reconstructed., Methods: A total of 60 patients (25 females, 35 males; mean age 39 years (SD 18)) undergoing iliosacral resection were reviewed. Most resections were performed for primary malignant tumours (n = 54; 90%). The mean follow-up for surviving patients was nine years (2 to 19)., Results: Overall, 27 patients (45%) were reconstructed, while 33 (55%) had no formal reconstruction. There was no difference in the use of chemotherapy (p = 1.000) or radiotherapy (p = 0.292) between the groups. Patients with no reconstruction had a mean larger tumour (11 cm (SD 5) vs 8 cm (SD 4); p = 0.014), mean shorter operating times (664 mins (SD 195) vs 1,324 mins (SD 381); p = 0.012), and required fewer blood units (8 (SD 7) vs 14 (SD 11); p = 0.012). Patients undergoing a reconstruction were more likely to have a deep infection (48% vs 12%; p = 0.003). Nine reconstructed patients had a hardware failure, with five requiring revision. Postoperatively 55 (92%) patients were ambulatory, with no difference in the proportion of ambulatory patients (89% vs 94%; p = 0.649) or mean Musculoskeletal Tumor Society Score (59% vs 65%; p = 0.349) score between patients who did or did not have a reconstruction. The ten-year disease-specific survival was 69%, with no difference between patients who were reconstructed and those who were not (78% vs 45%; p = 0.316). There was no difference in the rate of metastasis between the two groups (hazard ratio (HR) 2.78; p = 0.102)., Conclusion: Our results demonstrate that SIJ reconstruction is associated with longer operating times, greater need for blood transfusion, and more postoperative infections, without any improvement in functional outcomes when compared to patients who did not have formal SIJ reconstruction., Competing Interests: M. T. Houdek reports consulting fees from Link Orthopaedics, unrelated to this study. M. T. Houdek also reports leadership or a fiduciary role, paid or unpaid, for the Musculoskeletal Tumor Society, Connective Tissue Oncology Society, and Sarcoma Alliance for Research through Collaboration. P. S. Rose reports leadership or a fiduciary role, paid or unpaid, for the International Society of Limb Salvage and the Sacral Tumor Study Group, and is Editor of the Journal of the American Academy of Orthopaedic Surgeons., (© 2024 The British Editorial Society of Bone & Joint Surgery.)

The bony shoulder girdle consists of the clavicle, humerus, and scapula, which work synergistically to form a complex articulation that is essential for use of the upper extremity. The shoulder girdle is the most common location for primary and secondary bone tumors in the upper extremity, and following resection of these tumors, reconstruction of the upper extremity is challenging. Compared with those in the lower extremity, reconstructive techniques in the upper extremity have historically been unreliable and fraught with complications and poor functional outcomes. Newer reconstructive techniques using reverse total shoulder arthroplasty and functional muscle flaps have shown promise to improve outcomes while reducing complications for proximal humerus reconstructions. Despite these advancements, reconstruction following scapulectomy remains challenging and is still associated with more frequent complications and compromised function.

Background: Pubic symphysis osteomyelitis can result from urosymphyseal fistula formation. High rates of sacropelvic insufficiency fractures have been reported in this population. The aim of this study was to describe the presentation and risk factors for sacral insufficiency fractures (SIF) associated with surgical treatment of pubic symphysis osteomyelitis., Methods: A retrospective review was performed for 54 patients who underwent surgery for pubic symphysis osteomyelitis associated with a urosymphyseal fistula at a single institution from 2009 to 2022. Average age was 71 years and 53 patients (98%) were male. All patients underwent debridement or partial resection of the pubic symphysis at the time of fistula treatment. Average width of the symphyseal defect was 65 mm (range 9-122) after treatment., Results: Twenty patients (37%) developed SIF at a mean time of 4 months from osteomyelitis diagnosis. Rate of sacral fracture on Kaplan-Meier analysis was 31% at 6 months, 39% at 12 months, and 41% at 2 years. Eleven patients developed SIF prior to pubic debridement and 12 patients developed new or worsening of pre-existing SIF following surgery. Width of pubic resection was higher in patients who developed SIF (76 mm vs. 62 mm), but this did not meet statistical significance (p = 0.18)., Conclusion: Sacral insufficiency fracture is a common sequela of pubic symphysis osteomyelitis. These fractures are often multifocal within the pelvis and can occur even prior to pubic resection. Pubectomy further predisposes these patients to fracture. Clinicians should maintain a high index of suspicion for these injuries in patients with symphyseal osteomyelitis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

With advances in chemotherapy and radiation therapy, surgical treatment of patients with bone sarcomas has advanced from most patients undergoing an amputation to now most patients undergoing a limb salvage procedure. With the advances of limb salvage surgical techniques, reconstructive procedures have expanded to include autografts, allografts, endoprosthetic replacements, and rotationplasty. In a growing child, the decision to perform each of these reconstructive options is individualized and each needs to be considered to provide the patient with the optimal oncologic and functional outcome, while being durable to minimize the risk of complications and subsequent surgeries.

Aims: The scapula is a rare site for a primary bone tumour. Only a small number of series have studied patient outcomes after treatment. Previous studies have shown a high rate of recurrence, with functional outcomes determined by the preservation of the glenohumeral joint and deltoid. The purpose of the current study was to report the outcome of patients who had undergone tumour resection that included the scapula., Methods: We reviewed 61 patients (37 male, 24 female; mean age 42 years (SD 19)) who had undergone resection of the scapula. The most common resection was type 2 (n = 34) according to the Tikhoff-Linberg classification, or type S1A (n = 35) on the Enneking classification., Results: The ten-year disease-specific survival was 76%. High tumour grade (hazard ratio (HR) 4.27; p = 0.016) and a total resection of the scapula (HR 3.84; p = 0.015) were associated with worse survival. The ten-year metastasis-free and local recurrence-free survivals were 82% and 86%, respectively. Total scapular resection (HR 6.29; p = 0.004) was associated with metastatic disease and positive margins were associated with local recurrence (HR 12.86; p = 0.001). At final follow-up, the mean shoulder forward elevation and external rotation were 79° (SD 62°) and 27° (SD 25°), respectively. The most recent functional outcomes evaluated included the mean Musculoskeletal Tumor Society Score (76% (SD 17%)), the American Shoulder and Elbow Score (73% (SD 20%)), and the Simple Shoulder Test (7 (SD 3)). Preservation of the glenoid (p = 0.001) and scapular spine (p < 0.001) improved clinical outcomes; interestingly, preservation of the scapular spine without the glenoid improved outcomes (p < 0.001) compared to preservation of the glenoid alone (p = 0.05)., Conclusion: Resection of the scapula is a major undertaking with an oncological outcome related to tumour grade, and a functional outcome associated with the status of the scapular spine and glenoid. Positive resection margins are associated with local recurrence., Competing Interests: J. D. Barlow reports personal and institutional royalties and consulting payments from Stryker, unrelated to this study. M. Houdek reports consulting payments from Link Orthopedics, unrelated to this study, and a leadership or fiduciary role in the Musculoskeletal Tumor Society, the Connective Tissue Oncology Society, and the Sarcoma Alliance for Research through Collaboration. P. S. Rose reports a leadership or fiduciary role in the International Society of Limb Salvage, the Sacral Tumor Study Group, and the Journal of the American Academy of Orthopaedic Surgeons. J. Sanchez-Sotelo reports grants from Stryker, royalties from Stryker, Oxford University Press, and Elsevier, consulting payments from Stryker, Acumed, and Exactech, speaker payments from the Journal of Shoulder and Elbow Surgery, a leadership or fiduciary role in American Shoulder and Elbow Surgeons, and stock or stock options in Precision OS, Orthobullets, and PSI, all of which are unrelated to this study. E. Wagner reports consulting fees from Stryker, Zimmer Biomet, Acumed, and Osteoremedies, all of which are unrelated to this study., (© 2023 The British Editorial Society of Bone & Joint Surgery.)

Background: Extraskeletal Ewing sarcoma are rare tumors within the Ewing sarcoma family. Initial staging studies for extraskeletal Ewing sarcoma historically have included imaging and bone marrow aspiration and biopsy (BMAB). However, recent studies on Ewing sarcoma of bone have questioned the utility of BMAB in the initial staging of patients, but no studies of which we are aware have evaluated the role of BMAB in extraskeletal Ewing sarcoma. We suspected that BMAB was of low diagnostic yield in patients with extraskeletal Ewing sarcoma and exposed patients to potential morbidity without an impact on their clinical course., Question/purpose: Is BMAB a useful test in the staging of extraskeletal Ewing sarcoma?, Methods: Between January 1996 and December 2021, our institution evaluated 109 patients with a listed diagnosis of extraskeletal Ewing sarcoma. Those patients were retrospectively reviewed for this study. Of those, we considered patients with biopsy-confirmed diagnosis of extraskeletal Ewing sarcoma. Biopsy was performed based on institutional protocols, with all diagnoses assigned by a board-certified pathologist. Based on that criteria, 96% (105 of 109) were eligible. An additional 18% (20 of 109) were excluded because records of their initial diagnostic and staging workup were not available. This left 78% (85 of 109) for analysis. Of those, 52% (44 of 85) were male. The average age was 32 ± 16 years. Primary tumor locations included extremities in 26% (22 of 85), paraspinal in 20% (17 of 85), chest in 19% (16 of 85), retroperitoneum in 13% (11 of 85), intraabdominal in 12% (10 of 85), intrapelvic in 7% (6 of 85), and head or neck in 4% (3 of 85). Initial diagnostic and staging information, including the use of PET-CT, bone scan, CT chest, and BMAB, was collected. Metastatic disease at the time of presentation or during follow-up was noted. The utility of BMAB was determined by the rate of positive tests in those undergoing BMAB during the initial staging process. Descriptive statistical analysis was sufficient to address the study question, and therefore no comparative statistics were performed., Results: BMAB was obtained during the initial staging process in 64% (54 of 85) of patients. This BMAB was negative in all 54 patients, including those with known metastatic disease., Conclusion: Diagnosing metastatic disease in extraskeletal Ewing sarcoma is important as the presence of metastases at diagnosis adversely affects prognosis. The routine use of BMAB in the staging process of extraskeletal Ewing sarcoma is of low diagnostic yield. BMAB is unlikely to diagnose metastatic involvement even in patients with known metastases to bone. We do not have enough data to suggest whether other modalities, such as PET-CT, might be more useful. Similar studies should be pursued to determine the utility of the remainder of staging modalities in patients with extraskeletal Ewing sarcoma to elucidate the most efficient and effective staging protocol., Level of Evidence: Level III, diagnostic study., Competing Interests: Each author certifies that there are no funding or commercial associations (consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article related to the author or any immediate family members. All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research® editors and board members are on file with the publication and can be viewed on request., (Copyright © 2023 by the Association of Bone and Joint Surgeons.)

Background: Intraneural ganglion cysts involving the tibial nerve are rare. Recent evidence has supported an articular (synovial) theory to explain the joint-related origin of these cysts; however, optimal operative treatment for cysts originating from the STFJ remains poorly understood. Therefore, we present a novel strategy: addressing the joint itself without addressing the articular branch and/or the cyst., Methods: Records of patients with tibial intraneural ganglion cysts with a connection to the STFJ who were treated with a joint resection alone at a single academic institution were reviewed. The clinicoradiographic features, operative intervention, and postoperative course were recorded., Results: We identified a consecutive series of 7 patients. These patients (4/7 male, 57%) were 43 (range 34-61) years of age and all presented with symptoms of neuropathy. The patients underwent resection of the synovial surfaces of the STFJ without disconnection of the articular branch or decompression of the cyst. Postoperatively, three patients regained partial motor function (43%, n=7), although four patients noted continued sensory abnormality (57%, 4/7). All six patients with postoperative MRIs had some evidence of regression of the cyst., Conclusions: This novel surgical technique serves as a proof of concept-highlighting the fact that treating the primary source (the joint origin) can be effective in eliminating the secondary problem (the cyst itself). While this study shows that this simplified approach can be employed in select cases, we believe that superior results (faster, fuller recovery) can be achieved with combinations of disconnecting the articular branch, decompressing the cyst, and/or resecting the joint., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Large skeletal muscle defects owing to trauma or following tumor extirpation can result in substantial functional impairment. Purified exosomes are now available clinically and have been used for wound healing. The objective of this study was to evaluate the regenerative capacity of commercially available exosomes on an animal model of volumetric muscle loss (VML) and its potential translation to human muscle injury. An established VML rat model was used. In the in vitro experiment, rat myoblasts were isolated and cocultured with 5% purified exosome product (PEP) to validate uptake. Myoblast proliferation and migration was evaluated with increasing concentrations of PEP (2.5%, 5%, and 10%) in comparison with control media (F10) and myoblast growth medium (MGM). In the in vivo experiment, a lateral gastrocnemius-VML defect was made in the rat hindlimb. Animals were randomized into four experimental groups; defects were treated with surgery alone, fibrin sealant, fibrin sealant and PEP, or platelet-rich plasma (PRP). The groups were further randomized into four recovery time points (14, 28, 45, or 90 days). The isometric tetanic force (ITF), which was measured as a percentage of force compared with normal limb, was used for functional evaluation. Florescence microscopy confirmed that 5% PEP demonstrated cellular uptake ∼8-12 h. Compared with the control, myoblasts showed faster proliferation with PEP irrespective of concentration. PEP concentrations of 2.5% and 5% promoted myoblast migration faster compared with the control (<0.05). At 90 days postop, both the PEP and fibrin sealant and PRP groups showed greater ITF compared with control and fibrin sealant alone (<0.05). At 45 days postop, PEP with fibrin sealant had greater cellularity compared with control (<0.05). At 90 days postop, both PEP with fibrin sealant and the PRP-treated groups had greater cellularity compared with fibrin sealant and control (<0.05). PEP promoted myoblast proliferation and migration. When delivered to a wound with a fibrin sealant, PEP allowed for muscle regeneration producing greater functional recovery and more cellularity in vivo compared with untreated animals. PEP may promote muscle regeneration in cases of VML; further research is warranted to evaluate PEP for the treatment of clinical muscle defects.

Three-dimensional (3D) printing technology has proven to have many advantages in spine and sacrum surgery. 3D printing allows the manufacturing of life-size patient-specific anatomic and pathologic models to improve preoperative understanding of patient anatomy and pathology. Additionally, virtual surgical planning using medical computer-aided design software has enabled surgeons to create patient-specific surgical plans and simulate procedures in a virtual environment. This has resulted in reduced operative times, decreased complications, and improved patient outcomes. Combined with new surgical techniques, 3D-printed custom medical devices and instruments using titanium and biocompatible resins and polyamides have allowed innovative reconstructions., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Rotationplasty is a reconstructive, limb-sparing surgery indicated for patients with lower extremity musculoskeletal tumors. The procedure involves rotation of the distal lower extremity to allow the ankle to function as the new knee joint and provide an optimum weight-bearing surface for prosthetic use. Historically there is limited data comparing fixation techniques. The purpose of this study is to compare clinical outcomes between intramedullary nailing (IMN) and compression plating (CP) in young patients undergoing rotationplasty., Methods: A retrospective review of 28 patients with a mean age of 10±4 years undergoing a rotationplasty for either a femoral (n=19), tibial (n=7), or popliteal fossa (n=2) tumor was performed. The most common diagnosis was osteosarcoma (n=24). Fixation was obtained with either an IMN (n=6) or CP (n=22). Clinical outcomes of patients undergoing rotationplasty were compared between the IMN and CP groups., Results: Surgical margins were negative in all patients. The mean time to union was 24 months (range 6 to 93). There was no difference in the meantime to the union between patients treated with an IMN versus those with a CP (14±16 vs. 27±26 mo, P =0.26). Patients undergoing fixation with an IMN were less likely to have a nonunion (odds ratio: 0.35, 95% confidence interval: 0.03-3.54, P =0.62). Postoperative fracture of the residual limb only occurred in patients undergoing CP fixation (n=7, 33% vs. n=0, 0%, P =0.28). Postoperative fixation complications occurred in 13 (48%) patients, most commonly a nonunion (n=9, 33%). Patients undergoing fixation with a CP were more likely to have a postoperative fixation complication (odds ratio: 20, 95% CI: 2.14-186.88, P <0.01)., Conclusions: Rotationplasty is an option for limb salvage for young patients with lower extremity tumors. The results of this study reveal fewer fixation complications when an IMN can be used. As such, IMN fixation should be considered for patients undergoing a rotationplasty, though equipoise should be shown by surgeons when determining technique., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background/aim: Pre-emptive targeted muscle reinnervation (TMR) at the time of amputation results in less phantom limb pain (PLP) compared with untreated amputee controls. There is limited literature describing the technique in patients undergoing hindquarter amputation despite up to 90% of these patients reporting PLP and 50% presenting with painful neuroma. The purpose of the current study was to describe the motor nerves accessible through a primary hind-quarter amputation to be used for TMR and review pain outcomes in clinical case correlates of patients with TMR., Patients and Methods: Six limbs were obtained from three fresh adult cadavers and proximal sensory and motor nerves were dissected. A review of patients undergoing hindquarter amputation with TMR was conducted., Results: Transfers for the sciatic, femoral, and obturator nerves were identified in cadavers. In reviews of patients, they were taking narcotic and neuro-leptic pain medication for a mean of 23 days and 168 days. At most recent follow-up, no patient reported debilitating phantom pain nor pain associated with neuromas., Conclusion: Given the positive preliminary results in our study group as well as the accessible neuroanatomy, pre-emptive TMR should be considered at the time of surgery to limit PLP and dependence on pain medications., (Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Purpose: Extraskeletal Ewing sarcoma (EES), is a rare soft tissue sarcoma. Treatment for EES commonly involves chemotherapy and surgical resection (ST) or less commonly combined chemotherapy, surgery, and radiotherapy (ST + RT). The purpose of the current study was to evaluate our institutional experience treating EES., Methods: We reviewed 36 (18 males:18 females) patients (mean age 30 years) with a nonretroperitoneal/visceral EES treated with either ST (n = 24, 67%) or ST + RT (n = 12, 33%). All patients were treated with chemotherapy, most commonly vincristine, doxorubicin, cyclophosphamide/ifosfamide and etoposide (VDC/IE, n = 23, 66%) Radiotherapy was mostly delivered preoperatively (n = 9). The mean follow-up was 8 years., Results: The 10-year disease specific survival for patients was 78%, with no difference in the survival between patients in the ST versus the ST + RT groups (83% vs. 71%, p = 0.86). There was no difference in the 10-year local recurrence (91% vs. 100%, p = 0.29) or metastatic free survival (87% vs. 75%, p = 0.45) between the ST and ST + RT groups., Conclusion: The results of the current study highlight the ability to achieve excellent local control with chemotherapy and surgery for EES. We recommend for multidisciplinary management of patients with EES, including chemotherapy and surgery, with use of radiotherapy if there is concern for a potentially close margin of resection., (© 2023 Wiley Periodicals LLC.)

Merkel cell carcinoma (MCC) has a tendency for lymphatic spread and locoregional recurrence, although there is little data examining the risk factors for patients with lymph node-positive extremity lesions. The purpose of the current study was to examine the outcomes and risk factors associated with nodal metastasis in extremity MCC. We retrospectively reviewed the medical record of 120 patients with extremity MCC evaluated at our institution between 1994 and 2021. The mean age of this cohort was 71 years; 33% of patients were female; and 98% were Caucasian. Seventy-eight (65%) patients presented with localized disease. Thirty-seven (31%) patients had stage III disease, and five (4%) patients had stage IV disease. Treatment of primary lesions consisted primarily of margin-negative excision and adjuvant radiotherapy. Nodal metastases were most treated with adjuvant radiation or completion lymph node dissection. Five-year disease-specific survival in our series was 88% for patients with localized disease, 89% for stage IIIa disease, 40% for stage IIIb disease and 42% for stage IV. Factors associated with worse survival included immunosuppression and macroscopic nodal disease. In conclusion, extremity MCC has a low rate of local recurrence when treated with margin-negative excision and adjuvant radiation. However, treatment of nodal metastases remains a challenge with high rates of recurrence and mortality, particularly for patients who are immunosuppressed or who have macroscopic nodal disease.

Background: The distal radius is the most common location for giant cell tumors (GCT) in the upper extremity. Treatment should balance the goals of maximizing function and minimizing recurrence and other complications. Given the complexity in surgical treatment, various techniques have been described without clear standards of treatment., Objectives: The purpose of this review is to provide an overview of evaluation of patients presenting with GCT of the distal radius, discuss management, and provide an updated summary on outcomes of treatment options., Conclusion: Surgical treatment should consider tumor Grade, involvement of the articular surface, and patient-specific factors. Options include intralesional curettage and en bloc resection with reconstruction. Within reconstruction techniques, radiocarpal joint preserving and sparing procedures can be considered. Campanacci Grade 1 tumors can be successfully treated with joint preserving procedures, whereas for Campanacci Grade 3 tumors consideration should be given to joint resection to prevent recurrence. Treatment of Campanacci Grade 2 tumors is debated in the literature. Intralesional curettage and adjuvants can successfully treat cases where the articular surface can be preserved, while en-bloc resection should be used in cases where the articular surface cannot undergo aggressive curettage. A variety of reconstructive techniques are used for cases needing resection, with no clear gold standard. Joint sparing procedures preserve motion at the wrist joint, whereas joint sacrificing procedures preserve grip strength. Choice of reconstructive procedure should be made based on patient-specific factors, considering relative functional outcomes, complications, and recurrence rates., Competing Interests: None., (© 2023 Professor P K Surendran Memorial Education Foundation. Published by Elsevier B.V. All rights reserved.)

Background/aim: Skin cancers are the most common malignancy of the hand and wrist. Merkel cell carcinoma (MCC) is a rare, aggressive non-melanoma skin cancer arising from cutaneous neuroendocrine cells and is known for local and distant recurrence. The purpose of the current study was to examine the treatment outcome of patients with MCC of the hand and wrist., Patients and Methods: We reviewed 25 patients (18 males:7 females) with MCC that occurred in the hand and wrist. The mean age at the time of biopsy of 71±11 years., Results: Tumors were located on the hand (n=13), finger/thumb (n=9), and wrist (n=3). Local control included wide local excision (n=22). This included 21 non-amputation resections and one 5 th digit ray amputation. Sentinel lymph node biopsy was performed in 21 patients with positive nodal disease in seven cases. Adjuvant radiotherapy was delivered to the primary site in 17 patients and additionally to the regional lymph node basin in six patients. Recurrence within five years was noted in 40% of patients (mean time to recurrence 18.4±20.6 months). Recurrence-free and disease-specific survival rates at 5-years were 54.8% and 67.6%., Conclusion: MCC is a rare cutaneous neuroendocrine carcinoma with a high propensity for regional nodal spread. Despite aggressive local treatment, adjuvant radiotherapy to the primary site and regional nodes, MCC of the hand and wrist has a high rate of recurrence and mortality within five years of diagnosis., (Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Compared to other sarcomas, myxoid liposarcoma (ML) is known to be radiosensitive, with improved oncologic outcomes. Although these tumors "shrink" following radiotherapy, there is a paucity of data examining the degree of radiosensitivity and oncologic outcome. The purpose of the study was to evaluate pre- and postradiotherapy tumor volume to determine if size reduction impacts outcome., Methods: We reviewed 62 patients with ML undergoing surgical resection combined with preoperative radiotherapy, with pre- and postradiotherapy MRI. This included 34 (55%) males, with a mean age of 47 ± 14 years. All tumors were deep to the fascia, and 12 (19%) patients had tumors with a >5% round-cell component., Results: The mean volume reduction was 54% ± 29%. Compared to patients with >25% volume reduction, patients with reduction ≤25% had worse 10-year disease specific survival (86% vs. 37%, p < 0.01), in addition to an increased risk of metastatic disease (HR 4.63, p < 0.01) and death due to disease (HR 4.52, p < 0.01)., Conclusion: Lack of volume reduction is a risk factor for metastatic disease and subsequent death due to disease in patients with extremity ML treated with combined preoperative radiotherapy and surgery. This data could be used to stratify patients for adjuvant therapies and follow-up intervals., (© 2022 Wiley Periodicals LLC.)

Surgical treatment of pelvic sarcoma involving the bone is the standard of care but is associated with several sequelae and reduced functional quality of life (QOL). Treatment with photon and proton radiotherapy is associated with relapse. Carbon ion radiotherapy (CIRT) may reduce both relapse rates and treatment sequelae. The PROSPER study is a tricontinental, nonrandomized, prospective, three-arm, pragmatic trial evaluating treatments of pelvic sarcoma involving the bone. Patients aged at least 15 years are eligible for inclusion. Participants must have an Eastern Cooperative Oncology Group Performance Status score of two or less, newly diagnosed disease, and histopathologic confirmation of pelvic chordoma, chondrosarcoma, osteosarcoma, Ewing sarcoma with bone involvement, rhabdomyosarcoma (RMS) with bone involvement, or non-RMS soft tissue sarcoma with bone involvement. Treatment arms include (1) CIRT ( n = 30) delivered in Europe and Asia, (2) surgical treatment with or without adjuvant radiotherapy ( n = 30), and (3) proton therapy ( n = 30). Arms two and three will be conducted at Mayo Clinic campuses in Arizona, Florida, and Minnesota. The primary end point is to compare the 1-year change in functional QOL between CIRT and surgical treatment. Additional comparisons among the three arms will be made between treatment sequelae, local control, and other QOL measures.

(1) Background: Resection of soft-tissue sarcomas (STS) of the upper extremity can result in substantial functional impairment with limited options for functional reconstruction. Free functional latissimus flaps have been utilized to restore function of the thigh; however, there is limited data on the use of latissimus flaps for functional reconstruction in the upper extremity. As such, we sought to evaluate our institutional experience with these flaps. (2) Methods: We reviewed ten (seven male; three female; and a mean age of 63 years) patients undergoing soft-tissue sarcoma resection involving the triceps ( n = 4), biceps ( n = 4), and deltoid ( n = 2) reconstructed with a pedicled functional latissimus flap. All surviving patients had at least 1 year of follow-up, with a mean follow-up of 5 years. (3) Results: The mean elbow range of motion and shoulder elevation were 105° and 150°. The mean Musculoskeletal Tumor Society score was 88%, and the muscle strength was four. Four patients had a recipient site wound complication. There were no flap losses. One patient sustained a radiation-associated humerus fracture 5 years postoperatively, treated nonoperatively. (4) Conclusions: Although early complications are high, pedicled functional latissimus flaps allow for wound coverage, potential space obliteration, and restoration of function in the upper extremity following resection of large soft tissue sarcomas.

Introduction: Chordomas of the mobile spine are rare malignant tumors. The purpose of this study was to review the outcomes of treatment for patients with recurrent mobile spine chordomas., Methods: The oncologic outcomes and survival of 30 patients undergoing treatment of a recurrent mobile spine chordoma were assessed over a 24-year period. The mean follow-up was 3.5 years., Results: In patients presenting with a recurrent mobile spine chordoma, the mean 2- and 5-year overall survival was 73% and 39%, respectively. Enneking appropriate resection trended toward improved overall survival at 5 years (100% vs. 32%, P = 0.24). Those undergoing surgical resection for recurrence had improved metastatic-free survival (hazard ratio 0.29, CI 0.08 to 0.99, P = 0.05). Positive margins were found to be a risk factor of further local recurrence (hazard ratio 7.92, CI 1.02 to 61.49, P = 0.04). Those undergoing nonsurgical management trended toward having an increase in new neurologic deficits (P = 0.09), however, there was no difference in overall complications based on treatment type (P = 0.13)., Conclusion: Recurrent mobile spine chordoma portends a poor prognosis with an overall survival of less than 40% at 5 years. Surgical resection may help prevent new neurologic deficits and tumor metastasis while en bloc excision with negative surgical margins is associated with improved local recurrence-free survival., (Copyright © 2022 by the American Academy of Orthopaedic Surgeons.)

Diffuse-type tenosynovial giant cell tumors' (D-TGCTs) intra- and extra-articular expansion about the knee often necessitates an anterior and posterior surgical approach to facilitate an extensive synovectomy. There is no consensus on whether two-sided synovectomies should be performed in one or two stages. This retrospective study included 191 D-TGCT patients from nine sarcoma centers worldwide to compare the postoperative short-term outcomes between both treatments. Secondary outcomes were rates of radiological progression and subsequent treatments. Between 2000 and 2020, 117 patients underwent one-stage and 74 patients underwent two-stage synovectomies. The maximum range of motion achieved within one year postoperatively was similar (flexion 123-120°, p = 0.109; extension 0°, p = 0.093). Patients undergoing two-stage synovectomies stayed longer in the hospital (6 vs. 4 days, p < 0.0001). Complications occurred more often after two-stage synovectomies, although this was not statistically different (36% vs. 24%, p = 0.095). Patients treated with two-stage synovectomies exhibited more radiological progression and required subsequent treatments more often than patients treated with one-stage synovectomies (52% vs. 37%, p = 0.036) (54% vs. 34%, p = 0.007). In conclusion, D-TGCT of the knee requiring two-side synovectomies should be treated by one-stage synovectomies if feasible, since patients achieve a similar range of motion, do not have more complications, but stay for a shorter time in the hospital.

Background: Regional lymph node metastasis (RLNM) occurs infrequently in patients with soft tissue sarcoma (STS), although certain STS subtypes have a higher propensity for RLNM. The identification of RLNM has significant implications for staging and prognosis; however, the precise impact of node-positive disease on patient survival remains a topic of controversy. Although the benefits of sentinel lymph node biopsy (SLNB) are well documented in patients with melanoma and breast cancer, whether this procedure offers a benefit in STS is controversial., Methods: A systematic literature search was performed and articles reviewed to determine if SLNB in patients with extremity/truncal STS impacts disease-free or overall survival., Results: Six studies were included. Rates of sentinel lymph node positivity were heterogeneous (range 4.3-50%). The impact of SLNB on patient outcomes remains unclear. The overall quality of available evidence was low, as assessed by the Grading of Recommendations, Assessment, Development, and Evaluation system., Conclusions: The literature addressing the impact of nodal basin evaluation on the staging and management of patients with extremity/truncal STS is confounded by heterogeneous patient cohorts and clinical practices. Multicenter prospective studies are warranted to determine the true incidence of RLNM and whether SLNB could benefit patients with clinically occult RLNM at diagnosis., (© 2022. Society of Surgical Oncology.)

Background: Compared with other soft tissue sarcomas, myxoid liposarcoma (MLS) occurs in younger patients, has a propensity for intermuscular locations and is highly radiosensitive. With pre-operative radiotherapy, intermuscular MLS demonstrates substantial volume reduction and can be easily separated from surrounding tissues during resection. However, it is unclear whether marginal excision of MLS is oncologically safe. This study aimed to assess the association between margins and survival in irradiated, intermuscular MLS., Methods: The study identified 198 patients from seven sarcoma centres with a first presentation of localized, extremity, intermuscular MLS that received pre-operative radiotherapy and was diagnosed between 1990 and 2017. Patient and treatment characteristics, radiological and histological responses to neoadjuvant treatment and clinical surveillance were recorded., Results: Margins were microscopically positive in 11% (n = 22), <1.0 mm in 15% (n = 29) and ≥1.0 mm in 72% (n = 143). There was no association between margin status and local recurrence-free, metastasis-free or overall survival. This finding held true even in patients at higher risk of worse overall survival based on multivariable analysis (% round cell≥5%, percentage ellipsoid tumour volume change ≤ -60.1%)., Conclusion: Irradiated, extremity, intermuscular myxoid liposarcoma can safely undergo marginal resection without compromising oncologic control., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier Ltd.)

Scapular resections are large oncologic undertakings. Due to the soft tissue coverage of the scapula, tumors are often able to be resected with a negative margin. Involvement of the brachial plexus and axillary vessels is rare, allowing for a limb-salvage surgery in most cases. Functional outcomes are based on the magnitude of resection; patients undergoing a partial scapulectomy and those with glenoid preservation demonstrate improved outcomes compared to patients undergoing a total scapulectomy or glenoid resection. Although scapular endoprosthetics are available, there is limited data to support their routine use., Competing Interests: Disclosure The authors have no disclosures., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The proximal humerus is a common location for primary tumors, benign lesions, and metastatic disease. Advances in neoadjuvant and adjuvant therapy have allowed for limb-salvage surgery in most of the cases. There are numerous of options for surgical management of proximal humerus lesions and the decision to pursue one over another depends on factors such as age, comorbidities, pathology, location within the proximal humerus, planned resection margins/size of defect, and bone quality. Long-term outcomes for these techniques tend to be retrospective comparative studies, with recent studies highlighting the improved outcomes of reverse total shoulders., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: The use of implant arthroplasty in the distal radioulnar joint is increasing. Two main types of implants are commonly used, ulnar head prosthesis (UHP) and hemi or semi-constrained total distal radioulnar joint arthroplasty. The literature consists mainly of small patient series. The purpose of this study was to examine our long-term outcomes of distal radioulnar joint arthroplasty., Methods: Patient data were collected in a patient registry from 2000 to 2019. The follow-up included radiographic examination, physical examination, Mayo Wrist Scores, pain level, range of motion, and grip strength. Reoperations were recorded. The implants were a semi-constrained prosthesis and a metallic UHP. The mean age at surgery was 50 years. Patient demographics were similar, but the semi-constrained group had a higher preoperative percentage of instability (85 vs 52 percent). The median follow-up time was 30 months for the semi-constrained implants group and 102 months for the UHP group., Results: A total of 53 primary semi-constrained total joint arthroplasties and 102 UHPs were included. The grip strength and Mayo Wrist Score improved for both the implant groups. Pain reduced in 76% of the patients. Supination improved for the semi-constrained total joint arthroplasty group. Lifting capacity was better in the semi-constrained total joint arthroplasty patients. The unadjusted reoperation rate was 23% for the semi-constrained implants group and 34% for the UHP group. Twenty-two implants were bilateral; these had comparable results to unilateral implants. Kaplan-Meier survival curves demonstrated 94% survival rate for the semi-constrained implants group and 87% survival for the UHP group after 5 years. The risk factors associated with reoperation for the combined implant group included younger age at surgery, previous wrist surgery, ulnar shortening, and wrist fusion., Conclusions: Distal radioulnar joint arthroplasty improved functional outcomes in both the implant groups, but reoperations were frequent. The semi-constrained implants group had better lifting capacity. The bilateral implants had comparable outcomes to the unilateral implants., Type of Study/level of Evidence: Therapeutic IV., (Copyright © 2023. Published by Elsevier Inc.)

Background: Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a low rate of metastatic disease. Previous series have shown a superiority of Mohs micrographic surgery (MMS) compared with wide local excision (WLE). Likewise, there is paucity of data examining the long-term follow-up of patients., Objective: The purpose of the current study was to examine the outcome of surgical treatment of primary DFSP of the trunk and extremities., Methods: We reviewed 236 patients (115 females, 121 males, mean age 41 ± 15 years) undergoing MMS (n = 81, 34%) or WLE (n = 155, 66%) to treat a primary DFSP. Mean tumor size and follow-up was 4 ± 2 cm and 7 years, respectively. Final margins were negative in 230 (97%) patients., Results: There was no difference (p > 0.05) in patient age, sex, tumor size, negative margin excision, or history of a previous inadvertent excision between patients who underwent WLE and those undergoing MMS. There were two cases of local recurrence and two cases of metastasis, with no difference in the 5-year local recurrence-free survival (98% vs. 99%, p = 0.69) or metastatic-free survival (98% vs. 100%, p= 0.27) between WLE and MMS., Conclusion: There was no difference in oncologic outcome comparing MMS with WLE for DFSP outside the head and neck. The goal of treatment for DFSP is to achieve a negative margin, regardless of surgical treatment modalities. A 'less is more' approach to follow-up can likely be taken for patients with completely resected DFSP in easy-to-examine anatomical areas. In these patients, no formal follow-up should be required., (© 2022. Society of Surgical Oncology.)

Background: Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy that usually occurs in the head/neck or extremities. However, there are reports of MCC developing in the lymph nodes or parotid gland without evidence of a primary cutaneous lesion., Methods: We reviewed 415 patients with biopsy-proven MCC. Patients with MCC of unknown primary (n = 37, 9%, MCCUP) made up the study cohort. The primary endpoints of the study were rate of recurrence, disease-free survival, and overall survival., Results: Patients with MCCUP presented with tumors in lymph nodes (n = 34) or parotid gland (n = 3). Nodal disease was most commonly detected in the inguinal/external iliac (n = 15) or axillary (n = 14) regions. The mean age at diagnosis was 70 years and 24% were female. Patients presented with distant metastases in 24.3% of cases. Patients with stage IIIA disease treated with regional lymph node dissection (RLND) had a lower risk of disease recurrence (hazard ratio 0.26, p = 0.046). Recurrence-free survival was 59.3% at 5 years. Disease-specific survival was 63.3% at 5 years., Conclusion: Patients with MCCUP have a high risk of recurrence and mortality. The optimal treatment for MCCUP has yet to be elucidated, although therapeutic RLND appears beneficial for these patients., (© 2022 Wiley Periodicals LLC.)

Introduction: The distal radius is a common location for giant cell tumors (GCTs) of bone. Management includes intralesional curettage or wide excision, however, long-term comparisons of treatment options are limited. The purpose of the current study was to evaluate our institutions' outcomes of treatment of these tumors., Methods: We reviewed 24 GCT of the distal radius in 23 patients (12 males: 11 females) with a mean age of 42 years at the time of surgery. Functional outcomes were collected including the Musculoskeletal Tumor Society Score (MSTS), QuickDash, the Visual Analog Scale (VAS), and the Patient Rated Wrist Evaluation (PRWE). The mean follow-up was 13 years., Results: Tumor grade included Campanacci Grade II (n = 14) and Grade III (n = 10). Treatment included extended intralesional curettage (n = 16) and wide excision (n = 8). Reconstruction mainly included bone grafting/cement (n = 16) or free vascularized fibula radiocarpal arthrodesis (n = 5). At most recent follow-up, there was no difference in MSTS, VAS, and PRWE (p > 0.05) between patients undergoing a joint sparing or arthrodesis. Patients undergoing arthrodesis had a lower QuickDASH score (13.7 vs. 20.8, p = 0.04) CONCLUSIONS: Treatment for GCT of the distal radius is individualized however in the setting of articular surface involvement, arthrodesis can lead to superior functional results at long-term follow-up., (© 2022 Wiley Periodicals LLC.)