The mechanics of the collateral ligaments in the metacarpophalangeal joints: A scoping review.

BACKGROUND: The metacarpophalangeal (MCP) joint's collateral ligaments have been extensively debated, with no clear consensus on their mechanics. Understanding their function is crucial for comprehending joint movement and stability. METHODS: A thorough search was conducted across databases, including PubMed, Scopus, Cochrane library and grey literature. A total of 59 articles were identified, and after rigorous evaluation, six articles were included in the review. RESULTS: The analysis underscores two principal findings. Firstly, the principal and accessory collateral ligaments exhibit consistent tension influenced by the MCP joint's position. This tension varies across different sections of the ligaments. Secondly, the ligaments' interaction with the joint structure plays a pivotal role in defining the range of motion of the joint. CONCLUSION: Preliminary findings from this review indicate that MCP joint collateral ligament tension varies with joint position. Increased tension in the principal collateral ligament during flexion and isometric behavior of its volar portion in extension are observed. The accessory ligament may tighten during extension. The shape of the metacarpal head appears to influence this tension. These insights, while informative, call for further detailed research to deepen our understanding of MCP joint mechanics.

IDH mutations in G2-3 conventional central bone chondrosarcoma: a mono institutional experience.

BACKGROUND: Heterozygous isocitrate dehydrogenase (IDH) mutations occur in about half of conventional central bone chondrosarcomas (CCBC). Aim of this study was to assess the frequency and prognostic impact of IDH mutations in high grade CCBC patients. METHODS: 64 patients with G2 and G3 CCBC were included. DNA extraction, PCR amplification of IDH1/2 exon 4s, and sequencing analysis with Sanger were performed. RESULTS: IDH mutations were detected in 24/54 patients (44%): IDH1 in 18, IDH2 in 4, and both IDH1/2 in 2 patients. The frequency of mutations was 37% in G2 vs. 69% in G3 (p = 0.039), and 100% in three Ollier disease associated chondrosarcoma. 5-year overall survival (OS) at 124 months (range 1-166) was 51%, with no significant difference based on the IDH mutational status: 61% in IDHmut vs. 44% in IDH wild type (IDHwt). The 5-year relapse free survival (RFS) was 33% (95% CI:10-57) for IDHmut vs. 57% (95%CI: 30-77) for IDHwt. Progression free survival (PFS) was 25% (95%CI:1-65) IDHmut vs. 16% (95%CI: 0.7-52) IDHwt. 55% (5/9) of IDHmut G2 became higher grade at the recurrence, as compared with 25% (3/12) of G2 IDHwt. CONCLUSIONS: This study shows a higher frequency of IDH mutations in G3 CCBC as compared with G2. No significant differences in OS, RFS, and PFS by mutational status were detected. After relapse, a higher rate of G3 for IDH mutated CCBC was observed.

SELNET clinical practice guidelines for bone sarcoma.

Bone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient's outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context.

Reconstruction of the pelvic ring in iliac or iliosacral resections: allograft or autograft?

BACKGROUND: The necessity to reconstruct the pelvic ring after an iliac or iliosacral resection is still debated. Different reconstructive techniques are available, including autologous and homologous graft and custom-made prosthesis. The aim of this study was to evaluate the functional outcome and complications of patients who underwent resection of iliac bone and part of the sacrum for primary bone tumour and reconstruction with an allograft or autograft. METHODS: We retrospectively evaluated 18 patients (10 males and 8 female) with a mean age of 28 years (range 9-56) who were operated between 1992 and 2015. Six patients were reconstructed with an autograft (Group A) and 12 patients with an allograft (Group B). All complications were recorded. At final follow-up, patients with the original reconstruction still in site were functionally evaluated with MSTS Score. RESULTS: Mean follow-up time was 107 months (range 4-221). Two patients (11.1%) had an external hemipelvectomy for local recurrence, and 3 patients died of the disease. Mean MSTS Score was comparable between the two groups 21.7 (range 20-25) in Group A and 19.9 (range 4-28) in Group B. Patients with partial sacral resection had an increased risk of local recurrence and lower MSTS Score [15.5 (range 7-20) vs. 22.2 (range 4-28)]. CONCLUSIONS: Biological reconstruction of pelvic ring with autologous or homologous bone graft gives acceptable functional results. However, the decision on how to reconstruct the pelvic ring after iliac resection should be taken on a patient-by-patient basis.

The sacral chordoma margin.

OBJECTIVE: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. BACKGROUND: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. METHODS: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. RESULTS: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. CONCLUSION: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients.

Vascularized fibular autograft as salvage technique in failure of allograft intercalary reconstructions after tumor resections.

Intercalary allografts after diaphyseal resections for bone tumors represent the most frequent option of reconstruction. Main complications are non-unions, fractures and infections. The purpose of the current study was to report our experience with the use of vascularized fibular autograft as rescue technique in failed previous reconstructions after intercalary bone tumor resection of the extremities. Twenty-eight patients were followed over time. Causes of failure were non-union, allograft fracture and infection. Vascularized fibular autograft was used with mechanical support of massive bone allograft in 13 cases. Functional results were excellent in 19 cases, good in 8 and fair in one patient. Among complications we reported 4 non-unions, 2 allograft fractures, 1 non-union with plate breakage, 1 plate breakage, 1 infection, 1 limb shortening and 1 knee varus deformity. The rationale of vascularized fibular autograft is to provide biologic support. The association with massive bone allograft provides mechanical strength and early stability.

Humeral metastasis of renal cancer: Surgical options and review of literature.

INTRODUCTION: The humerus is the second most common long bone site of metastatic disease from renal cell carcinomas (RCC) after femur. Surgery has an important role in the treatment of these lesions due to renal cell tumor's resistance to chemotherapy and radiotherapy. HYPOTHESIS: Prosthetic replacement is an effective and safe solution in treatment of renal humeral metastasis. MATERIAL AND METHODS: Fifty-six patients affected by RCC bone metastases of the humerus that underwent a surgical reconstruction were rewised. Thirty-five lesions were localized on proximal third, 12 on the shaft, 9 on distal third. Among proximal 29 were treated with resection and endoprosthetic replacement and 6 with plate and cement. Six diaphyseal lesions were stabilized with intramedullary nailing, 5 with plate and cement and 1 with an intercalary prosthesis. Regarding distal lesions, 7 elbow prostheses and 2 plates and cement were used. RESULTS: The average age was 63years. Metastasis was single in 55% of cases, and in 45% metachronous. A pathologic fracture (PF) occurred in 64% of cases. Only 9% of patients had a mechanical complication, 7% an infection and 5% neurological deficit. A local recurrence occurred in 14% of patients. An implant failure has been observed in 10 patients, 5 for mechanical complications, 2 for infections and 3 for local recurrence; of these 7 were treated with a prosthesis and 3 with plate and cement. The mean value of MSTS score was 64%, 63% and 59% respectively in patients with proximal, diaphyseal and distal humerus metastases. DISCUSSION: Solitary and metachronous bone metastases have a longer survival. Disease-free interval>2years is another important prognostic factor. Reconstruction with a modular prosthesis is recommended in proximal and distal third. Instead in diaphyseal lesions a closed reduction and fixation with intramedullary locked nailing are preferred. When surgical indications are correctly followed, good oncologic and functional outcomes are obtained, leading to markedly improvement of patients' quality of life. RETROSPECTIVE STUDY: Level of evidence: IV.

Angiosarcoma around total hip arthroplasty: case series and review of the literature.

BACKGROUND: Angiosarcoma (AS) is a rare and malignant tumor which mainly arises in the skin and superficial soft tissue and less frequently in deep soft tissue and bones. Some cases of AS are described in association with vascular and orthopedic devices. Nonetheless, only a few cases of AS around THA are reported in the literature. MATERIALS AND METHODS: We describe five cases of AS arising around total hip arthroplasty who received surgery at our institution (Istituto Ortopedico Rizzoli, Bologna, Italy), and we report the cases described in literature. RESULTS: Foreign bodies such as polyethylene were demonstrated to have a carcinogenic role in animals, but reports of similar cases in humans are rare. Nevertheless, osteolysis induced by wear particles of polyethylene is a frequent event and could induce to desist form considering other more rare causes of osteolysis such as AS. This could be the reason why the diagnosis in several cases was significantly delayed. Common features of these cases could be helpful for doing a prompt diagnosis. The initial presentation is suggestive for septic or aseptic loosening with a massive osteolysis around the cup and/or the stem associated with peculiar aspects as bleeding and loss of weight. Frequently, needle biopsy is negative because foreign-body reaction might have "covered" the most relevant condition of epithelioid AS. CONCLUSIONS: In conclusion in a patient who presents with uncontrollable bleeding, loss of weight and massive osteolysis, AS must be actually considered as possible diagnosis.

Histology and grading are important prognostic factors in synovial sarcoma.

INTRODUCTION: The diagnosis of synovial sarcoma (SS) is currently based on clinical, morphological, immunohistochemical and cytogenetic data. Some of these factors such as grade and histology, specific translocations (SS18-SSX1 vs. SS18-SSX2) and the reduced expression of INI1, were proposed as prognostic variables. The aim of this study was to verify whether histological (grading and histology) and molecular (type of SSX translocation and INI1 expression) characteristics of SS influence the prognosis of the disease. MATERIAL AND METHODS: We retrospectively evaluated 196 patients affected by SS of the extremities treated at our Institution (Istituto Ortopedico Rizzoli, Bologna, Italy). All cases were histologically revised and tumor grade was assessed according to the FNLCC system. Tissue specimens were retrospectively evaluated to check for SS18-SSX fusion type and INI1 expression. RESULTS: Most SS were monophasic, 28% were biphasic. Eighty tumors (41%) were grade 3. Sixty percent harbored SSX1 translocation, 40% SSX2; 51% maintained the expression of INI1. Sarcoma specific survival (OS) was 56.6% at 5 years and 46.9% at 10 years. Prognosis was worse in those patients monophasic SS (p = 0.011) as in those with a grade 3 tumors (p = 0.083). No correlation was found neither between SSX fusion type nor INI1 expression and survival. LR-free survival was 78.9% at 5 years and 75.9% at 10 years. A higher LR rate was observed in tumors with SSX2 translocation and (p = 0.049) in grade 3 SS (0 = 0.028). DISCUSSION: Our data confirm that not all cases of SS present the same severe outcome. High-risk patients identified on the basis of these parameters may qualify for an aggressive treatment approach.

Dedifferentiated chondrosarcoma with "adamantinoma-like" features: A case report and review of literature.

Dedifferentiated chondrosarcoma is defined by the presence of a low grade malignant cartilaginous component juxtaposed to a high grade malignant non-cartilaginous sarcomatous components. Only 4 cases in which the high grade component showed epithelial differentiation have been reported in the literature; three featured a squamous and the one a glandular epithelial component. Here we describe a case of dedifferentiated chondrosarcoma exhibiting epithelial "adamantinoma-like" basaloid features. The patient underwent wide resection of the proximal tibia and post-operative chemotherapy and died 8 months after the diagnosis due to lung and bone metastases.

Impact of residual disease after "unplanned excision" of primary localized adult soft tissue sarcoma of the extremities: evaluation of 452 cases at a single Institution.

BACKGROUND: Soft tissue sarcomas are often inappropriately excised; it is, however, still a matter of debate whether the presence of residual disease in the re-excision specimen can affect patients' prognosis. The aim of this study is to investigate the impact of re-excision after unplanned surgery of primary soft tissue sarcomas (STS) of the extremities. PATIENTS AND METHODS: We retrospectively evaluated 452 adults with grade 2-3, localized STS (349 primary and 103 unplanned excisions). RESULTS: In the re-excision group, a full 43% of the patients had residual tumor. The re-excision group achieved a significantly better outcome in terms of sarcoma-specific survival (SS) (p = 0.002), local recurrence (LR) (p = 0.004) and distant metastasis (DM) (p = 0.028). Residual tumor was associated with a higher risk of DM (p = 0.005). CONCLUSION: We confirm that unplanned surgery does not compromise patients' prognosis; scar re-excision guarantees at least the same SS, LR and DM rates compared to STS primarily treated in a referral center. Routine use of radiation therapy after re-excision could improve local control. Distant metastases seem to be negatively affected by the presence of residual tumor, and therefore, the use of CT in deep and large STS is suggested. The main goal is to avoid unplanned surgery by referring suspected lumps (especially deep, large, increasing in size) to a specialist center.

Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients.

PURPOSE: Prognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ESOS prognosis. PATIENTS AND METHODS: Members of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS. RESULTS: Data from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40-54%). Five-year OS for metastatic patients was 27% (95% CI 13-41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44-59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35-51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis. At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival. CONCLUSION: Patient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin.

Elastofibroma of the gluteal region with a concomitant contralateral lesion: case report and review of the literature.

A 51-year-old man presented with elastofibroma (EF) of the gluteal region with a concomitant contralateral lesion. The patient presented with a slow growing mass of the proximal third of the right buttock and had swelling, discomfort in sitting, and right-hip pain during walking for 2 months. On MRI, a soft-tissue mass was noted between the gluteus maximus and the gluteus medius muscle. The mass showed similar signal intensity to the surrounding tissue on T1- and T2-weighted images and with linear hyperintense areas in its internal structure. At surgery, a soft, non-encapsulated, irregular, and rubber-like mass was found attached to the gluteus medius muscles. It was pathologically confirmed to be an EF. This unusual manifestation of an EF is discussed.

When size matters: differences in demineralized bone matrix particles affect collagen structure, mesenchymal stem cell behavior, and osteogenic potential.

Demineralized bone matrix (DBM) is a natural, collagen-based, osteoinductive biomaterial. Nevertheless, there are conflicting reports on the efficacy of this product. The purpose of this study was to evaluate whether DBM collagen structure is affected by particle size and can influence DBM cytocompatibility and osteoinductivity. Sheep cortical bone was ground and particles were divided in three fractions with different sizes, defined as large (L, 1-2 mm), medium (M, 0.5-1 mm), and small (S, <0.5 mm). After demineralization, the chemical-physical analysis clearly showed a particle size-dependent alteration in collagen structure, with DBM-M being altered but not as much as DBM-S. DBM-M displayed a preferable trend in almost all biological characteristics tested, although all DBM particles revealed an optimal cytocompatibility. Subcutaneous implantation of DBM particles into immunocompromised mice resulted in bone induction only for DBM-M. When sheep MSC were seeded onto particles before implantation, all DBM particles were able to induce new bone formation with the best incidence for DBM-M and DBM-S. In conclusion, the collagen alteration in DBM-M is likely the best condition to promote bone induction in vivo. Furthermore, the choice of 0.5-1 mm particles may enable to obtain more efficient and consistent results among different research groups in bone tissue-engineering applications. © 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 105A: 1019-1033, 2017.

Short, full-dose adjuvant chemotherapy (CT) in high-risk adult soft tissue sarcomas (STS): long-term follow-up of a randomized clinical trial from the Italian Sarcoma Group and the Spanish Sarcoma Group.

BACKGROUND: To report on long-term results of a phase 3 trial comparing three versus five cycles of adjuvant chemotherapy (CT) with full-dose epirubicin+ifosfamide in high-risk soft tissue sarcomas (STS). METHODS: Patients (pts) were randomized to receive three preoperative cycles of epirubicin 120 mg/m2 and ifosfamide 9 g/m2 (Arm A) or to receive the same three preoperative cycles plus two postoperative cycles (Arm B). Radiotherapy could be either delivered in the preoperative or in the postoperative setting. Non-inferiority of the primary end point, OS, was assessed by the confidence interval of the hazard ratio (HR; Arm A/Arm B) derived from Cox model. RESULTS: Between January 2002 and April 2007, 164 pts were assigned to arm A and 164 to arm B. At a median follow-up (FU) of 117 months (IQ range 103-135 months), 123 deaths were recorded: 58 in Arm A and 65 in Arm B. Ten-year OS was 61% for the entire group of patients: 64% in Arm A and 59% in Arm B. The intention-to-treat analysis confirmed that three cycles were not inferior to five cycles (one-sided 95% upper confidence limit was 1.24). A per protocol analysis was consistent with these results. Pts with leiomyosarcoma and undifferentiated pleomorphic sarcoma (UPS) had the lowest, and the highest response rates, respectively. Consistently, Leiomyosarcoma and UPS had the worse and the best prognosis, respectively. CONCLUSIONS: At a longer FU, the non-inferiority of three cycles of a full-dose conventional CT in comparison to five is confirmed. Response to therapy is also confirmed to be associated with better survival. This regimen is currently tested within an ongoing international trial against three cycles of a neoadjuvant histology-tailored CT (ClinicalTrials.gov Identifier: NCT01710176).

Surgical margins do not affect prognosis in high grade myxofibrosarcoma.

INTRODUCTION: Myxofibrosarcoma (MFS) is one of the most common soft tissue sarcomas (STS) in elderly patients and it primarily affects the extremities. The aim of this retrospective analysis is to understand the natural history of MFS and whether adequate treatment influence prognosis. PATIENTS AND METHODS: We reviewed 129 adult patients with primary, localized, FNCLCC grade 3 MFS of the extremities operated at Istituto Ortopedico Rizzoli, Bologna. Sarcoma specific survival (SS), local recurrence (LR) and distant metastasis (DM) were analyzed. RESULTS: Among excised MFS (119), 106 (89.9%) had R0 margins, 13 (10.1%) R1 margins. No significant correlation between margins adequacy and tumor depth, location and size was found. Estimated SS was 73.2% at 5 years and 66.3% at 10 years, with a better SS in superficial MFS (p = 0.011). Local recurred MFS had a worse SS (p = 0.049). Local recurrence-free rate was 74.3% at 5 and 10 years. Even if not significant, a better outcome in term of LR was observed in superficial MFS and R0 margins. Distant metastasis-free survival was 75.6% at 5 years and 72.9% at 10 years, with a better outcome in superficial MFS (p = 0.012). DISCUSSION: Myxofibrosarcoma remain a debated entity with specific behavior features. Myxofibrosarcoma tends to local recur due to its infiltrative grow pattern making difficult to achieve "safe margins". To date, surgical margins as classified for other STS are not predictive of LR and patients' survival. Tumor grade and depth are still the most important prognostic factors.

Total elbow arthroplasty for primary and metastatic tumor.

BACKGROUND: Prostheses can be used in elbow reconstruction in both primary and metastatic lesions. Several authors have reported their experience with different types of implant, but not with modular prostheses. HYPOTHESIS: Limb salvage using an elbow prosthesis is effective in obtaining good functional results and reliable local tumor control. MATERIAL AND METHODS: Forty-seven patients treated at the Rizzoli Institute for elbow neoplasm from 1990 to 2012 were evaluated. There were 30 primary tumors (64%), 24 bone tumors and 6 soft tissue sarcomas, and 17 bone metastases. Elbow reconstruction used a modular prosthesis in 25 patients and a standard prosthesis in 22. Reconstruction was primary in 30 patients and secondary in 17. RESULTS: At last control, 15 (32%) were dead of disease (DOD) at a mean follow-up of 35 months, 12 (25%) were alive with disease (AWD) at a mean follow-up of 29 months, 19 (40%) showed no evidence of disease (NED) at a mean follow-up of 80 months. Early complications were related to unexpected neurological damage, observed in 12 patients (25%): in 5 cases the deficit resolved in a mean 6 months; in the others, no or only partial recovery was observed. Two implants (4%) developed infection: 1 was treated with antibiotic therapy, and the other required implant revision. One implant showing cement extrusion was revised. In 3 patients (6%) radiography showed a radiolucent halo around the stem (2 humeral, 1 ulnar); no measures were taken, as the patients were completely asymptomatic at every follow-up. In 3 patients (6%) partial resorption of the allograft was observed on X-ray, but remained unchanged at last follow-up, without pain or functional impairment. Seven local recurrences (15%) were observed, at a mean of 16 months after surgery; 5 were treated by resection and/or radiotherapy, and 2 by amputation. Mean functional scores on MEPS and MSTS were respectively 84% and 22/30 (73%). CONCLUSIONS: Elbow prostheses provided better function in primary than in metastatic tumor. Elbow prosthesis reconstruction after tumor resection is a viable option both for primary and secondary bone neoplasms. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: IV, retrospective study.