Surgical Outcomes in Paediatric Lower Limb Sarcomas: A Single Institutional Experience.

Primary bone sarcomas are rare tumours, comprising approximately 1-2% of adult and around 13-15% of all paediatric cancers. Limb salvage surgery is a challenge in complex musculoskeletal tumours. Young age adds to its complexity, since the growth potential of the reconstruct has to be considered to have optimal function of the salvaged limb. An observational retrospective study performed on 52 paediatric patients between Jan 2011 and Dec 2018 with malignant bone tumour of lower limbs had been assessed for postoperative functional outcomes with questionnaires and clinico-radiological examination for functional and oncological outcome. Out of 52 patients, 44 (85%) were diagnosed with osteosarcoma and the rest (15%) were diagnosed with Ewing sarcoma. Mean age was 12.88 years. Three-year overall survival rate was found to be 61.73%. Fifteen patients (28%) had metastasis at the time of presentation; non-metastasis patients (56.33 months) had a higher mean survival time, as compared to metastasis patient (29.06 months). Overall survival in group of patients undergoing biological, prosthesis and amputation surgeries were found to be similar. Mean MSTS score at final follow-up was 24.05 (80.1%), and in our study, 3.85% (2 out of 52) had local recurrence, and local recurrence free rate was found to be 96.15% (50 out of 52). The present study had excellent functional and oncological outcome at final follow-up which is comparable to the world literature. Complete surgical resection, if feasible, remains essential for cure.

Functional Augmentation with the Use of a Mesh in a Traditional Cement Spacer Reconstruction in Proximal Humerus Resections.

Cement spacer as a reconstruction technique in proximal humerus resections in malignant and benign conditions is well established. Functional outcome of any reconstruction is decided by how well soft tissue reconstruction has been carried out. The cement spacer technique allows soft tissue reattachment at any site required as compared to prosthetic reconstruction, but such reattachment sites have to be decided before setting of the cement. Here we describe a technique in which a prolene mesh is used as a mould for the cement. The outer prolene mesh gives the surgeon the opportunity to anchor soft tissues during closure at any area desired. The mesh creates a porous surface over the stem which will aid in better healing by fibrosis for the muscle as compared to healing over smooth cement. Mesh fibrosis is another added point of stability at the bone-construct junction. Post-operative shoulder range of motion was easier to achieve in such patients.

A Review of Core Biopsies in Musculoskeletal Oncology.

Biopsy as a procedure is technically simple but conceptually a challenge. The principles of biopsy have remained more or less the same for 3 decades. With the advent of imaging, hospital facilities, and pathology modalities, we feel it is important that these principles are revisited. Uncertainties are spread across the literature regarding this very important first step in the care of an oncology patient. A surgeon planning the biopsy should be equipped with an MRI for planning. A biopsy has to be considered even for a benign-looking lesion. Even though literature is not clear on what kind of biopsy a surgeon should undertake, it is, without doubt, core biopsy has the edge that fine needle biopsies fail to reproduce. Image guidance for a biopsy is paramount for the accuracy of the sample. Pre-biopsy embolization has to be an option in the toolbox of a surgeon. Biopsy samples from an open biopsy or expensive or difficult interventional radiologist-assisted biopsy should undergo a rapid assessment to reduce the error of sample collection and thereby avoid subjecting the patient to a repeat procedure. Even though clean procedures do not require antibiotic prophylaxis, in view of major endoprosthetic implantations in the future, antibiotic use may be justified. An appropriate biopsy set and methodology as per institution experience are described in the paper.

Outcome of re-operation for local recurrence following pre-operative denosumab administration and curettage for giant cell tumour of bone with difficult joint preservation.

PURPOSE: Denosumab enables joint-sparing surgery (curettage) and surgical downstaging in patients with giant cell tumour of bone (GCTB), where joint preservation is not possible. However, denosumab treatment causes osteosclerosis of the lesion, making it difficult to curet the lesion, leaving the tumour behind, and increasing the local recurrence rate. We performed a three-centre retrospective study to investigate the postoperative local re-recurrence rate, joint preservation status, and functional outcomes of locally recurrent lesions after preoperative denosumab treatment and curettage in patients with difficult joint preservation. METHODS: We included 38 of 142 patients with primary GCTB of the extremities who underwent preoperative denosumab and curettage between 2009 and 2021 with local recurrence. Preoperative denosumab was indicated in patients with minimal residual periarticular and subchondral bones, large extraosseous lesions (Campanacci stage 3), and pathological fractures that made joint preservation difficult. RESULTS: Local re-recurrence occurred in 6 (15.8%) of the 38 patients. In 29 patients who underwent re-curettage, local re-recurrence occurred in six patients (20.7%); however, in nine patients who underwent en bloc resection, no local re-recurrence was observed. The joint preservation rate was 63.2% (24 of 38 patients), with a median Musculoskeletal Tumor Society score of 28 (interquartile range: 26.8-29.0). The median follow-up period after surgery for local recurrence was 63.5 months (interquartile range: 42.5-82.4). CONCLUSION: Since the local re-recurrence rate after re-curettage for local recurrence was low, and the joint preservation rate and affected limb function were good, preoperative denosumab administration may be considered in patients who require downstaging to maintain good limb function (joint preservation).

Outcome of Reoperation for Local Recurrence Following En Bloc Resection for Bone Giant Cell Tumor of the Extremity.

En bloc resection is typically performed to treat giant cell tumors of bone (GCTB), particularly when curettage can be challenging owing to extensive bone cortex destruction with soft tissue extension. Few reports have addressed the clinical outcomes after reoperation for local recurrence in patients with GCTB who underwent en bloc resection. In this multicenter retrospective study, we investigated local recurrence, distant metastasis, malignant transformation, mortality, and limb function in patients treated for local recurrence following en bloc resection for GCTB. Among 205 patients who underwent en bloc resection for GCTB of the extremities between 1980 and 2021, we included 29 with local recurrence. En bloc resection was performed for large tumors with soft tissue extension, pathological fractures with joint invasion, complex fractures, and dispensable bones, such as the proximal fibula and distal ulna. Local re-recurrence, distant metastasis, malignant transformation, and mortality rates were 41.4% (12/29), 34.5% (10/29), 6.9% (2/29), and 6.9% (2/29), respectively. The median Musculoskeletal Tumor Society score was 26 (interquartile range, 23-28). The median follow-up period after surgery for local recurrence was 70.1 months (interquartile range, 40.5-123.8 months). Local recurrence following en bloc resection for GCTB could indicate an aggressive GCTB, necessitating careful follow-up.

Hemiarthroplasty of Pedicle-Frozen Proximal Tibia for Primary Malignant Bone Tumours.

The proximal tibia is one of the most common locations for primary malignant bone tumours and is usually seen in a population that has not attained skeletal maturity. Most of the surgical techniques for resection and reconstruction of the proximal tibia in children involves sacrificing the distal femoral epiphysis, which leads to a significant limb length discrepancy. Hemiarthroplasty is a relatively lesser known technique for knee joint reconstruction that preserves one of the epiphyses around the knee joint. Pedicle freezing is a sterilization technique used to treat malignant bone tumours, without performing an osteotomy of the diaphysis, thereby preventing non-union which is the most common complication of biological reconstruction techniques. We describe the surgical technique of hemiarthroplasty of the pedicle-frozen proximal tibia for malignant bone tumours of the proximal tibia. This is a novel, safe and effective reconstructive method in children, resulting in reduced limb length discrepancy and excellent functional outcomes. Supplementary Information: The online version contains supplementary material available at 10.1007/s43465-022-00658-9.

Angiosarcoma in Chronic Filarial Lymphedema - A Report of Two Cases.

Introduction: Cutaneous angiosarcoma is an uncommon aggressive tumor known to arise in chronic lymphedema of various etiologies with post mastectomy lymphedema being the most common cause also known as Stewart-Treves syndrome. Chronic filarial lymphedema is the most uncommon etiology for development of angiosarcoma. Case Report: We describe two cases of angiosarcoma developing in a setting of long-standing filarial lymphedema. The patient presented with the same clinical features of rapidly progressing nodules. Conclusion: With India harboring most of the filarial load, this scenario should be kept in mind by clinicians for early diagnosis and treatment. Angiosarcoma is a rapidly progressing tumor and is fatal; hence, an idea about its diagnosis is of utmost importance.

Recurrence of GCTB of Wrist in an Autografted Non-vascularized Fibula: A Case Report.

Introduction: Distal radius is known to have high percentage of recurrences in giant cell tumor (GCT) despite best efforts. We would like to present a case where the recurrence unusually occurred in the graft and the complications associated with it. Case Report: A 45-year-old woman, who had previously undergone curettage of GCT distal radius, presented with recurrence of lesion which was managed initially with resection and non-vascularized fibular autograft reconstruction. Tumor again recurred in the autografted fibula which was managed by curettage and cementing. Due to progressive collapse of the carpus, resection of the autograft and wrist arthrodesis was carried out. Conclusion: Recurrence of GCT is a challenging problem. Wide resections may not always avoid recurrences. Patients should be made aware of the extend of recurrence that can happen in spite of the best efforts.

Long-Term Follow-up in a High-Grade Sarcoma of the Arm Encasing the Radial Nerve Treated by ISP Technique - A Case Report.

Introduction: A combined technique of In situ preparation (ISP) and adjuvant radiotherapy in a case of undifferentiated pleomorphic sarcoma (UPS) encasing the radial nerve was attempted successfully, which can be considered for the preservation of neurovascular structure close to soft-tissue sarcomas for a good functional and oncological outcome. Case Presentation: A 41-year-old female diagnosed with UPS of the left arm underwent, en bloc excision of the lesion along with the preservation of encased radial nerve using ISP followed by adjuvant radiotherapy. The patient has good functional outcome and a local recurrence free and overall survival of 5 years. Conclusion: We reported a case of UPS encasing the left radial nerve, where ISP technique and adjuvant radiotherapy were successfully attempted for attaining a good functional and oncological outcome.

A Report of a Rare Case of Multiple Fibro adipose Venous Anomaly.

Introduction: Although not described more than a decade ago, fibro adipose vascular anomaly has grown of increasing importance as conventional management of arterio venous malformation with interventional radiology measures carry little success and cause significant morbidity in pediatric age groups like the case report we present here. Surgical resection, even though requiring significant loss of muscle bulk, is the mainstay of the treatment. Case Report: An 11-year-old patient presented with equinus deformity and intensely tender calf and foot swellings in the right leg. Magnetic resonance imaging showed presence of two distinct lesions, one involving the gastrocnemius and soleus, and the other in the Achilles tendon for which en bloc resection of the tumor was performed. Histopathology of the specimens confirmed the diagnosis of fibro adipose venous anomaly. Conclusion: As per our knowledge, this is the first case of multiple fibro adipose venous anomaly confirmed by clinical features, radiology, and histopathology.

What Is the Survival of the Telescope Allograft Technique to Augment a Short Proximal Femur Segment in Children After Resection and Distal Femur Endoprosthesis Reconstruction for a Bone Sarcoma?

BACKGROUND: Large, malignant bone tumors and revision limb salvage procedures often result in the resection of extensive lengths of the involved bone segment, leaving a residual segment of bone that may be too short to support a standard intramedullary stem for endoprosthetic reconstruction. Telescope allografting, in which an allograft is used to augment the remaining bone segment by telescoping it into the residual bone segment, was described for situations in which residual bone stock is insufficient after tumor resection or prosthetic revision. Apart from one study that first described the procedure [15], there are no other studies reporting the outcome of this telescopic concept for restoring bone stock. QUESTIONS/PURPOSES: For patients younger than 18 years who underwent the telescopic allograft technique to augment a short segment of the proximal femur after resection of bone sarcomas who also underwent endoprosthesis reconstruction of the distal femur, we asked: (1) What is the survivorship free from removal of the telescopic allograft and the endoprosthetic stem at 7 years after surgery? (2) What proportion of these reconstructions will heal to the host bone without delayed union or nonunion? (3) What is the functional outcome based on the Musculoskeletal Tumor Society (MSTS) score? METHODS: We retrospectively studied our institutional database and identified 127 patients younger than 18 years who underwent surgery for a primary malignant bone tumor of the distal femur between December 2008 and October 2018. After excluding 16 patients undergoing amputation and rotationplasty and 57 patients undergoing recycled autograft/allograft reconstruction, 54 patients who underwent primary or revision distal femur endoprosthesis reconstruction were identified. Among these patients, we considered 15 patients who underwent telescopic allograft augmentation of the femur for analysis. One patient was lost to follow-up before 2 years but was not known to have died, leaving 14 for analysis at a median (range) 49 months (24 to 136 months) of follow-up. The indications for telescopic allograft augmentation of the femur in our institution were a proximal femur length of less than 120 mm after resection or resection of more than two-thirds of the total length of the femur. Ten of 14 patients underwent telescopic allograft augmentation as a revision procedure (distal femur resorption in five patients, endoprosthesis stem loosening in three patients, implant fracture in one patient, and infection in one patient), and the remaining four patients underwent telescopic allograft augmentation as a primary limb salvage procedure for large malignant bone tumors of the distal femur. The histologic diagnosis in all patients was osteosarcoma. At the time of telescopic allograft augmentation and reconstruction, the median age of the patients was 14 years (7 to 18 years). The size and the type of bone allograft to be used (femoral shaft or proximal femur) was planned before surgery, with consideration of the extent of resection, level of osteotomy, diameter of the host bone at the osteotomy site, and approximate diameter of the endoprosthesis stem to be used. The segment of the cylindrical allograft used for telescoping was thoroughly washed, prepared, and impacted onto the native femur to achieve telescoping and overlap. Serial digital radiographs were performed once a month for the first 6 months after the procedure, every 2 months until 1 year, and then every 6 months thereafter. Two surgeons in the department (at least one of which was involved in the surgery) retrieved and reviewed clinical notes and radiographs to determine the status of the telescopic allograft and endoprosthesis stem. We defined delayed union as radiological union at the osteotomy site more than 6 months after the procedure without additional surgery; we defined nonunion as no radiological evidence of callus formation at the osteotomy site 9 months after the procedure, necessitating additional surgery. The reviewers did not disagree about the definition of healing time. None of the patients missed radiographic follow-up. Kaplan-Meier survivorship free from removal of telescopic allograft and the endoprosthesis stem at 7 years after surgery was estimated. Patient function was assessed using the 1993 version of the MSTS [9], as determined by chart review of the institutional database performed by one of the surgeons from the department. RESULTS: The survivorship free from removal of the telescopic allograft and endoprosthesis stem at 7 years after surgery was 80% (95% confidence interval 22% to 96%). The allograft united with the host bone in 100% (14 of 14) of the patients. Though 21% (3 of 14) had delayed union, no nonunions were seen. The median (range) MSTS score at the final follow-up interval was 27 (22 to 30). CONCLUSION: Although this is a small group of patients, we believe that allograft segments help augment short bone stock of the proximal femur after long-segment resections, and the telescopic technique seems to be associated with a low proportion of nonunion or delayed union, which is one of the most common complications of allografts. Maintaining an adequate length of the proximal femur is important in preserving the hip, and this technique may be especially useful for young individuals who may undergo repeated revision procedures. LEVEL OF EVIDENCE: Level IV, therapeutic study.

What Are the Challenges and Complications of Sterilizing Autografts with Liquid Nitrogen for Malignant Bone Tumors? A Preliminary Report.

BACKGROUND: Reconstruction of defects after resection of malignant bone tumors with liquid nitrogen-sterilized recycled autografts is an alternative to bone allografts and endoprostheses in resource-constrained environments. Most studies reporting favorable outcomes with liquid nitrogen-sterilized autografts for bone reconstruction are geographically restricted to a few countries, and the technical challenges of routinely using liquid nitrogen intraoperatively, especially when using the pedicle freezing technique, has not been documented. QUESTIONS/PURPOSES: (1) What are the technical challenges of liquid nitrogen sterilization of bone tumors for inexperienced surgeons? (2) What are the complications associated with the procedure? METHODS: Between May 2017 and October 2019, 88 patients underwent limb salvage procedures for malignant bone tumors of the extremities at our institution. An endoprosthesis was used for reconstruction of the defect following resection in 45% (40 of 88) of these patients, mostly in adults (median age 21 years; range 9 to 68). In the remaining 55% (48 of 88) of patients undergoing biological reconstruction, liquid nitrogen-sterilized autograft was used in 90% (43 of 48), extracorporeal irradiation-sterilized autograft was used in 4% (2 of 48) and allograft was used in 6% (3 of 48). Of the 43 patients receiving liquid nitrogen-sterilized autograft, 5% (2 of 43) were excluded due to loss to follow-up and the remaining 95% (41 of 43) were included for the analysis. Liquid nitrogen-sterilized autograft was the preferred method of reconstruction at our institution during the study period, unless the patient had an indication for prosthesis reconstruction; extracorporeal irradiation-sterilized autograft was used due to resource constraints with liquid nitrogen and allograft was used when patients insisted.All surgical procedures were performed by the same team of trained orthopaedic oncology surgeons. The medical records of the included 41 patients were retrieved using an institutional database search in this retrospective study, and all were used to ascertain technical challenges associated with the operations as well as early (within 3 weeks of the index procedure) and late complications (those occurring 3 weeks or more after surgery). The technical challenges were defined as follows: the quantity of liquid nitrogen to be used; arranging, storing and handling of liquid nitrogen in the operating room, type and size of the container to be used for sterilization, the positioning of the container during pedicle freezing, level of fibular osteotomy for pedicle freezing of tibia, soft tissue protection, limb rotation during pedicle freezing, managing tourniquet time, and any other intraoperative factors with the use of liquid nitrogen for sterilizing the autograft. As our experience with the technique gradually grew, the answers to the above-mentioned factors were determined. Considering the removal of autograft as the endpoint of interest, survival of the autograft was determined by Kaplan-Meier analysis.The median (range) patient age was 14 years (2 to 49), and 54% (22 of 41) were males. Osteosarcoma was the most common diagnosis (68%, [28 of 41]) followed by Ewing's sarcoma (20%, [8 of 41]). On presentation, 27% of patients (11 of 41) had radiological evidence of pulmonary metastasis. Tumors were seen frequently around the knee (39% [16 of 41] proximal tibia and 22% [9 of 41] distal femur). Before resection 85% (35 of 41) underwent neoadjuvant chemotherapy. Sixty-six percent (27 of 41) underwent pedicle-freezing and the remaining 34% (14 of 41) underwent free-freezing of the tumor segment of the bone. The median (range) duration of surgery was 280 minutes (210 to 510). The patients were followed up for a median (range) duration of 21 months (5 to 30); two patients were lost to follow-up. RESULTS: With gradual experience using liquid nitrogen-sterilization over time at our institution, we determined that the following factors helped us in performing liquid nitrogen-sterilization more efficiently. For every operation 15 L to 20 L of unsterilized liquid nitrogen was arranged, 1 or 2 days before the procedure, and stored in industrial-grade cryocylinders in the operating complex. During the procedure, the operating surgeons wore additional plastic aprons under the surgical gowns, surgical goggles, and rubber boots. The staff managing the liquid nitrogen in the operating room wore thermal protective gloves. For most of the pedicle freezing procedures, we used a cylindrical stainless-steel container that was 30 cm in height and 15 cm in diameter, with a narrow opening. The container was kept on a separate moveable cart that was placed next to the operating table at a slightly lower level, and it was wrapped in multiple cotton rolls, plastic sheets, surgical sheets, and a crepe bandage. For pedicle freezing of the tibia, we performed the fibular osteotomy at least 5 cm away from the planned surgical margin, roughly around the axis of rotation of the limb. The soft tissue at the base of the delivered bone segment was dissected for at least 5 cm beyond the planned surgical margin of bone, and was protected with multiple layers of cotton rolls, plastic drapes, a single roll of Esmarch and crepe bandage. The tumor segment was externally rotated during pedicle freezing for all anatomic sites (proximal tibia, distal tibia, proximal humerus, and proximal femur). The tourniquet was inflated just before pedicle freezing to prevent tumor dissemination and not before the initial incision in all pedicle freezing procedures.Thirty-nine percent of patients (16 of 41) experienced complications associated with the procedures, and 15% (6 of 41) underwent revision surgery. Early complications (occurring within 3 weeks of the index procedure) were skin necrosis in four of 16 patients, intraoperative fracture in one of 16, superficial infection in one of 16, and neurapraxia in one of 16 patients. Late complications (occurring 3 weeks or more after surgery) were resorption of the recycled bone in four of 16 patients, nonunion of the osteotomy site in two of 12, delayed union of the osteotomy site in one of 16, collapse of the recycled bone in one of 16, and local recurrence in 1 of 16 patients. Kaplan-Meier survivorship free from removal of autograft at 2 years after surgery was 92% (95% confidence interval 89 to 96). CONCLUSION: Liquid nitrogen-sterilization is an alternative technique that requires some training and experience for the surgeon to become proficient in treating primary malignant bone tumors. Because it is widely available, it may be an option worth exploring in resource-constrained environments, where allografts and endoprostheses cannot be procured. The methods we developed to address the technical challenges will require more study and experience, but we believe these observations will aid others who may wish to use and evaluate liquid nitrogen sterilization of extremity bone sarcomas. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Postoperative delirium in patients undergoing surgery for bone metastases.

Postoperative delirium (PD), characterized by acute onset of global impairment in consciousness and cognition, is a common complication following major surgeries and is often associated with adverse outcomes. Because of the multiple comorbidities of the patient along with extensive nature of the surgery, patients undergoing surgery for bone metastases may be prone to developing PD. However, no study exists regarding PD in patients who undergo surgery for bone metastases.Two hundred seventy six patients with mean age of 64 years (range, 16-94) who underwent surgery for bone metastases were reviewed. The diagnosis of PD was made by the psychiatrist, according to fourth edition of the Diagnostic and Statistical Manual of Mental Disorders. Possible perioperative clinic-pathologic factors that may be associated with the development of PD were investigated.Among the 276 patients, 9% (n = 25) developed PD. On multivariate logistic regression analysis, history of psychiatric disorders (odds ratio [OR] = 9.63; 95% confidence interval [CI] 1.78-21.74, P = .004), high preoperative serum C-reactive protein (CRP) level (OR = 1.17; 95% CI 1.06-1.29, P = .001), low preoperative serum albumin level (OR = 0.13; 95% CI 0.03-0.48, P = 0.002), and high dose of opioid analgesics received in the immediate postoperative period (OR = 1.05; 95% CI 1.01-1.07, P = .001) were independently associated with the development of PD. Patients with PD had lower survival (log rank, P = .001) than patients without PD.Incidence of PD is considerable in patients undergoing surgery for bone metastases. History of psychiatric disorders, preoperative serum albumin and CRP levels, and the dose of postoperative opioid analgesics are associated with the development of PD.

Is a Short-course of Preoperative Denosumab as Effective as Prolonged Therapy for Giant Cell Tumor of Bone?

BACKGROUND: Denosumab is an inhibitor of monoclonal receptor activator of nuclear factor-ĸB ligand, approved to treat giant cell tumors of bone (GCTB). It is commonly used for unresectable tumors and for downstaging the tumor to perform less-morbid procedures. Although denosumab has been used extensively for GCTBs, there are no recommendations regarding the duration of therapy. The risk factors associated with local recurrence (LR) in patients receiving preoperative denosumab for GCTB also are unknown. QUESTIONS/PURPOSES: (1) Is short-course (three doses or fewer) preoperative denosumab treatment as effective as longer course (more than three doses) of treatment in terms of achieving a clinical, radiologic, and histologic response in patients with GCTB? (2) Is there an increased risk of LR after short-course denosumab therapy compared with long-course denosumab therapy; and after controlling for confounding variables, what factors were associated with LR after surgery for GCTB in patients receiving preoperative denosumab? METHODS: A retrospective study was performed using an institutional database of 161 skeletally mature patients with a histologic diagnosis of GCTB who received denosumab between November 2010 and July 2019 to downstage the tumor before surgery. In general, we used denosumab when we thought it would facilitate either resection or curettage (by formation of a sclerotic rim around the osteolytic lesion), when a less-morbid procedure than initially planned might be performed, and in patients with complex presentations like cortical breech and soft tissue extension, pathological fracture, thinning of more than three cortices of the extremity. From 2010 to late 2015, denosumab was administered for approximately 4 to 6 months; starting in late 2015 through 2020, the number of denosumab doses has been reduced. We divided patients into two groups: Those who received three or fewer doses of denosumab (short-course, n = 98) and those who received more than three doses of denosumab (long-course, n = 63). Comparing those in the long-course group with those in the short-course group whose procedures were performed at least 2 years ago, there were no differences in loss to follow-up before 2 years (3% [3 of 98] versus. 3% [2 of 63]). The mean patient age was 30 years (± 6.1) and the mean number of denosumab doses was 4.4 (range 1 to 14). Overall, 77% (37 of 48) of patients taking short-course denosumab and 75% (27 of 36) of patients on long-course denosumab underwent curettage, and the remaining patients with an inadequate bony shell around the tumor or destruction of articular cartilage in both groups underwent tumor resection. With the numbers available, the patients with short- and long-course denosumab were not different in terms of age, sex, MSTS score on presentation, lesion size, lesion location, Campanacci grade, presence of pathological fracture and pulmonary metastasis on presentation, and the type of surgery performed (curettage versus resection). We analyzed the change in the Musculoskeletal Tumor Society score, change in Campanacci grade, radiologic objective tumor response (defined as a partial or complete response, per the modified inverse Choi criteria), and histologic response (defined as reduction of more than 90% of osteoclast-like giant cells or a reduction of more than 50% of mesenchymal spindle-like stromal cells, along with evidence of lamellar or woven bone formation, when compared with the biopsy sample) between the two groups (short- and long-course denosumab). LR rates were compared between the two groups, and after controlling for confounding variables, factors associated with LR in all operated patients were analyzed with a Cox proportional hazards regression analysis. RESULTS: With the numbers available, there was no difference between the short- and long-course denosumab groups in terms of mean percentage improvement in MSTS score (20 [± 18.5] versus 24 [± 12.6]; p = 0.37), radiologic objective tumor response (90% [43 of 48] versus 81% [29 of 36]; p = 0.24) and histologic response (79% [38 of 48] versus 83% [30 of 36]; p = 0.81). With the numbers available, there was no difference between the short- and long-course denosumab groups in terms of Kaplan-Meier survivorship free from LR at 5 years after surgery (73% [95% confidence interval, 68 to 76] versus 64% [95% CI 59 to 68]; log-rank p = 0.50). After controlling for potential confounding variables like age, sex, Campanacci grade and MSTS score on presentation, number of denosumab doses administered before surgery, clinical, radiologic and histologic response to denosumab, and time duration between denosumab therapy and surgery, we found that tumors involving the bones of the hand and the foot (hazard ratio 7.4 [95% CI 2.0 to 27.3]; p = 0.009) and curettage (HR 6.4 [95% CI 2.8 to 23.0]; p = 0.037) were independently associated with a higher risk of LR. CONCLUSIONS: In this preliminary, single-center study, we found that a short-course of preoperative denosumab (three or fewer doses) was associated with no differences in clinical scores, histological and radiological response, or LR-free survivorship, compared with longer-course of denosumab (more than three doses). Fewer preoperative doses can reduce the complications and costs associated with more-prolonged therapy. Denosumab must be used cautiously before curettage for GCTB, and only if the benefit of joint salvage outweighs the possibility of LR. However, given the small number of patients, potentially clinically important differences might have been missed, and so our findings need to be confirmed by larger, multicenter, prospective trials. LEVEL OF EVIDENCE: Level III, therapeutic study.

Evaluation of Local Recurrence in Giant-Cell Tumor of Bone Treated by Neoadjuvant Denosumab.

BACKGROUND: Giant-cell tumor of bone (GCTB) is a locally aggressive primary benign tumor presenting as an expansile osteolytic lesion affecting the epiphysis of long bones. Denosumab halts the osteolysis by giant cells thereby downstaging the tumor, helping in performing less morbid procedures to remove the tumor. Our aim was to report the incidence of local recurrence (LR) in patients operated following neoadjuvant denosumab, to investigate factors associated with LR following extended curettage for GCTB, and to compare the postoperative functional and oncological outcome of patients operated with and without neoadjuvant denosumab. METHODS: A total of 123 patients with a mean age of 29.6 years undergoing extended curettage for GCTB were retrospectively divided into group 1 receiving neoadjuvant denosumab and group 2 operated without denosumab. The mean follow-up period was 35 months. The perioperative characteristics and outcome were compared between the two groups and the factors for LR of GCTB were analyzed. RESULTS: The incidence of LR among patients operated after neoadjuvant denosumab therapy was 42.8% and was significantly high compared to that in patients without denosumab (p < 0.001). On multivariate logistic regression analysis, use of denosumab as a neoadjuvant was the only factor independently associated with LR following surgery (p = 0.002). Patients treated with denosumab had a lower LR-free survival rate (log-rank, p = 0.018). CONCLUSIONS: Denosumab was independently associated with increased LR following surgery for GCTB. Denosumab has to be used cautiously in patients in whom the burden of downstaging the disease outweighs the possible chance of LR.

Massive FungatingEwing's Sarcoma of Scapula Treated by Limb Salvage Surgery: A Case Report.

INTRODUCTION: Ewing's sarcoma (ES) is a malignant tumor of the bone and soft tissue arising from the neuroectodermal cells. It commonly arises from the extremities of children and young adults and very rarely from flat bones like the scapula. Locally advanced disease with prior history of surgery and poor response to neoadjuvant chemotherapy in a bedridden patient is usually treated by forequarter amputation, considering the anatomical complexity of the shoulder girdle. CASE REPORT: A young adult diagnosed and previously operated for ES of the left scapula, presented to us with a massive fun gating mass over the left upper back, excruciating pain and inability to use the left upper limb. Limb salvage surgery was performed, followed by adjuvant radiotherapy, chemotherapy, and autologous stem-cell transplantation. The patient is disease free with a functional limb on 3 years of follow-up. CONCLUSION: ES of scapula is very rare. With meticulous surgery and multidisciplinary treatment approach, limb salvage can be achieved even in such advanced cases, thereby prolonging survival and providing better quality of life.

Robotic Surgery Assisted Staged En-Bloc Sacrectomy for Sacral Chordoma: A Case Report.

CASE: Two male patients aged 37 years and 39 years, diagnosed with sacral chordoma, underwent robotic-assisted preparatory adhesiolysis from the anterior aspect of the tumor, followed by posterior en-bloc partial sacrectomy. The average total operative time was 360 minutes (anterior docking + anterior console + posterior excision), and mean blood loss was 930 mL. Both patients were mobilized early, had no postoperative complications, and were free of local recurrence at 18 month of follow-up. CONCLUSIONS: Robotic-assisted surgery is a novel, valid, safe, and minimally invasive technique which drastically reduces the associated surgical complications of single-staged posterior sacrectomy, resulting in excellent functional and oncological outcome.