Management of Chondroblastoma in Pediatric Patients: 21 Years of Single-Center Experience.

BACKGROUND: Chondroblastoma (CB), a rare benign bone tumor that produces chondrocytes, often develops in the epiphysis or apophysis of children and young adults. The treatment of these rare tumors is complex. The standard treatment protocol involves curettage with local adjuvants and bone graft or cement application. The authors examined 38 CBs to determine risk factors for local recurrence, complications, and functional outcomes following epiphyseal curettage. METHODS: Twenty-two girls and sixteen boys aged 10 to 17 years with histologically confirmed chondroblastoma who arrived at our hospital between January 2000 and June 2021 were reviewed retrospectively. Clinical data, radiographic images, histological results, treatment, functional outcomes, and the local recurrence rate were examined-surgical treatment involved total tumor curettage, followed by bone grafting and adjuvant techniques. Local recurrences have also been reported. RESULTS: The most frequently affected site was the proximal femur. Sites of involvement included the proximal femur in 10 (26.3%) cases, the proximal tibia in 8 (20.8%), the humerus in 5 cases (13.2%), the distal tibia in 4 cases (10.5%), the distal femur in 3 cases (7.9%), the supracetabular region in 3 cases (7.9%), the talus in 1 case (2.6%), the calcaneus in 1 case (2.6%), the scapula in 1 case (2.6%), the lumbar spine in 1 case (2.6%), and the iliac bone in 1 (2.6%) patient. The mean follow-up was 144.2 months (24 to 276). The local recurrence rate was 7.9%. The mean Musculoskeletal Tumor Society (MSTS) score was 28.3 points (17 to 30). The mean duration of symptoms at presentation was 5.8 (range, 1 to 28) months. CONCLUSION: Aggressive curettage and bone grafting resulted in local control and good outcomes in most pediatric patients. In a relatively small proportion of cases, long-term complications and recurrence can occur due to growth plate damage and late diagnosis. In patients admitted to the pediatric clinic with pain, which is often accompanied by localized edema and joint effusion, early detection via advanced radiological scans (X-ray, CT, or MRI) may prevent delays in diagnosis.

Composite Reconstruction With Irradiated Autograft Plus Total Hip Replacement After Type II Pelvic Resections for Tumors Is Feasible but Fraught With Complications.

BACKGROUND: Malignancies involving the pelvic ring present numerous challenges, especially in the periacetabular area. Extensive resection of the pelvic region without reconstruction can lead to severe functional impairment. Numerous reconstructive options exist, but all have drawbacks. Extracorporeally irradiated autografts are one option for reconstruction after periacetabular resections; they offer the potential advantages of eliminating the risk of allogeneic reactions associated with allografts and preserving local anatomy. However, little is known about the durability and risks of this approach in pelvic reconstruction. QUESTIONS/PURPOSES: (1) What are the survival rates of the autograft used, and if there is graft loss, what is the extent of this loss? (2) What are the functional outcomes after the implementation of this method? (3) What is the rate and nature of complications associated with this approach? METHODS: This is a retrospective case series from one subspecialty tumor unit that evaluated patients treated between January 2005 to January 2022. During that time, three surgeons treated 48 patients with Type II resections (defined as resection of periacetabular area). Patients treated with isolated Type II resections were eligible, as were those treated either with Type I+II resections, Type II+III resections, Type I+II+III resections, and Type I+II+III+IV resections. Of those, 21% (10 of 48) were treated primarily with a cone prosthesis, 13% (6 of 48) were treated without femoral reconstruction, 10% (5 of 48) were treated with resection without reconstruction, and 6% (3 of 48) had a THA on the sacrum, leaving 50% (24 of 48) of patients who were treated with femoral and acetabular reconstruction using extracorporeally irradiated autograft and total hip replacement; those patients were potentially eligible for this study. During that time span, we used this approach in all Type II pelvic resection procedures, when a part of the hemipelvis could be preserved without resection (other than Type I+II+III+IV) and where we predicted that there would be sufficient bone stock after tumor removal. Of those, 21% (5 of 24) were lost to follow-up before 2 years, and 13% (3 of 24) died within 2 years with the reconstruction intact and without any reoperation or graft loss, leaving 67% (16 of 24) for analysis here. Demographic characteristics, type of tumor, tumor origin site, type of applied resection, and extent of applied surgical procedure were noted. Of 16 patients, 12 were male, with a mean age of 38 ± 21 years. Tumor types included chondrosarcoma in eight patients, malignant mesenchymal tumor in four patients, osteosarcoma in two patients, and Ewing sarcoma in two patients. Among these, 10 patients had tumors originating from the pelvis, whereas six patients had tumors originating from the proximal femur. We used a Kaplan-Meier estimator to calculate survivorship free from total or partial graft removal at 72 months. To measure functional results, the Musculoskeletal Tumor Society (MSTS) scoring system was utilized at most recent follow-up so as to be able to evaluate the impact of complications (if any) on the ultimate result. The MSTS score ranges from a minimum of 0 points to a maximum of 30 points, where a higher score reflects lower pain and higher functional and emotional capacity. Related complications, time of complications, secondary interventions, and mortality rates were also ascertained from chart review. RESULTS: Graft survival rate at 72 months after initial reconstruction, free from partial or total graft removal, was 50% (95% CI 26% to 75%). Kaplan-Meier analyses revealed estimated mean time of graft removal as 43 months (95% CI 28 to 58). The graft was protected in eight patients on their final follow-up radiographs. The median (range) MSTS score was 18 (6 to 25) of 30 points at most-recent follow-up (these scores include patients who have had their grafts removed). In all, 15 of 16 patients had 17 complications; 16 were major complications (defined as those substantial enough to result in further surgery or a life- or limb-threatening event). A total of 14 of those 15 patients underwent one or more secondary procedures (a total of 21 unplanned additional procedures were performed in those patients). Deep infection was the most common complication, occurring in eight patients. Prosthesis dislocation occurred in four patients. Three patients developed aseptic acetabular component loosening, two had graft fractures, and one patient developed heterotopic ossification. CONCLUSION: Composite reconstruction with extracorporeal irradiated autografts plus total hip replacement is a feasible reconstruction technique after Type II pelvic resections, although complications and reoperations were common. Although no reconstruction technique has been proven superior to other alternatives, the high risk of complications and reoperations associated with this technique should be considered when selecting from among possible alternative reconstruction methods. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Tumor size and surgical margins are important prognostic factors of synovial sarcoma - A retrospective study.

Background: Synovial Sarcoma (SS) is a rare soft tissue sarcoma. Mean time to get a SS diagnosis from the onset of symptoms is 10 years, furthermore, SS is associated with late metastasis. Surgery is the main treatment option, whose quality deeply affects SS outcomes, and it can be associated to preoperative or post-operative radiotherapy. Chemotherapy is considered very effective in Children, while in adults its efficacy is still under debate. The aim of this study was to investigate the oncologic results in SS treatment and to identify the risk factors for local and systemic control of the disease. Methods: From 1994 to 2018, 211 patients affected by SS were treated in 3 Referral Centres of Orthopaedic Oncology. One hundred seventy-seven patients were included in the study, the median follow-up length was 96 months (5-374). Results: Overall Survival on the Kaplan Meier Analyses was 80%, 70% and 56% at 5, 10 and 20 years. In multivariate analyses, OS correlated with tumour size and negative surgical margins. Chemotherapy use wasn't associated with better survival although patients who underwent CT had bigger and more aggressive tumours. Conclusions: Our findings suggests that surgery with negative margins is the most important factor in Synovial Sarcoma. Adjuvant treatments as chemotherapy and radiation therapy didn't change the disease's course.

What has changed in the last 25 years in osteosarcoma treatment? A single center experience.

BACKGROUND: Osteosarcoma is the most common type of primary malignant bone tumor in the extremities. The main purpose of this study was to determine clinical features, prognostic factors, and treatment results of patients with osteosarcoma at our center. METHODS: We retrospectively analyzed the medical records of children with osteosarcoma between the years 1994-2020. RESULTS: 79 patients were identified (54.4% male, 45.6% female). The most common primary site was the femur (62%). Twenty-six of them (32.9%) had lung metastasis at diagnosis. The patients were treated between 1995- 2013 according to the Mayo Pilot II Study protocol, while the others were treated with the EURAMOS protocol between the years 2013-2020. Sixty-nine patients underwent limb salvage surgery as a local treatment, whereas seven underwent amputation. The median follow-up time was 53 months (2.5-265 months). The event-free survival (EFS) and overall survival (OS) rates at 5 years were 52.1% and 61.5%. The 5-year EFS and OS rates were 69.4% and 80% in females; 37.1% and 45.5% in males (p=0.008/p=0.001). The 5-year EFS and OS rates of the patients without metastasis were 63.2% and 66.3%; with metastasis 28.8% and 51.8% (p=0.002/p=0.05). For good-responders, the 5-year EFS and OS rates were 80.2% and 89.1%; while for poor-responders, 35% and 46.7% (p=0.001). Mifamurtide was used in addition to chemotherapy as of the year 2016 (n=16). The 5-year EFS and OS rates were 78.8% and 91.7%, respectively for the mifamurtide group; 55.1% and 45.9%, respectively for the non-mifamurtide group (p=0.015, p=0.027). CONCLUSIONS: Metastasis at diagnosis and poor response to preoperative chemotherapy were the most important predictors of survival. Females had a better outcome than males. In our study group, the mifamurtide group`s survival rates were significantly higher. Further large studies are needed to validate the efficacy of mifamurtide.

Preoperative radiotherapy with concomitant chemotherapy in extremity soft tissue sarcomas: long-term results of a single center.

PURPOSE: To assess oncological outcomes of patients receiving neoadjuvant radiochemotherapy (RCT) for soft tissue sarcoma (STS) of the extremities. METHODS: Patients who were treated with preoperative radiotherapy and concomitant chemotherapy-3 cycles of mitomycin/doxorubicin/cisplatin (MAP) or 2-4 cycles of doxorubicin/cisplatin (AP)-followed by surgery were analyzed retrospectively. Survival rates were estimated, and prognostic factors were identified. RESULTS: Between 1994 and 2017, a total of 108 patients were included. Median ages were 43 years and 51 years for patients receiving MAP and AP, respectively. The 5­year local relapse-free survival (LRFS), disease-free survival (DFS), disease-specific survival (DSS), and overall survival (OS) were 94.1, 63.6, 75.3, and 71.9%, respectively. In the multivariate analysis, significant predictors were identified as follows: de novo or R1/R2 resected tumor on admission before RCT (p = 0.017; hazard ratio [HR] 0.112, 95% confidence interval [CI] 0.019-0.675) and R0 resection after RCT (p = 0.010; HR 0.121, 95% CI 0.024-0.598) for LRFS; female gender (p = 0.042; HR 0.569, 95% CI 0.330-0.979) and liposarcoma histology (p = 0.014; HR 0.436, 95% CI 0.224-0.845) for DFS; liposarcoma histology (p = 0.003; HR 0.114, 95% CI 0.027-0.478) and AP regimen (p = 0.017; HR 0.371, 95% CI 0.165-0.836) for DSS; age ≤ 45 years (p = 0.043; HR 0.537, 95% CI 0.294-0.980) and liposarcoma histology (p = 0.006; HR 0.318, 95% CI 0.141-0.716) for OS, respectively. CONCLUSION: An increased risk for local failure seems to exist for patients with relapsed tumor on admission and having positive surgical margins after neoadjuvant RCT. Intensity of chemotherapy influenced DSS but not OS, which could be due to younger patients receiving MAP.

Impact of the unplanned excision on the oncological outcomes of patients with soft tissue sarcomas: a single-center retrospective review of 490 patients.

OBJECTIVE: This study aimed to (1) compare the oncological results of patients who underwent re-excision after unplanned excision with those who underwent planned excision and (2) analyze the impact of local recurrences on oncological outcomes. METHODS: Patients with soft tissue sarcoma who had been treated in our center between 2000 and 2018 were retrospectively reviewed. Patients were divided into two groups: Group PE (Planned excision; n=345) and group UE (Unplanned excision; n=145). Two groups were compared in terms of local recurrence-free survival (LRFS), metastasis-free survival (MFS), and overall survival (OS). Local recurrences effects over MFS and OS were also analyzed. RESULTS: There were 26 (17.9%) local recurrences in the UE group and 30 (8.7%) local recurrences in the PE group (P=0.005). There was no difference in MFS and OS between study groups (P=0.278 and P=0.848, respectively). Five years MFS rates of UE and PE groups were 76.4% and 73.6%, and five-year OS rates of UE and PE groups were 70.3% and 73.9%, respectively (P=0.417, P=0.656). Patients with local recurrence had a 1.96 times higher risk of metastasis than patients without local recurrence (P=0.008). Patients with local recurrence had 1.65 times higher risk of mortality than patients without local recurrence (P=0.047). CONCLUSION: Although local recurrence is much more common in the UE group, this outcome does not seem to affect MFS or OS. These results indicate that similar outcomes can be achieved if UE patients are referred and appropriately treated with wide re-resections. LEVEL OF EVIDENCE: Level III, Therapeutic Study.

Is Hydatid Cyst with Musculoskeletal Involvement a Problem that Causes Morbidity? Long-Term Follow-Up and Functional Results.

Background: The aim of study is to evaluate the involvement characteristics of hydatid cysts, which are rarely involved the musculoskeletal system, and the results of recurrence, morbidity, and functional and mental scoring. Methods: We retrospectively investigated 18 patients with skeletal hydatid disease. Patients were categorized as those with bone or skeletal muscle involvement. Pre- and post-operative physical component scores (PCS) and mental component scores (MCS) on the functional Short Form 12-item Survey were recorded in these patients.We compared the functional scores, number of recurrences, and lesion volumes between patients with hydatid cyst of bone (HCOB) and those with hydatid cyst of soft tissue (HCOST). Results: This study included 11 women and 7 men with bone hydatid cysts. Patients' mean age was 38 years (range 22-70 years). Patients were followed up for a mean of 118.16 months (range 49-230 months). The mean lesion volume was 447.39 cm3 (36-1260 cm3). The 12th and 48th month PCS, the PCS was lower in the HCOB group during both periods (p = 0.04 and p = 0.001, respectively). The 48th month MCS was lower in the HCOB group (p = 0.04). Postoperative residual cysts were detected in five patients who underwent surgical treatment for bone c; all residual lesions were located in the pelvis. Conclusions: Bone hydatid cysts are associated with high recurrence rates, and treatment is challenging. In view of the low functional results and high infection and recurrence rates observed in patients over long-term follow-up, particularly in those with pelvic hydatid cysts, we conclude that this disease is associated with significant morbidity.

Life or limb: an international qualitative study on decision making in sarcoma surgery during the COVID-19 pandemic.

OBJECTIVES: The COVID-19 pandemic is unprecedented as a global crisis over the last century. How do specialist surgeons make decisions about patient care in these unprecedent times? DESIGN: Between April and May 2020, we conducted an international qualitative study. Sarcoma surgeons from diverse global settings participated in 60 min interviews exploring surgical decision making during COVID-19. Interview data were analysed using an inductive thematic analysis approach. SETTING: Participants represented public and private hospitals in 14 countries, in different phases of the first wave of the pandemic: Australia, Argentina, Canada, India, Italy, Japan, Nigeria, Singapore, Spain, South Africa, Switzerland, Turkey, UK and USA. PARTICIPANTS: From 22 invited sarcoma surgeons, 18 surgeons participated. Participants had an average of 19 years experience as a sarcoma surgeon. RESULTS: 17/18 participants described a decision they had made about patient care since the start of the pandemic that was unique to them, that is, without precedence. Common to 'unique' decisions about patient care was uncertainty about what was going on and what would happen in the future (theme 1: the context of uncertainty), the impact of the pandemic on resources or threat of the pandemic to overwhelm resources (theme 2: limited resources), perceived increased risk to self (theme 3: duty of care) and least-worst decision making, in which none of the options were perceived as ideal and participants settled on the least-worst option at that point in time (theme 4: least-worst decision making). CONCLUSIONS: In the context of rapidly changing standards of justice and beneficence in patient care, traditional decision-making frameworks may no longer apply. Based on the experiences of surgeons in this study, we describe a framework of least-worst decision making. This framework gives rise to actionable strategies that can support decision making in sarcoma and other specialised fields of surgery, both during the current crisis and beyond.

Comparison of the Effects of Extracorporeal Irradiation and Liquid Nitrogen on Nerve Recovery in a Rat Model.

AIM OF THE STUDY: Biologic reconstruction using tumor-bearing bone autografts devitalized by liquid nitrogen or extracorporeal irradiation (oncological sterilization) is a safe and effective method in musculoskeletal surgery. The purpose of this study was to examine the effects of these two oncological sterilization methods on nerve recovery. METHODS: A total of 48 rats were randomly divided into 3 groups as autograft, irradiation and liquid nitrogen groups. A nerve defect created in the right sciatic nerve was reconstructed with an autograft obtained from the nerve itself. Group I underwent reconstruction with standard nerve autograft. Group II and Group III underwent reconstruction with devitalized nerve autograft treated through extracorporeal irradiation and liquid nitrogen, respectively. The left sciatic nerves of the rats served as control. Electromyography, motor function test and histomorphological analysis were performed to assess the nerve recovery on the 3rd (early stage) and 4th months (late stage). RESULTS: Electrophysiological assessment revealed better results in irradiation group compared with liquid nitrogen group in terms of myelinization and axonal regeneration. Motor performance of the autograft group was slightly better than the other groups. Histologically, autograft group demonstrated better results compared with other groups. Late-stage assessments revealed high rates of myelinization in the graft segment in liquid nitrogen group and in the segment distal to the graft in irradiation group. CONCLUSIONS: This study has demonstrated that nerve autografts treated by oncological sterilization methods may be used for nerve reconstruction in limb salvage surgery. However, further studies are needed to clarify the applicability of these methods.

Treatment of pelvic Ewing's sarcoma: Pros and cons of chemotherapy plus definitive radiotherapy versus surgery.

OBJECTIVE: The aim of this study was to compare the results of chemotherapy or combined chemotherapy-radiation therapy with surgical intervention following neodjuvant therapy in pelvic Ewing's sarcoma patients. METHODS: The study population consisted of 39 patients with pelvic Ewing's sarcoma treated in our clinic between 1994 and 2014. Of these patients, 28 patients (11 boys and 17 girls; mean age: 19.57±6.8 years) were treated with chemotherapy and radiation therapy and the remaining 11 patients (9 boys and 2 girls; mean age: 18.64±8.1 years) patients underwent surgical intervention after neoadjuvant chemotherapy or chemotherapy plus radiation therapy. Internal hemipelvectomy was performed in 10 patients, and external hemipelvectomy was performed in one patient. Survival rates were compared between the surgical and non-surgical treatment groups. Predictive factors, such as treatment protocol (surgery, neoadjuvant chemotherapy, definitive radiotherapy), mass localisation, mass size, presence of metastasis at the time of diagnosis, and presence of late metastases were compared between the groups. The effects of each variable on survival were also examined. RESULTS: The overall 3- and 5-year survival rates of the 28 non-surgical patients were 41.4% and 26.1%, respectively, while those of the surgical patients were 53% and 35.4%, respectively (p=0.777). Large mass size, presence of metastasis at the time of diagnosis, and presence of late metastases were significantly associated with lower survival rates. CONCLUSION: The survival rates of the patients who underwent surgery were higher than those of non-surgical patients, although the difference was not statistically significant. Definitive radiation and chemotherapy would be preferable in selected cases, such as patients with sacral localisation, without surgical intervention. LEVEL OF EVIDENCE: Level III, Therapeutic Study.

Patient-Specific Three-Dimensional Model for a Safe Surgical Pathway in Sacral Chondrosarcoma.

Sacral tumors are amongst the most challenging procedures to treat because of the complex anatomy. This study determined whether patient-specific models change preoperative planning decisions made in preparation for en bloc resection of complex sacral mass surgical procedures. Imaging showed a big encapsulated mass at the S2-3 level involving the neural foramina and obscuring the nerve roots. High-resolution images were acquired and utilized to generate a patient-specific 3D tumor model. The visual and tactile inspection of 3D models allowed the best anatomical understanding, with faster and clearer comprehension of the surgical anatomy. The 3D sacral model was for observation of previously unapparent anatomical details; with this new technology, surgeon can observe their planned surgical intervention, explore the patient-specific anatomy and extension of the tumor, and sharpen their procedure choices. Moreover, multiple planes showed how far the angles on the plane would extend for osteotomy of the sacrum. Another result was identifying correct guides and safe venture landmarks. The study helped to establish safe osteotomy line wherever the nerve roots were retained and enabled osteotomy by preserving bilaterally the S1 and S2 nerve roots for wide excision of wide excision of primary sacral tumor to get adequate bowel and bladder functions. Finally, it helped to determine whether or not the remaining bone in the sacrum is sufficient for spinopelvic stability and needed fixation. It was decided spinopelvic fixation was not necessary for this case. Surgical intervention of sacral tumors varies depending on the tumor, its size, extension, and location. Surgery can have profound risks including unnecessary nerve root resection spinopelvic instability and suboptimal oncological resection. 3D models help to transfer complex anatomical information to clinicians and provide guidance in the preoperative planning stage, for intraoperative navigation and for surgical training purposes.

Multidisciplinary Assessment of Planning and Resection of Complex Bone Tumor Using Patient-Specific 3D Model.

Oncological interventions in thoracic cavity have some important problems such as choice of correct operative approaches depending on the tumor, size, extension, and location. In sarcoma surgery, wide resection should be aimed for the curative surgery. Purpose of this study was to evaluate pre-operative planning of patient-specific thoracic cavity model made by multidisciplinary surgeon team for complex tumor mass for oncological procedures. Patient's scans showed a large mass encroaching on the mediastinum and heart, with erosion of the adjacent ribs and vertebral column. Individual model of this case with thoracic tumor was reconstructed from the DICOM file of the CT data. Surgical team including six interdisciplinary surgeons explained their surgical experience of the use of 3D life-size individual model for guiding surgical treatment. Before patients consented to surgery, each surgeon explained the surgical procedure and perioperative risks to her. A questionnaire was applied to 10 surgical residents to evaluate the 3D model's perception. 3D model scans were useful in determining the site of the lesion, the exact size, extension, attachment to the surrounding structures such as lung, aorta, vertebral column, or vascular involvement, the number of involved ribs, whether the diaphragm was involved also in which order surgeons in the team enter the surgery. 3D model's perception was detected statistical significance as < 0.05. Viewing thoracic cavity with tumor model was more efficient than CT imaging. This case was surgically difficult as it included vital structures such as the mediastinal vessels, aorta, ribs, sternum, and vertebral bodies. A difficult pathology for which 3D model has already been explored to assist anatomic visualization was mediastinal osteosarcoma of the chest wall, diaphragm, and the vertebral column. The study helped to establish safe surgical line wherever the healthy tissue was retained and enabled osteotomy of the affected spinal corpus vertically with posterior-anterior direction by preserving the spinal cord and the spinal nerves above and distal the tumor. 3D tumor model helps to transfer complex anatomical information to surgeons, provide guidance in the pre-operative planning stage, for intra-operative navigation and for surgical collaboration purposes. Total radical excision of the bone tumor and reconstructions of remaining structures using life-size model was the key for successful treatment and better outcomes. The recent explosion in popularity of 3D printing is a testament to the promise of this technology and its profound utility in orthopedic oncological surgery.

The role of radiotherapy in local control of nonextremity Ewing sarcomas.

PURPOSE: To evaluate the results of radiotherapy and the prognostic factors affecting local control in nonextremity Ewing sarcomas. METHODS: Between 1995 and 2011, 44 patients with nonextremity Ewing sarcomas were treated with radiotherapy. Tumor localizations were pelvis in 23, spine in 13, thoracic region in 5, and cranium in 3 patients. Tumor size was ≥8 cm in 56.8% of patients. Distant metastases were present in 19 of the patients at the time of diagnosis (43.1%). All patients were treated with 12 weeks of neoadjuvant chemotherapy followed by surgery and radiotherapy (45-54 Gy) or radiotherapy alone (54-64.8 Gy). Radiotherapy was applied due to microscopic residue (R1) in 5 patients after the operation and macroscopic tumor in 39 patients (macroscopic residue [R2] and nonresectable tumor). RESULTS: Median follow-up was 49 months (range 9-195). Local failures developed in 7 patients (15.9%) and local control at 5 years was 81.4%. Local recurrence was detected in 6 patients (6/38) who did not have residual tumor after RT. Progression was detected in 1 patient (1/6) who had residual tumor. All those patients with local failure experienced further distant metastases. Possible prognostic factors such as age (≤17 vs >17), tumor localization, tumor volume (≤8 cm vs >8 cm), and M status at diagnosis (0 vs 1) were not related to local control. CONCLUSIONS: Radiotherapy, either alone or adjuvant to surgery, provides local control in 80% of nonextremity Ewing sarcomas and plays an important role in treatment.

Effect of adjuvant therapies on recurrence in aneurysmal bone cysts.

OBJECTIVE: The aim of this study was to define the effective factors such as surgical method, age and cavity filling materials on local recurrence in the treatment of aneurysmal bone cysts. METHODS: The study included 85 patients (mean age: 17.9 years) that received surgical treatment for primary aneurysmal bone cyst. Nine were treated with cyst excision and 76 with intralesional curettage. The intralesional curettage group was divided into 3 subgroups according to adjuvant therapies applied; the first group received no additional adjuvant therapy, the second group received additional high-speed burr and the third group received additional high-speed burr and alcohol/phenol adjuvant treatments. Bone graft or bone cement was used to fill in the cavity. Mean follow-up period was 107.5 months. Groups were analyzed statistically in terms of local recurrence. Onset of cyst in ages under 10 and 20 years were considered a negative prognostic factor and analyzed statistically. RESULTS: Local recurrence occurred in 10 (11.8%) patients. Mean duration between the initial operation and recurrence was 10 months. There was no significant difference in terms of local recurrence among the surgical treatment groups, adjuvant therapy groups, age groups and bone graft and bone cement groups. CONCLUSION: Careful curettage of the entire cyst wall remains the most important step in the intralesional treatment of aneurysmal bone cyst.

Aggressive fibromatosis: evaluation of prognostic factors and outcomes of surgical treatment.

OBJECTIVE: The aim of this study was to evaluate the results of surgical treatment of aggressive fibromatosis and the effects of prognostic factors on recurrence. METHODS: Forty patients (24 female, 16 male; average age: 31.2 years) diagnosed with aggressive fibromatosis were evaluated retrospectively. Nine patients with tumor-positive surgical margins macroscopically were excluded. Prognostic factors such as age, gender, localization, admission status, compartment status, surgical margin, tumor size and adjuvant radiotherapy were evaluated. RESULTS: Recurrence rate was 29%. Average disease free survival was 46±4 months. There was no statistically significant relation between prognostic factors and recurrence. Clinical results of the patients receiving adjuvant radiotherapy were more satisfactory. CONCLUSION: Adjuvant radiotherapy administration appears to be a rational treatment method instead of sacrificing function to achieve wide surgical margins.

Experimental remodellation of extracorporeal irradiated autogenous and allogenic patellar grafts.

BACKGROUND: Irradiated autografts have been used to aid the recovery of bone defects, and the results are well documented. Recently, bone allografts with tendinous attachments have been used to restore the function of joints. Similar reconstructions can be performed with irradiated autografts. However, little information is available on the biology of extracorporeal irradiated tendon autografts. QUESTIONS/PURPOSES: An experimental rabbit model was used to investigate the quality of healing and remodeling of the irradiated autogenous patellar tendon graft after 3 and 12 weeks using immunohistochemical and morphometric analyses. METHODS: New Zealand rabbits (n = 40) were randomly divided into autograft and allograft groups. The right knees of all animals served as the control (sham). The patellar tendon graft of the control right knee was reimplanted into its original location without any processing, while the patellar tendon of the left knee in the autograft group was reimplanted into the original location after 50 Gy irradiation. In the allograft group, the patellar tendon was sutured to the knee of another rabbit following 50 Gy irradiation. Five rabbits from each group were sacrificed and examined histologically. RESULTS: There were significant differences in the number of fibroblasts after 12 weeks between allograft and sham groups (P = 0.002). On the other hand, there were no differences between the allograft and autograft groups at the 12th week (P = 0.139). The difference in fibroblast numbers between autograft and allograft groups was statistically significant after the 3rd week (P < 0.05). Collagen fibril thickness was different between both the allograft and sham groups (P = 0.002) and the allograft and autograft groups at the 12th week (P = 0.000). Collagen fibrils were thicker in the sham and autograft groups compared with the allograft group at the 3rd week of evaluation (P < 0.05). The Ki67 index was significantly different between the allograft and sham groups at the 12th week (P < 0.032), while there was no difference between the allograft and autograft groups (P > 0.05). At the 3rd week, Ki67 reactivity was higher in the allograft group compared with the other two groups (P < 0.05).

Feasibility and clinical utility of intraoperative consultation with frozen section in osseous lesions.

Intraoperative consultation (IOC) with frozen section (FS) allows the surgeon to make therapy decisions during the operation. However, there is relatively little information on the use of IOC in skeletal lesions. We performed a retrospective study to examine the indications for IOC and compare the histological findings of FS and permanent paraffin section (PS) results to determine its clinical benefits. Ninety-seven consecutive cases evaluated between 2008 and 2011 were retrieved from IOC archives of our Pathology Department. In 79 % of the cases, there was no prior core needle biopsy (CNB), and IOC was performed to confirm the clinical or radiological diagnosis. In 5 (5 %) cases, no definitive result could be obtained with FS, and diagnosis was deferred. The reasons for a deferred diagnosis (DD) included poor section quality in two lipomatous lesions and the sample heterogeneity in the others. When adjusted for DD, FS and PS results showed 100 % concordance in terms of discriminating "benign vs. malignant" and defining diagnostic categories as "benign non-tumoral," "benign tumoral/tumor-like," "malignant primary tumor," "malignant metastatic tumor," or "hematopoietic malignancy." The presence of non-sclerotic osseous tissues does not have a significant adverse impact on the FS section quality and diagnosis made by FS. Thus, sections with diagnostic value can be obtained from bone lesions via FS. In this study, specific diagnoses were made in 88 % of the cases. We believe that IOC with FS can be safely performed in tertiary referral centers where there is a multidisciplinary team working in collaboration.

Acquired activated protein C resistance in sarcoma patients.

Acquired activated protein C resistance (aAPCR) is seen more frequently in solid and hematological cancer patients. We aimed to investigate the presence of aAPCR and the frequency of clinically detectable thrombosis in sarcoma patients. Normalized activated protein C sensitivity ratio (nAPCSR), factor V Leiden (FVL) mutation, factor V (FV) levels and factor VIII (FVIII) levels were prospectively measured in 52 patients and in 52 healthy controls. Clinically detectable thrombosis was present in one patient (1.92%). Compared with healthy controls (106%), the sarcoma patients had significantly lower values of the nAPCSR at pre (87.25%) and post (94.35%) treatment period (P < 0.0001). aAPCR was found as 4.2, 13 and 0%, respectively. The post-treatment FV levels (178.1 U/dl) were significantly (P < 0.001) higher than the pretreatment levels (147.5 U/dl). Inverse correlation was found between post-treatment FV levels and nAPCSR values (r = -0.38, P < 0.02). We found out a slightly increased frequency of venous thromboembolism in sarcoma patients. As an original finding which has not been reported previously in the literature, we also found out a decrease in the nAPCSR, persisting even after treatment. Thirdly, we found out that the significantly higher rate of aAPCR at the time of diagnosis totally disappeared after treatment.

Prevalence of bone and soft tissue tumors.

OBJECTIVE: Multidisciplinary approach is a necessity for the appropriate diagnosis and treatment of bone and soft tissue tumors. The Ege University Musculoskeletal Tumor Council offers consultation services to other hospitals in the Aegean region. Since 1988 the Council has met weekly and spent approximately 1,500 hours evaluating almost 6,000 patients with suspected skeletal system tumors. Our objective was to present the data obtained from this patient group. METHODS: A total of 5,658 patients, suspected to have a musculoskeletal tumor, were evaluated retrospectively. Multiple records of the patients due to multiple attendance to the Council were excluded. The prevalance of the bone and soft tissue tumors in these patients were analysed. RESULTS: Malignant mesenchymal tumors accounted for 39.7% of the total patients, benign tumors for 17%, tumor-like lesions for 17.8% and metastatic carsinomas for 8.6%. Malignant bone tumors were 50.2% and malignant soft tissue tumors were 49.8% of all the sarcomas. Among the malignant bone tumors the most common was osteosarcomas at a rate of 33.6%, followed by Ewing-PNET at 25.5%, chondrosarcomas at 19.4% and haematopoietic tumors at 17.6%. Pleomorphic sarcomas (24.5%), liposarcoma (16.4%), synovial sarcoma (13%) and undifferential sarcomas (8.8%) were the most common types of malignant sof tissue tumors. Benign soft tissue tumors (48%), benign cartilage tumors (28%), giant cell tumor (15%) and osteogenic tumors (9%) were found among the benign tumors. Hemangioma, lipoma, agressive fibromatosis, enchondroma, solitary chondroma and osteoid osteoma were the most common tumors in their groups. Lung (27%), breast (24%), gastrointestinal system (10.5%) and kidney (8.2%) carcinomas were the most common primary sites of the bone metastasis. CONCLUSION: Turkey still lacks a comprehensive series indicating the incidence and diagnostic distribution of bone and soft tissue tumors. The presented data would add to our knowledge on the specific rates of the bone and soft tissue tumors in Aegean region.

Dedifferentiated chondrosarcoma of the thumb: a case report.

Dedifferentiated chondrosarcoma (DDCS) comprises approximately 10% of all chondrosarcomas and has the worst outcome with 5-year survival of 10%. The preferred localizations are femur, humerus and pelvis. We report a case of DDCS that arose in proximal phalanx of left-hand thumb of a 66-year-old man. He was admitted with a 2-month history of pain and swelling in his thumb. He had experienced a fracture 15 years ago in the same localization with a history of suspected preexisting enchondroma. Plain radiographs showed an expansive osteolytic lesion with milimetric calcifications while magnetic resonance imaging revealed intraosseous focal globular hyperintense spots consistent with chondroid areas as well as a surrounding soft tissue mass with intermediate signal intensity. In biopsy specimen we observed a low-grade chondrosarcoma next to a spindle cell sarcoma with an abrupt transition. He was treated with amputation and died of the disease within 9 months following the surgery. The presence of dedifferented chondrosarcoma in bones of hands was not previously reported. The presented case was provided with the macroscopic and microscopic features observed in amputation specimen in comparison with radiological findings.