Does the Clinical Presentation of Secondary Osteosarcoma in Patients Who Survive Retinoblastoma Differ From That of Conventional Osteosarcoma and How Do We Detect Them?

BACKGROUND: Osteosarcoma is the most common secondary malignancy among survivors of retinoblastoma. Most previous reports on secondary malignancy of retinoblastoma included all types of secondary malignancies without a focus on osteosarcoma, owing to its rarity. In addition, there are few studies suggesting tools for regular surveillance for early detection. QUESTIONS/PURPOSES: (1) What are the radiologic and clinical characteristics of secondary osteosarcoma after retinoblastoma? (2) What is the clinical survivorship? (3) Is a radionuclide bone scan a reasonable imaging modality for early detection in patients with retinoblastoma? METHODS: Between February 2000 and December 2019, we treated 540 patients for retinoblastoma. Twelve patients (six male, six female) subsequently developed an osteosarcoma in the extremities; two of these patients had two sites of osteosarcoma (10 femurs, four tibiae) . A Technetium-99m bone scan image was examined annually in all patients for regular surveillance after the treatment of retinoblastoma as per our hospital's policy. All patients were treated with the same strategy as that used for primary conventional osteosarcoma, namely neoadjuvant chemotherapy, wide excision, and adjuvant chemotherapy. The median follow-up period was 12 years (range 8 to 21 years). The median age at the time of diagnosis of osteosarcoma was 9 years (range 5 to 15 years), and the median interval from retinoblastoma diagnosis to osteosarcoma diagnosis was 8 years (range 5 to 15 years). Radiologic characteristics were assessed with plain radiographs and MRI, while clinical characteristics were assessed through a retrospective review of medical records. For clinical survivorship, we evaluated overall survival, local recurrence-free survival, and metastasis-free survival. We reviewed the results of bone scans and clinical symptoms at the time of diagnosis for osteosarcoma after retinoblastoma. RESULTS: In nine of 14 patients, the tumor had a diaphyseal center, and five of the tumors were located at the metaphysis. The femur was the most common site (n = 10), followed by the tibia (n = 4). The median tumor size was 9 cm (range 5 to 13 cm). There was no local recurrence after surgical resection of the osteosarcoma, and the 5-year overall survival rate after the diagnosis of osteosarcoma was 86% (95% CI 68% to 100%). In all 14 tumors, the Technetium bone scan showed increased uptake in the lesions. Ten of 14 tumors were examined in clinic because of patient complaints of pain in the affected limb. Four patients showed no clinical symptoms detected by abnormal uptake on bone scan. CONCLUSION: For unclear reasons, secondary osteosarcomas in patients who were alive after the treatment of retinoblastoma had a slight predilection for the diaphysis of the long bone compared with patients with spontaneous osteosarcoma in other reports. The clinical survivorship of osteosarcoma as a secondary malignancy after retinoblastoma may not be inferior to that of conventional osteosarcoma. Close follow-up with at least yearly clinical assessment and bone scans or other imaging modalities appears to be helpful in detecting secondary osteosarcoma after the treatment of patients with retinoblastoma. Larger multi-institutional studies will be needed to substantiate these observations.Level of Evidenc e Level IV, therapeutic study.

Does reverse total shoulder arthroplasty with allograft-prosthesis composite (APC) have surgical benefits over hemiarthroplasty with APC in patients with tumors of the proximal humerus?

BACKGROUND: The research on surgical outcomes of hemiarthroplasty and reverse total shoulder arthroplasty using allograft-prosthesis composites for the proximal humeral oncologic condition is still scarce. Therefore, this study aimed to compare surgical outcomes of shoulder joint reconstruction with hemiarthroplasty and reverse total shoulder arthroplasty using allograft-prosthesis composites for tumors of the proximal humerus. METHODS: Eleven patients underwent hemiarthroplasty or reverse total shoulder arthroplasty using allograft-prosthesis composites for tumors of the proximal humerus between July 2011 and April 2018 were reviewed. Radiographic analysis for bone union of allograft-host bone junction, implant loosening, stress shielding and shoulder dislocation or subluxation was performed. Functional outcomes were evaluated using visual analog scales for pain, range of motion, Simple Shoulder Test score and Musculoskeletal Tumor Society score. Furthermore, oncologic outcome and complications were also assessed, respectively. RESULTS: There were five patients with hemiarthroplasty (mean age, 23.2 years) and six patients with reverse total shoulder arthroplasty (mean age, 46.8 years, P = 0.05). Radiographically, there were no events associated with implant loosening, stress shielding and shoulder dislocation or subluxation in the two groups. There were no differences in functional outcomes between the two groups. There was no local recurrence in entire cohort. In the hemiarthroplasty group, one patient was required revision surgery to reverse total shoulder arthroplasty at postoperative 6 years due to rotator cuff dysfunction. In the reverse total shoulder arthroplasty group, one patient showed the fracture occurred at allograft-host bone junction at postoperative 6 months. CONCLUSIONS: Surgical outcomes of hemiarthroplasty with allograft-prosthesis composites were not inferior to reverse total shoulder arthroplasty when applied in properly selected patients. The authors recommended that hemiarthroplasty with allograft-prosthesis composites could be used for young age patients without glenoid metastasis involvement, and reverse total shoulder arthroplasty with allograft-prosthesis composites could be used for patients with old age or metastatic bone tumors.

Surgical treatment and long-term outcomes of low-grade myofibroblastic sarcoma: a single-center case series of 15 patients.

BACKGROUND: Low-grade myofibroblastic sarcoma (LGMS) is a poorly studied, rare, soft tissue sarcoma. LGMS is characterized by a low malignancy potential, tendency for local recurrence, and low likelihood of distant metastases. However, no studies have reported on the surgical treatment method and its long-term outcomes. METHODS: We included all patients treated for LGMS at our institution between March 2010 and March 2021. Medical charts were retrospectively reviewed to collect demographic information, as well as information about the clinical course, tumor characteristics, and outcomes. Statistical analysis was performed to identify the factors associated with the recurrence rate. RESULTS: Fifteen patients who underwent surgical treatment were enrolled in this study. There were seven cases in the upper extremities, four in the trunk area, three in the lower extremities, and one in the head and neck area. There were no metastatic cases and two cases of local recurrence. CONCLUSIONS: The incidence of LGMS in the extremities or trunk may be higher than expected based on the current literature. Univariate analysis showed that local tissue invasion and surgical method could be associated with local recurrence. Although further large studies are needed to establish risk factors of local recurrence or extent of resection margins, based on our study, wide local excision under the proper diagnosis is the most important treatment.

Ambulation Recovery After Surgery for Metastases to the Femur.

BACKGROUND: Postoperative ambulation recovery after surgery for femur metastases has significant implications for not only the patient's quality of life but also administration of further cancer treatment. Thus, identification of preoperative predictors of ambulation recovery is necessary to set appropriate expectations and guide treatment. This study aimed to assess ambulation recovery rate and identify predictors of ambulation recovery in patients undergoing surgery for femur metastases. MATERIALS AND METHODS: A total of 244 patients who underwent surgery for femur metastases at our institution were reviewed. Patients were considered ambulatory if they were able to walk independently or walk with aids and nonambulatory if they were wheelchair bound or bedridden. The following potential clinicopathologic factors that might predict postoperative ambulation recovery were evaluated: premorbid general status, cancer burden, and local factors. RESULTS: A total of 165 patients (68%) regained ambulatory status postoperatively. A multivariate analysis revealed poor Eastern Cooperative Oncology Group (ECOG) performance status (odds ratio [OR], 5.327; p < .001) and nonambulatory premorbid ambulatory status (OR, 7.459; p < .001) as independent predictors of poor ambulation recovery after surgery for femur metastases. Postoperative ambulatory status was significantly associated with postoperative survival time (p < .001). CONCLUSION: Postoperative ambulation recovery rate in our cohort was 68%. Premorbid ambulatory status and ECOG performance status are predictors of ambulation recovery in patients undergoing surgery for femur metastases. IMPLICATIONS FOR PRACTICE: Postoperative ambulation recovery rate in this cohort was 68%. Premorbid ambulatory status and Eastern Cooperative Oncology Group performance status are predictors of ambulation recovery in patients undergoing surgery for femur metastases.

Changing odds of survival over time among patients undergoing surgical resection of extremity soft tissue sarcoma.

BACKGROUND: While survival after surgical treatment of extremity soft tissue sarcoma (STS) is traditionally reported as actuarial survival, conditional survival (CS) may be more clinically relevant as it accounts for time already survived. We compared actuarial survival and CS of STS patients. MATERIALS AND METHODS: We analyzed 567 patients who underwent surgery for localized extremity STS. Actuarial survival was estimated using the Kaplan-Meier method. Cox proportional hazards model was used to evaluate factors associated with disease-specific survival. Five-year CS (CS5) estimates at "χ" year(s) after surgery were calculated as CS5 = S(χ + 5)/S(χ). RESULTS: Whereas actuarial survival decreased over time, CS5 increased. The postsurgical 1-, 3-, and 5-year CS5 values were 84.5%, 90.0%, and 93.8%, respectively, whereas the 6-, 8-, and 10-year actuarial survival rates were 82.0%, 79.4%, and 78.5%, respectively. The calculated CS5 exceeded actuarial survival especially in patients with risk factors such as large tumor size and Federation Nationale des Centers de Lutte Contre le Cancer (FNCLCC) grades 2 and 3 tumors. Patients with tumor size ≥5 cm had an actuarial survival of 73.9% at 10 years compared to a CS5 of 95.4% in patients alive at 5 years. Likewise, patients with FNCLCC grade 3 tumors had an actuarial survival of 71.1% at 10 years compared to a CS5 of 96.0% in patients alive at 5 years. CONCLUSIONS: Survival estimation by determination of CS can be dynamic and accurate especially in high-risk patients. CS can be useful for survival prediction and clinical decision making in extremity STS patients.

EWSR1-PBX3 fused myoepithelioma arising in metatarsal bone: Case report and review of the literature.

Intraosseous myoepithelial tumors are very rare. Due to the low incidence and diverse histologic features, accurate diagnosis is challenging, necessitating ancillary immunohistochemistry. Moreover, genetic abnormality in this tumor was not revealed until recently. Although EWSR1 translocation is involved in half of the cases of intraosseous myoepithelioma, only a few cases have indicated its counterpart gene. We herein describe a case of intraosseous myoepithelioma with a novel localization in the fourth metatarsal bone of a 36-year-old female. Cytogenetic analysis using next generation sequencing detected a rare EWSR1-PBX3 fusion. Next generation sequencing could be useful in understanding the cytogenetic characteristics of intraosseous myoepithelioma, and in obtaining an accurate diagnosis of this rare condition.

Histological findings of regeneration in retrieved pasteurized bone grafts.

BACKGROUND: Despite a number of radiologic evaluations of the incorporation of pasteurized bone (PB) in human and histologic evaluations in animal models, there has been a scarce documentation regarding the histologic evaluation of PB from human. Herein, we present histologic findings of regeneration in retrieved PB graft from pediatric and adult patients. METHODS: PB was retrieved for various reasons in 7 patients (10-52 years old). Two bone pathologists independently counted the number of empty lacunae and lacunae with living cells in up to 10 randomly selected fields on medium-power (H&E, ×200) for each patient. Regeneration of PB was assessed as the ratio of the number of lacunae with nucleated cells to that of whole lacunae, which was defined as the "repair rate (RR)". RESULTS: The mean interval between initial reconstruction and retrieval (graft removal time; GRT) was 47.4 months (range, 11-144 months). The length of original PBs ranged from 5.8 to 20.6 cm. Microscopic examination of PBs showed areas with empty lacunae indicating necrosis and other areas contained lacunae with nucleated osteocytes, indicative of regeneration. Some Haversian canals of the PBs were filled with fibrovascular tissue and surrounded by lamellar bones including living osteocytes. RR varied widely from 21.7 to 62.4% with a mean of 36.8%. It was much higher in adult patients (46.6-62.4%, mean = 55.3%) than in pediatric patients (21.7-28.6%, mean = 25.3%), which was correlated with GRT (pediatric patients; mean of 14 months, adult patients; mean of 72.3 months). In adult patients, RR was higher in a patient with prosthesis composite in the proximal humerus (Case No. 3; 62.4%). CONCLUSIONS: RR was higher in whom GRT was longer, being correlated with GRT in retrieved PBs. In terms of our histological observation, PB is thought to be an acceptable temporary biologic spacer in limb-sparing surgery for malignant bone or soft tissue tumors.

Factors associated with local recurrence after surgery for bone metastasis to the extremities.

BACKGROUND AND OBJECTIVES: With increasing life expectancy of patients with bone metastasis, durable surgical stabilization of bone metastasis is necessary. Local recurrence (LR) can compromise surgical stabilization and necessitate retreatment. We analyzed LR rate and factors associated with LR in patients undergoing surgery for bone metastasis. METHODS: Patients (n = 301) who underwent surgery for bone metastasis to the extremities were reviewed. Possible factors that might be associated with LR were investigated. RESULTS: LR rate was 16% (49/301). Surgical margin was associated with LR, as patients with en-bloc resection had significantly less LR than patients who underwent curettage (5/66 vs 44/235, P = 0.03). Prostate cancer had lowest rate (0%) of LR and colon cancer had highest rate (31%). Interval from surgery to LR differed among primary cancer types (4.5 ± 3.9 months [lung cancer], vs 12.3 ± 12.9 months [other cancers], P = 0.041). In multivariate analysis, en-bloc surgical margins (HR = 0.372, P = 0.036) and primary cancers of breast or prostate (HR = 0.391, P = 0.049) were independent factors associated with longer LR-free survival. CONCLUSIONS: LR after surgery for bone metastasis to extremities is affected by surgical margin and primary cancer type. These factors, along with expected patient survival, need to be considered when planning surgery for bone metastasis to extremities.

Can Augmented Reality Be Helpful in Pelvic Bone Cancer Surgery? An In Vitro Study.

BACKGROUND: Application of surgical navigation for pelvic bone cancer surgery may prove useful, but in addition to the fact that research supporting its adoption remains relatively preliminary, the actual navigation devices are physically large, occupying considerable space in already crowded operating rooms. To address this issue, we developed and tested a navigation system for pelvic bone cancer surgery assimilating augmented reality (AR) technology to simplify the system by embedding the navigation software into a tablet personal computer (PC). QUESTIONS/PURPOSES: Using simulated tumors and resections in a pig pelvic model, we asked: Can AR-assisted resection reduce errors in terms of planned bone cuts and improve ability to achieve the planned margin around a tumor in pelvic bone cancer surgery? METHODS: We developed an AR-based navigation system for pelvic bone tumor surgery, which could be operated on a tablet PC. We created 36 bone tumor models for simulation of tumor resection in pig pelves and assigned 18 each to the AR-assisted resection group and conventional resection group. To simulate a bone tumor, bone cement was inserted into the acetabular dome of the pig pelvis. Tumor resection was simulated in two scenarios. The first was AR-assisted resection by an orthopaedic resident and the second was resection using conventional methods by an orthopaedic oncologist. For both groups, resection was planned with a 1-cm safety margin around the bone cement. Resection margins were evaluated by an independent orthopaedic surgeon who was blinded as to the type of resection. All specimens were sectioned twice: first through a plane parallel to the medial wall of the acetabulum and second through a plane perpendicular to the first. The distance from the resection margin to the bone cement was measured at four different locations for each plane. The largest of the four errors on a plane was adopted for evaluation. Therefore, each specimen had two values of error, which were collected from two perpendicular planes. The resection errors were classified into four grades: ≤ 3 mm; 3 to 6 mm; 6 to 9 mm; and > 9 mm or any tumor violation. Student's t-test was used for statistical comparison of the mean resection errors of the two groups. RESULTS: The mean of 36 resection errors of 18 pelves in the AR-assisted resection group was 1.59 mm (SD, 4.13 mm; 95% confidence interval [CI], 0.24-2.94 mm) and the mean error of the conventional resection group was 4.55 mm (SD, 9.7 mm; 95% CI, 1.38-7.72 mm; p < 0.001). All specimens in the AR-assisted resection group had errors < 6 mm, whereas 78% (28 of 36) of errors in the conventional group were < 6 mm. CONCLUSIONS: In this in vitro simulated tumor model, we demonstrated that AR assistance could help to achieve the planned margin. Our model was designed as a proof of concept; although our findings do not justify a clinical trial in humans, they do support continued investigation of this system in a live animal model, which will be our next experiment. CLINICAL RELEVANCE: The AR-based navigation system provides additional information of the tumor extent and may help surgeons during pelvic bone cancer surgery without the need for more complex and cumbersome conventional navigation systems.

Cortical Atrophy Related to Tumor Prosthesis in Skeletally Immature Osteosarcoma Patients.

BACKGROUND: Cortical atrophy is commonly observed after prosthetic reconstruction for bone sarcomas. However, relevant literature regarding this phenomenon in skeletally immature patients is limited. Therefore, in this study, we evaluated the incidence and patterns of cortical atrophy. We then assessed its predisposing factors. Finally, we analyzed whether cortical atrophy was associated with poor prosthesis survival. METHODS: We retrospectively reviewed 31 stems in 19 skeletally immature osteosarcoma patients who were treated with resection and tumor prosthesis surgery. We measured the cortical thickness using plain radiographs annually. The mean patient age was 11 years (range, 6 to 14 y) and the mean follow-up period was 10 years (range, 2 to 14 y). RESULTS: Cortical atrophy developed in 13 stems, all within 3 years. There were 3 gross types of cortical atrophy: hourglass in 5, sharpening in 4, and shortening atrophy in 4. On multivariate analysis, stem-cortex diameter ratio of ≥0.5 and age of less than 10 years were significantly associated with cortical atrophy (P=0.002 and P=0.019, respectively). Cortical atrophy was significantly associated with eventual prosthesis failure (9/13 in the cortical atrophy group vs. 4/18 in the nonatrophy group, P=0.035). CONCLUSIONS: Cortical atrophy is common in the skeletally immature patients with tumor prosthesis and is associated with prosthesis failure. Patients with cortical atrophy should be closely monitored for prosthesis failure and appropriately managed to prevent failures. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Optimal Treatment of Osteofibrous Dysplasia of the Tibia.

BACKGROUND: Our study of a large patient group reports on the behavior and postoperative recurrence of osteofibrous dysplasia (OFD). METHODS: We reviewed the medical records of 55 patients who were diagnosed with OFD of the tibia and showed typical features of this tumor. The patients' presentation, disease course, history of pathologic fracture, typical radiographic features, surgical treatment history, and surgical results were investigated. RESULTS: The longitudinal OFD lesion size peaks at a mean 13.3 years of age with a mean maximum proportionate size of 0.33. These lesions in 92% of the patients spontaneously presented with a stable disease course, while those in the other 8% continued increasing. There were no significant predictive factors of disease course. Among surgically treated lesions, we noted a higher rate of recurrence after curettage than after excision (P<0.001). Patients who underwent curettage and developed recurrence were significantly younger than those who did not (P=0.01). CONCLUSIONS: Our data suggest that observation can be the primary form of treatment for patients with OFD and that the disease would stop advancing with time. During observation, clinicians should carefully observe all patients with OFD because the relation between OFD and adamantinoma is unclear. If surgery is necessary due to severe morbidity, we recommend excision rather than curettage to prevent recurrence, especially for younger patients. LEVEL OF EVIDENCE: Level III.

Does routine colonoscopy help diagnose familial adenomatous polyposis in patients presenting with desmoid tumors but no gastrointestinal symptoms?

PURPOSE: Desmoid tumor (DT) is a rare myofibroblastic proliferative neoplasm, but can occur in up to 26% of patients with familial adenomatous polyposis (FAP). Therefore, DT may be an extraintestinal manifestation of FAP that precedes the development of advanced malignancies. The aim of this study was to identify the incidence of polyps in DT patients and investigate value of colonoscopy for diagnosis of FAP in DT patients without gastrointestinal symptoms. MATERIALS AND METHODS: The records of patients diagnosed with DT were retrospectively reviewed using the clinical data warehouse (CDW) system. Clinical data, including location of tumor, type(s) of treatment, and colonoscopic findings, were collected. RESULTS: Sixty-five patients were diagnosed with DT during the study period; 10 patients received colonoscopies before diagnosis of DT, and 20 patients received colonoscopies after diagnosis of DT. The mean age at diagnosis of DT was 41.9 ± 16.7 years old (range 17-74). Most tumors were extra-abdominal (n = 24, 80.0%), and fewer were intra-abdominal (n = 4, 13.3%) or in an abdominal wall (n = 2, 6.7%). The colonoscopy results revealed one or two tubular adenomas in 11 patients (36.6%), although none of the patients had colorectal polyposis or colorectal cancer. CONCLUSION: None of the patients in our study population had colorectal polyposis. Routine colonoscopy of DT patients without gastrointestinal symptoms seems to have low diagnostic yield for detection of FAP.

A portable surgical navigation device to display resection planes for bone tumor surgery.

INTRODUCTION: Surgical navigation has been used in musculoskeletal tumor surgical procedures to improve the precision of tumor resection. Despite the favorable attributes of navigation-assisted surgery, conventional systems do not display the resection margin in real time, and preoperative manual input is required. In addition, navigation systems are often expensive and complex, and this has limited their widespread use. In this study, we propose an augmented reality surgical navigation system that uses a tablet personal computer with no external tracking system. MATERIAL AND METHODS: We realized a real-time safety margin display based on three-dimensional dilation. The resection plane induced by the safety margin is updated in real time according to the direction of sawing. The minimum separation between the saw and the resection plane is also calculated and displayed. The surgeon can resect bone tumors accurately by referring to the resection plane and the minimum separation updated in real time. RESULTS: The effectiveness of the system was demonstrated with experiments on pig pelvises. When the desired resection margin was 10 mm, the measured resection margin was 9.85 ± 1.02 mm. CONCLUSIONS: The proposed method exhibits sufficient accuracy and convenience for use in bone tumor resection. It also has favorable practical applicability due to its low cost and portability.

Subtrochanteric femoral stress fracture with features of atypical femoral fracture in patient with Paget's disease: a case report.

Atypical femoral fracture (AFF) has been widely issued since the American Society for Bone and Mineral Research (ASBMR) taskforce stated a possible association with long-term use of bisphosphonate in 2010. ASBMR taskforce recommended to include Paget's disease as one of variables to investigate the AFF in 2010. On the other hand, the ASBMR taskforce in 2014 excluded Paget's disease in the definition of AFF. Still, any rationale has been barely documented about this change. We described the rationale by reporting an 85-year-old man who had a subtrochanteric fracture with features of atypical femoral fracture which turned out to be Paget's disease of bone in the pathology.

Low-heat treated autograft versus allograft for intercalary reconstruction of malignant bone tumors.

INTRODUCTION: Low-heat treated autograft may have an advantage over allograft in that they provide a size-matched and biologically compatible graft. MATERIALS AND METHODS: We compared the graft survival and complications between the low-heat treated autografts (n = 13) and allografts (17) for intercalary reconstruction of malignant bone tumors. Average age was 25 years with follow-up of 6.6 years (1.8-12.5). For low-heat treatment, the resected bone was treated in saline at 65°C for 30 min. Nineteen grafts were fixed with plates and 13 with intramedullary nails. RESULTS: Until last follow-up, five low-heat treated autografts (38%) and one allograft (6%) had been removed, with allografts showing a trend toward better survival on Kaplan-Meier analysis (P = 0.293). Complications were more common in low-heat treated autograft group [10/13 (77%) vs. 8/17 (47%), P = 0.098)]. Low-heat-treated autograft resulted in significantly higher rate of nonunion [11/26 (42%) vs. 5/34 (15%), P = 0.017] and required higher number of operations to treat nonunion (P = 0.004). Rates of graft fracture [3/13 (23%) vs. 2/17 (12%), P = 0.713)] or infection [2/13 (15%) vs. 1/17 (6%), P = 0.565)] were similar. CONCLUSION: Our data suggest that intercalary allograft has better survival and fewer complications than the low-heat treated autograft.

Glomus tumor as a cause of coccydynia.

Glomus coccygeum is a network of glomus bodies located around pericoccygeal soft tissue. The question of whether it accounts for coccydynia has been debated. We report on a patient whose preoperative symptoms suggested the impression of glomus tumor of the coccyx; pain was relieved after removal of the lesion without coccygectomy. A 57-year-old woman was referred to us with a history of a pain in the coccygeal area lasting longer than 3 years. The patient complained of sharp pain whenever pressure was applied to the coccyx. Pain was aggravated by exposure to cold. MRI revealed a coccygeal lesion measuring 2 cm with a well-circumscribed margin. The lesion was excised without removal of any portion of the coccyx. The pathology report confirmed a glomus tumor, which was an identical finding to the conventional one of the subungual region. Ten months after surgery, she had no pain on the coccygeal region and no difficulty with sitting on a chair and in performing activities of daily living. Findings reported here suggest that although most glomus bodies are normal anatomical variants, development of a glomus tumor could occur in the coccygeal region and could be a cause of coccydynia.

Isolation and characterization of endothelial cells from intramuscular hemangioma.

BACKGROUND: Intramuscular hemangiomas (IMHs) are benign vascular tumors of deep soft tissue characterized by endothelial cell (EC) proliferation. The purpose of this study was to isolate ECs from IMH, characterize their angiogenic phenotype and functional characteristics, and search for a possible signaling pathway related to IMH development. METHODS: EC Isolation from IMH was performed by digestion, filtration, washing, incubation, and purification in sequence. Tie2 expression was compared between ECs from IMH and controls using reverse transcriptase polymerase chain reaction (RT-PCR). Cell invasion and proliferation assays were used to analyze functional responses of ECs to angiopoietin 1 (Ang1) and vascular endothelial growth factor (VEGF). Expression of downstream targets was analyzed using Western blot analysis. RESULTS: Isolated ECs showed typical cobblestone appearance under light microscopy and formed capillary-like tubular structures using Matrigel tube-forming assay. RT-PCR of isolated ECs from six patients showed increased expression of Tie2 and VEGF receptor 1 (VEGFR1) compared with control ECs. Tie2 activation by Ang1 compared with VEGFR1 by VEGF resulted in increased EC migration and proliferation. Western blot analysis showed increased Tie2 expression in hemangioma samples compared with normal ECs. Phosphorylated Akt and phosphorylated forkhead box O1 (FOXO1) expression was observed in hemangioma samples only. CONCLUSION: EC isolation from IMH could be a useful tool for further research. These results suggest that increased Tie2 expression, via Akt-FOXO1 pathway activation, may play an important role in IMH pathogenesis.

Minimal invasive surgery for unicameral bone cyst using demineralized bone matrix: a case series.

BACKGROUND: Various treatments for unicameral bone cyst have been proposed. Recent concern focuses on the effectiveness of closed methods. This study evaluated the effectiveness of demineralized bone matrix as a graft material after intramedullary decompression for the treatment of unicameral bone cysts. METHODS: Between October 2008 and June 2010, twenty-five patients with a unicameral bone cyst were treated with intramedullary decompression followed by grafting of demineralized bone matrix. There were 21 males and 4 female patients with mean age of 11.1 years (range, 3-19 years). The proximal metaphysis of the humerus was affected in 12 patients, the proximal femur in five, the calcaneum in three, the distal femur in two, the tibia in two, and the radius in one. There were 17 active cysts and 8 latent cysts. Radiologic change was evaluated according to a modified Neer classification. Time to healing was defined as the period required achieving cortical thickening on the anteroposterior and lateral plain radiographs, as well as consolidation of the cyst. The patients were followed up for mean period of 23.9 months (range, 15-36 months). RESULTS: Nineteen of 25 cysts had completely consolidated after a single procedure. The mean time to healing was 6.6 months (range, 3-12 months). Four had incomplete healing radiographically but had no clinical symptom with enough cortical thickness to prevent fracture. None of these four cysts needed a second intervention until the last follow-up. Two of 25 patients required a second intervention because of cyst recurrence. All of the two had a radiographical healing of cyst after mean of 10 additional months of follow-up. CONCLUSIONS: A minimal invasive technique including the injection of DBM could serve as an excellent treatment method for unicameral bone cysts.

Does the modified Glasgow Prognostic Score aid in the management of patients undergoing surgery for a soft-tissue sarcoma? : an international multicentre study.

AIMS: The modified Glasgow Prognostic Score (mGPS) uses preoperative CRP and albumin to calculate a score from 0 to 2 (2 being associated with poor outcomes). mGPS is validated in multiple carcinomas. To date, its use in soft-tissue sarcoma (STS) is limited, with only small cohorts reporting that increased mGPS scores correlates with decreased survival in STS patients. METHODS: This retrospective multicentre cohort study identified 493 STS patients using clinical databases from six collaborating hospitals in three countries. Centres performed a retrospective data collection for patient demographics, preoperative blood results (CRP and albumin levels and neutrophil, leucocyte, and platelets counts), and oncological outcomes (disease-free survival, local, or metastatic recurrence) with a minimum of two years' follow-up. RESULTS: We found that increased mGPS, tumour size, grade, neutrophil/lymphocyte ratio, and disease recurrence were associated with reduced survival. Importantly, mGPS was the best at stratifying prognosis and could be used in conjunction with tumour grade to sub-stratify patient survival. CONCLUSION: This study demonstrated that prognosis of localized STS strongly correlates with mGPS, as an increasing score is associated with a poorer outcome. We note that 203 patients (41%) with an STS have evidence of systemic inflammation. We recommend the mGPS and other biochemical blood indicators be introduced into the routine diagnostic assessment in STS patients to stratify patient prognosis. Its use will support clinical decision-making, especially when morbid treatment options such as amputation are being considered. Cite this article: Bone Joint J 2022;104-B(1):168-176.

Prognostic value of computed tomography for monitoring pulmonary metastases in soft tissue sarcoma patients after surgical management: a retrospective cohort study.

BACKGROUND: The lung is the most common site of metastases in patients with a soft tissue sarcoma. However, surveillance protocol for pulmonary metastasis after surgical management has not been proved. The goal of this retrospective study was to evaluate the prognostic value of computed tomography of the chest in soft tissue sarcoma patients after surgery. METHODS: A retrospective study was performed on 176 patients who were followed up with chest computed tomographic (CT) scan or plain chest radiographs for pulmonary metastasis monitoring after surgery. Ninety-six patients were followed up with plain chest radiographs and 80 patients with chest CT scan. Mean duration of follow-up was 55.8 months. The Kaplan-Meier method and the log-rank test were used to calculate overall survival rates and survival rate difference between chest CT and plain radiograph cohorts. RESULTS: The overall 5- and 10-year survival rates of 176 patients were 75.6% and 70.3%, respectively. The 5-year survival estimates of 96 patients who were included in the plain radiograph cohort and 80 patients in the chest CT cohort were 74.2% and 76.6%, respectively (P=0.70). Fifty-four patients (30.7%) had pulmonary metastasis. Of the 54 patients, 26 belonged to the plain radiograph cohort and 28 patients belonged to the chest CT cohort. Pulmonary metastasis of chest CT cohort had the tendencies of unilaterality, a smaller number of patients, and management with metastasectomy other than palliative management. The 2- and 4-year survival ratess after detection of pulmonary metastasis were 20.1% and 0% in the plain radiograph cohort and 47.4% and 31.6% in the chest CT cohort (P<0.05). CONCLUSION: Serial monitoring with chest CT could give rise to early detection of pulmonary metastases, chance for metastasectomy, and eventually survival advantage.