The use of vascularized fibula flap with allograft in post-oncologic microsurgical bone reconstruction of lower limbs in pediatric patients.

BACKGROUND: Post-oncologic surgical reconstruction of lower limbs in pediatrics remains a challenging topic. Microsurgical techniques allow reconstructions of large bony defects. The use of vascularized fibular flap with allograft has proven to be an ideal biologic construct. We aim to assess the success rate of this operation, including flap survival, bony union, weight-bearing ambulation, and complications in a long-term follow-up in our case series compared to the literature. PATIENTS AND METHODS: Our case-series includes 18 femoral resections (9 osteosarcomas, 8 Ewing sarcoma, and 1 desmoid tumor) and 15 tibial resections (10 osteosarcoma, 4 Ewing sarcoma, and 1 Malignant Fibrous Histiocytoma). We collected patients' demographics, type of tumor, type of resection, defect size, fibula-flap length, method of fixation, anastomosis site, follow-up data, complications, and their management. All survivals were examined by X-ray and CT-scan to evaluate the morphological changes of the vascularized fibula and follow-up. The functional evaluation was performed by the 30-point Musculoskeletal Tumor Society Rating Score (MSTS) for the lower limb (Enneking et al., Clinical Orthopaedics and Related Research 1993(286):241-246). RESULTS: The mean age of the femur resection patients' group was 11.2 years with a mean defect size of 14 cm and a mean length of the fibular flap of 18 cm; for the tibia the mean age was 12 years with a mean defect size of 14 cm and a mean length of the fibular flap of 16.6 cm. The overall survival of the reconstructions at 5 years follow-up was 17 out 18 cases for the femur and 13 out of 15 cases for the tibia. MSTS score was 28.2 for the femur and 23.7 for the tibia. The average time of union of the fibula was seen after 5 months, while allograft consolidation was observed around 19.7 months. The mean time of follow-up was 144.5 months for the femur and 139.2 months for the tibia. The complication rate observed was 7 out of 18 for the femur and 7 out of 15 for the tibia reconstructions. CONCLUSIONS: The viability of the fibula is a cornerstone in the success of reconstruction as well as the successful management of complications in intercalary defects after tumor resection in pediatrics to restore good functionality. Our results are in line with those reported in the literature in terms of overall complication rates. The high primary union of allograft, the high MSTS score obtained, and the low rate of severe complications reflect the mechanical role of this reconstructive technique over a long follow-up.

Sarcoma of the uterine cervix: experience of a single center.

OBJECTIVES: To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix. METHODS: We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020 and analyzed the clinicopathological characteristics and prognosis. RESULTS: 34 patients were identified, 7 (20.6%) patients had leiomyosarcoma, 6 (17.6%) had carcinosarcoma, 5 (14.7%) had Ewing sarcoma, 4 (11.8%) had rhabdomyosarcoma, 4 (11.8%) had undifferentiated sarcoma, 2 (5.9%) had adenosarcoma, 2 (5.9%) had endometrial stromal sarcoma, 1 (2.9%) had dermatofibrosarcoma protuberans, 1 (2.9%) had alveolar soft tissue sarcoma and 2 (5.9%) had sarcoma not otherwise specified. The median age of the whole patients was 43.5 years (range, 13-63). The median age of patients with Ewing sarcoma or rhabdomyosarcoma was 22 years (range, 13-39) and 17 years (range, 13-36 years), respectively. The distribution by stage was: stage I in 21 (61.8%) patients, stage II in 4 (11.8%), stage III in 6 (17.6%) and stage IV in 3 (8.8%). Overall, 30 patients (88.2%) received surgical treatment. The median follow-up was 33.3 months (range 3.6-187.3 months). 11 patients died within 2 years after diagnosis, most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). In the entire cohort, 2- and 5-year OS were 67.2% and 56.9%, respectively. 5-year OS was 25.0% for undifferentiated sarcoma, 50.0% for rhabdomyosarcoma, 50.0% for carcinosarcoma, 53.3% for Ewing sarcoma, 57.1% for leiomyosarcoma. CONCLUSION: Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Prognosis may be associated with tumor histology and stage.

Intraoperative radiation therapy for pediatric sarcomas and other solid tumors.

PURPOSE: To evaluate local failure (LF) and toxicity after intraoperative radiation therapy (IORT) in pediatric solid tumors (ST). METHODS: A single-institution retrospective study of 96 pediatric patients (108 applications) with ST treated from 1995 to 2022 with IORT. LF was calculated via cumulative incidence function and overall survival (OS) by Kaplan-Meier method, both from the day of surgery. RESULTS: Median age at time of IORT was 8 years (range: 0.8-20.9 years). Median follow-up for all patients and surviving patients was 16 months and 3 years, respectively. The most common histologies included rhabdomyosarcoma (n = 42), Ewing sarcoma (n = 10), and Wilms tumor (n = 9). Most (95%) received chemotherapy, 37% had prior external beam radiation therapy to the site of IORT, and 46% had a prior surgery for tumor resection. About half (54%) were treated with upfront IORT to the primary tumor due to difficult circumstances such as very young age or challenging anatomy. The median IORT dose was 12 Gy (range: 4-18 Gy), and median area treated was 24 cm2 (range: 2-198 cm2). The cumulative incidence of LF was 17% at 2 years and 23% at 5 years. Toxicity from IORT was reasonable, with postoperative complications likely related to IORT seen in 15 (16%) patients. CONCLUSION: Our study represents the largest and most recent analysis of efficacy and safety of IORT in pediatric patients with ST. Less than one quarter of all patients failed locally with acceptable toxicities. Overall, IORT is an effective and safe technique to achieve local control in patients with challenging circumstances.

Use of modern three-dimensional imaging models to guide surgical planning for local control of pediatric extracranial solid tumors.

INTRODUCTION: In complex pediatric surgical oncology, surgical planning is contingent upon data gathered from preoperative imaging. Three-dimensional (3D) modeling and printing has been shown to be beneficial for adult presurgical planning, though pediatric literature is less robust. The study reviews our institutional experience with the use of 3D image segmentation and printed models in approaching resection of extracranial solid tumors in children. METHODS: This is a single institutional series from 2021 to 2023. Models were based on computed tomography and magnetic resonance imaging studies, optimized for 3D imaging. The feasibility and creation of the models is reviewed, including specific techniques, software, and printing materials from our institution. Clinical implications for surgical planning are also described, along with detailed preoperative and intraoperative images. RESULTS: 3D modeling and printing was performed for four pediatric patients diagnosed with extracranial solid tumors. Diagnoses included Ewing sarcoma, hepatoblastoma, synovial sarcoma, and osteosarcoma. No intraoperative complications or discrepancies with the preoperative 3D-printed model were noted. No evidence of local recurrence was identified in any patient thus far. CONCLUSION: Our institutional series demonstrates a wide spectrum of clinical application for 3D modeling and printing technology within pediatric surgical oncology. This technology may aid in surgical planning for both resection and reconstruction, can be applied to a diverse breadth of diagnoses, and may potentially augment patient and/or family education about their condition.

Racial disparities in the management and outcomes of primary osseous neoplasms of the spine: a SEER analysis.

PURPOSE: Primary osseous neoplasms of the spine, including Ewing's sarcoma, osteosarcoma, chondrosarcoma, and chordoma, are rare tumors with significant morbidity and mortality. The present study aims to identify the prevalence and impact of racial disparities on management and outcomes of patients with these malignancies. METHODS: The 2000 to 2020 Surveillance, Epidemiology, and End Results (SEER) Registry, a cancer registry, was retrospectively reviewed to identify patients with Ewing's sarcoma, osteosarcoma, chondrosarcoma, or chordoma of the vertebral column or sacrum/pelvis. Study patients were divided into race-based cohorts: White, Black, Hispanic, and Other. Demographics, tumor characteristics, treatment variables, and mortality were assessed. RESULTS: 2,415 patients were identified, of which 69.8% were White, 5.8% Black, 16.1% Hispanic, and 8.4% classified as "Other". Tumor type varied significantly between cohorts, with osteosarcoma affecting a greater proportion of Black patients compared to the others (p < 0.001). A lower proportion of Black and Other race patients received surgery compared to White and Hispanic patients (p < 0.001). Utilization of chemotherapy was highest in the Hispanic cohort (p < 0.001), though use of radiotherapy was similar across cohorts (p = 0.123). Five-year survival (p < 0.001) and median survival were greatest in White patients (p < 0.001). Compared to non-Hispanic Whites, Hispanic (p < 0.001) and "Other" patients (p < 0.001) were associated with reduced survival. CONCLUSION: Race may be associated with tumor characteristics at diagnosis (including subtype, size, and site), treatment utilization, and mortality, with non-White patients having lower survival compared to White patients. Further studies are necessary to identify underlying causes of these disparities and solutions for eliminating them.

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension.

BACKGROUND AND OBJECTIVE: Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. CLINICAL PRESENTATION: This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. CONCLUSION: The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

Disparities in Primary Spinal Osseous Malignant Bone Tumor Survival by Medicaid Status: A National Population-Based Risk Analysis.

BACKGROUND: The impact of Medicaid status on survival outcomes of patients with spinal primary malignant bone tumors (sPMBT) has not been investigated. METHODS: Using the SEER-Medicaid database, adults diagnosed between 2006 and 2013 with sPMBT including chordoma, osteosarcoma, chondrosarcoma, Ewing sarcoma, or malignant giant cell tumor (GCT) were studied. Five-year survival analysis was performed using the Kaplan-Meier method. Adjusted survival analysis was performed using Cox proportional-hazards regression controlling for age, sex, marital status, cancer stage, poverty level, vertebral versus sacral location, geography, rurality, tumor diameter, tumor grade, tumor histology, and therapy. RESULTS: A total of 572 patients with sPMBT (Medicaid: 59, non-Medicaid: 513) were identified. Medicaid patients were more likely to be younger (P < 0.001), Black (P < 0.001), live in high poverty neighborhoods (P = 0.006), have distant metastases at diagnosis (P < 0.001), and less likely to receive surgery (P = 0.006). The 5-year survival rate was 65.7% (chondrosarcoma: 70.0%, chordoma: 91.5%, Ewing sarcoma: 44.6%, GCT: 90.0%, osteosarcoma: 34.2%). Medicaid patients had significantly worse 5-year survival than non-Medicaid patients (52.0% vs. 67.2%, P = 0.02). Minority individuals on Medicaid were associated with an increased risk of cancer-specific mortality compared with White non-Medicaid patients (adjusted hazard ratio [aHR] = 2.51, [95% CI 1.18-5.35], P = 0.017). Among Medicaid patients, those who received surgery had significantly better survival than those who did not (64.5% vs. 30.6%, P = 0.001). For all patients, not receiving surgery (aHR = 1.90 [1.23-2.95], P = 0.004) and tumor diameter >50 mm (aHR=1.89 [1.10-3.25], P = 0.023) were associated with an increased risk of mortality. CONCLUSIONS: Medicaid patients may be less likely to receive surgery and suffer from poorer survival. These disparities may be especially prominent among minorities.

Primary Ewing's sarcoma of the talus.

Ewing's sarcoma is a malignant round cell tumour of bones and soft tissues that usually arises from the diaphyseal or meta-diaphyseal parts of long bones and less commonly from flat bones. It occurs rarely in the foot and if occurs, the calcaneus and the metatarsals are commonly involved. We present a case of a young woman diagnosed with primary Ewing's sarcoma of the talus with local spread to adjacent tarsals and the ankle joint. Ewing's sarcoma of feet, if present with even a trivial suspicion of spread either locally or distant, makes limb salvage surgery difficult. So, the treatment with radical surgery or by combined chemotherapy and radiotherapy should be considered-keeping in mind the complex anatomy of the foot and the difficulty in achieving tumour-free margins. Based on this experience, she underwent below-knee amputation. The patient received adjuvant chemotherapy and survived with a disease-free survival at the latest follow-up of 1 year.

Does primary tumor resection improve survival for patients with sarcomas of pelvic bones, sacrum, and coccyx who have metastasis at diagnosis ?

BACKGROUND: Recent studies demonstrated that primary tumor resection (PTR) improves survival of patients with metastatic bone sarcomas. However, it remains quite unclear regarding the role of PTR in the treatment of sarcomas of pelvic bones with synchronous metastasis at diagnosis. METHODS: Using the Surveillance, Epidemiology, and End Results Program, we enrolled a total of 385 patients with sarcomas of pelvic bones, sacrum, and coccyx who have metastasis at initial diagnosis, including 139 patients with osteosarcoma, 176 with Ewing sarcoma, and 70 with chondrosarcoma. Association between PTR and disease-specific survival (DSS) were investigated using the univariable and multivariable Cox regression models. Hazard ratio (HR) and 95% confidence interval (CI) were reported. Representative institutional PTR strategies and clinical outcomes for patients with metastatic pelvic sarcomas from our cancer center were displayed. RESULTS: The usage rate of PTR was 28.1% (39/139) in osteosarcoma, 13.6% (24/176) in Ewing sarcoma, and 41.4% (29/70) in chondrosarcoma with synchronous metastatic lesions. PTR was not associated with an improved DSS for metastatic pelvic osteosarcoma (HR = 0.686, 95% CI = 0.430 ~ 1.094, P = 0.113) and Ewing sarcoma (HR = 0.580, 95% CI = 0.291 ~ 1.154, P = 0.121). The use of PTR was associated with an improved DSS for metastatic pelvic chondrosarcoma (HR = 0.464, 95% CI = 0.225 ~ 0.954, P = 0.037). CONCLUSION: Primary lesion resection may provide a survival benefit for metastatic chondrosarcoma, but not for osteosarcoma and Ewing sarcoma of pelvic bones, sacrum, and coccyx. This population-based study recommends an active surgical intervention for metastatic chondrosarcoma while non-surgical treatment for metastatic osteosarcoma and Ewing sarcoma of the pelvis in terms of survival improvement.

Postoperative Relapse Prediction in Patients With Ewing Sarcoma Using Computed Tomography-Based Radiomics Models Covering Tumor Per Se and Peritumoral Signatures.

OBJECTIVE: We aimed to develop and validate a computed tomography (CT)-based radiomics model for early relapse prediction in patients with Ewing sarcoma (ES). METHODS: We recruited 104 patients in this study. Tumor areas and areas with a tumor expansion of 3 mm were used as regions of interest for radiomics analysis. Six different models were constructed: Pre-CT, CT enhancement (CTE), Pre-CT +3 mm , CTE +3 mm , Pre-CT and CTE combined (ComB), and Pre-CT +3 mm and CTE +3 mm combined (ComB +3 mm ). All 3 classifiers used a grid search with 5-fold cross-validation to identify their optimal parameters, followed by repeat 5-fold cross-validation to evaluate the model performance based on these parameters. The average performance of the 5-fold cross-validation and the best one-fold performance of each model were evaluated. The AUC (area under the receiver operating characteristic curve) and accuracy were calculated to evaluate the models. RESULTS: The 6 radiomics models performed well in predicting relapse in patients with ES using the 3 classifiers; the ComB and ComB +3 mm models performed better than the other models (AUC -best : 0.820-0.922/0.823-0.833 and 0.799-0.873/0.759-0.880 in the training and validation cohorts, respectively). Although the Pre-CT +3 mm , CTE +3 mm, and ComB +3 mm models covering tumor per se and peritumoral CT features preoperatively forecasted ES relapse, the model was not significantly improved. CONCLUSIONS: The radiomics model performed well for early recurrence prediction in patients with ES, and the ComB and ComB +3 mm models may be superior to the other models.

Pelvic bone sarcomas, prognostic factors, and treatment: A narrative review of the literature.

Primary sarcomas of bone are rare malignant mesenchymal tumors. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The prognosis has improved over the years, but bone sarcomas are still life-threatening tumors that need a multidisciplinary approach for diagnosis and treatment. Bone sarcomas arising in the pelvis present a unique challenge to orthopedic oncologists due to the absence of natural anatomical barriers, the close proximity of vital neurovascular structures, and the high mechanical demands placed on any pelvic reconstruction following the excision of the tumor. While radiotherapy has an important role especially in Ewing's sarcoma and chemotherapy for both Ewing's sarcoma and osteosarcoma, surgery remains the main choice of treatment for all three entities. While external hemipelvectomy has remained one option, the main aim of surgery is limb salvage. After complete tumor resection, the bone defect needs to be reconstructed. Possibilities to reconstruct the defect include prosthetic or biological reconstruction. The method of reconstruction is dependent on the location of tumor and the surgery required for its removal. The aim of this article is to give an insight into pelvic bone sarcomas, their oncological and surgical outcomes, and the options for treatment based on the authors' experiences.

Ewing's sarcoma of the duodenum: a rare clinical condition managed with surgical resection.

A man in his 40s with no medical history presented with right-sided abdominal and chest pain. A CT scan of the abdomen demonstrated a 7.7 cm heterogeneous mass arising from the second part of the duodenum. Oesophagogastroduodenoscopy confirmed a malignant-appearing duodenal lesion, with biopsy showing features consistent with small cell carcinoma. The patient underwent three cycles of neoadjuvant chemotherapy, followed by elective Kausch-Whipple pancreaticoduodenectomy. A combination of immunohistochemistry and molecular studies confirmed the diagnosis of a rare Ewing's sarcoma tumour originating from the duodenum with invasion into the duodenal lumen. The patient recovered well from surgery and remains disease-free 18 months following resection.

Extracorporeal irradiation in malignant bone tumors: Single institution experience and review of literature.

In the last couple of decades, the management of malignant bone tumor (MBT) has seen a sea change. With the advent in surgical technics, radiation therapy, and chemotherapy, it has moved from disabling amputation to limb salvage surgery. Extracorporeal irradiation (ECI) and re-implantation of resected bone is a useful method of limb salvage of MBTs. In our study, we analyzed and presented the results of 8 cases of MBTs treated with this modality. Between 2014 and 2017, 8 patients with primary MBT were enrolled for ECI technique who are meeting the eligibility criteria. Before taking the patient for ECI treatment, a multispecialty tumor board discussion was done for each patient. All of them received neo-adjuvant and adjuvant chemotherapy except the patients with histology of giant cell tumor. Following neoadjuvant chemotherapy bone excision surgery was performed, and the resected bone was taken for ECI with the dose of 50 Gray in a single fraction. After ECI, bone segment was re-implanted at osteotomy site in the same setting. After completion of adjuvant chemotherapy, the patients were then followed up for any sequelae, local and systemic control, ambulation, and functional outcome. Out of 8 patients, there were 5 males and 3 females with mean age of 22 (range 13-36). The involved bone was the tibia in 6 patients, ischium in 1 patient, and femur in 1 patient. Histopathologically, the malignancies included 3 osteosarcoma, 3 Giant cell tumor, 1 Ewing's sarcoma and 1 chondrosarcoma. At median follow-up of 12 months (range 6-26 months), local control rate was 87.5% and systemic control rate was 75%. Perioperative ECI and re-implantation is a useful, convenient, and inexpensive technique. The overall treatment time is reduced. The patient's own bone fits perfectly to the resection site with reduced risk of graft site infection. The risk of local recurrence due to tumor re-implantation is negligible with tumoricidal radiation doses of ECI, and it is usually associated with manageable sequelae. Recurrence rates are acceptable and salvageable with surgery.

Large retroperitoneal extraskeletal Ewing's sarcoma with renal pedicle invasion: a case report.

BACKGROUND: Extraskeletal Ewing's sarcoma (EES) is a rare malignant tumor primarily found in children and young adults. Localized disease can present with nonspecific symptoms such as local mass, regional pain, and increased skin temperature. More severe cases may present with systemic symptoms such as malaise, weakness, fever, anemia, and weight loss. Among these lesions, retroperitoneal sarcomas are relatively uncommon and difficult to diagnose. Since they are usually asymptomatic until large enough to compress or invade the surrounding tissues, most are already advanced at first detection. Traditionally, the treatment of choice is complete surgical resection, sometimes combined with postoperative radiotherapy and chemotherapy. We report a case of EES with left renal artery invasion in the left retroperitoneal cavity successfully treated with transarterial embolization and surgery. CASE PRESENTATION: A 57-year-old woman with a negative family history of cancer presented at our Urology Department with a large left retroperitoneal tumor found by magnetic resonance imaging during the health exam. Physical examination showed a soft abdomen and no palpable mass or tenderness. Imaging studies showed that the tumor covered the entire left renal pedicle, but the left kidney, left adrenal gland, and pancreas appeared tumor free. Since the tumor tightly covered the entire renal pedicle, tumor excision with radical nephrectomy was advised. The patient underwent transarterial embolization of the left renal artery with 10 mg of Gelfoam pieces daily before surgical excision. Tumor excision and left radical nephrectomy were uneventful the day after embolization. Post-operatively, the patient recovered well and was discharged on day 10. The final histopathological analysis showed a round blue cell tumor consistent with an Ewing sarcoma, and the surgical margins were tumor free. CONCLUSIONS: Retroperitoneal malignancies are rare but usually severe conditions. Our case report showed that retroperitoneal EES with renal artery invasion could be treated safely with transarterial embolization and surgery.

Clinical characteristics of primary cutaneous and subcutaneous Ewing sarcoma.

OBJECTIVE: Given the rarity of cutaneous/subcutaneous Ewing sarcoma, their clinical characteristics remain poorly understood. In this study, we aimed to analyse the clinical characteristics of patients with cutaneous/subcutaneous Ewing sarcoma and review the treatment strategy. METHODS: We reviewed the clinical data of 154 patients with Ewing sarcoma who were treated at our hospital between 2005 and 2019. Amongst these patients, 21 patients with cutaneous/subcutaneous Ewing sarcoma were analysed. As a basic strategy, patients with localized disease received intensive chemotherapy (vincristine-doxorubicin-cyclophosphamide/ifosfamide-etoposide), followed by definitive surgery with or without radiotherapy. In total, 15 patients underwent pre-diagnostic resection with macroscopic residue (seven patients) or non-macroscopic residue (eight patients) before intensive chemotherapy. RESULTS: The median tumour length of the measurable lesions was 3.2 cm, and the ratio of metastasis was significantly lower than the Ewing sarcoma of other anatomical sites (10% vs. 37%, P = 0.013). Despite the pre-diagnostic resection, local recurrence after additional resection and/or adjuvant radiotherapy did not occur in any of the patients with localized disease. Overall survival was significantly higher in patients with cutaneous/subcutaneous Ewing sarcoma than in patients with Ewing sarcoma of other anatomical sites (hazard ratio = 0.33, P = 0.013). The event-free survival rate of cutaneous/subcutaneous Ewing sarcoma was also superior to that of Ewing sarcoma of other anatomical sites (hazard ratio = 0.35, P = 0.01). CONCLUSIONS: Patients with cutaneous/subcutaneous Ewing sarcoma may have better prognosis than those with Ewing sarcoma at other anatomical sites. Although pre-diagnostic resection without appropriate investigations is not recommended, local control may be recovered by using a combination of additional resection, chemotherapy and radiotherapy.

Extended Neoadjuvant Chemotherapy in Delayed Primary Resection of Ewing Sarcoma During the COVID-19 Era: A Case Report.

CASE: A 13-year-old adolescent boy visited our hospital with a growing mass on his left leg. Investigations and examinations were performed to obtain a final diagnosis of Ewing sarcoma in the head of the left fibula with lung metastasis. Neoadjuvant chemotherapy was extended to 11 courses with radiation before wide tumor resection could be performed. The final 3 adjuvant chemotherapy courses were administered to complete the original protocol while surgical resection complications were also treated. The pathological report revealed free margin resection with nonviable tumor cells. CONCLUSION: An extended neoadjuvant chemotherapy regimen with additional radiation therapy for Ewing sarcoma provided extra local control and allowed limb salvage.

Survey of Palliative Care Use in Primary Malignant Bone Tumors: A National Cancer Database Review.

Background/Objectives: Palliative care (PC) has been associated with reduced patient symptom burden, improved physician satisfaction, and reduced cost of care. However, its use in primary bone tumors has not been well classified. Design/Setting and Subjects: Patients diagnosed with primary malignant bone tumors (osteosarcoma, chondrosarcoma, Ewing sarcoma, and chordoma) between 2004 and 2018 were identified in the National Cancer Database. Cross tabulations with chi-square analysis were performed to evaluate frequencies of PC use by patient, facility, and tumor characteristics. Multivariate logistic binary regression was performed to evaluate relationships between patient, treatment facility, and tumor characteristics and the use of PC. Results: Around 24,401 patients were identified. Overall, 2.52% had any form of PC utilization. Of those receiving PC, 55.5-65.1% were treated with only noncurative surgery, radiation, chemotherapy, or any combination of these modalities. Odds of PC utilization were decreased for patients with chordomas, patients living >24 miles from the treatment facility, or patients with private insurance, Medicare, or unknown insurance status. Odds of PC utilization were increased in patients with greater tumor diameter or unknown tumor size, tumors in midline, increased tumor grade, stage IV tumors, or living in urban areas. Conclusion: PC use in patients with primary bone tumors increases with tumor stage, tumor grade, tumor size, and if the tumor is midline, and in patients living in urban areas. However, overall utilization remains markedly low. Future studies should be done to investigate these patterns of care and help expand the utilization of PC.

Primary Intracranial Ewing Sarcoma Invading the Superior Sagittal Sinus with EWSR1-FLI1 Gene Fusion and EWSR1 Gene Mutation: A Case Report and Literature Review.

BACKGROUND: Primary intracranial Ewing sarcoma (ES) is an extremely rare intracranial malignant tumor, mostly occurring in children and adolescents. Because of its rarity, the magnetic resonance imaging (MRI) features and treatment strategies of primary intracranial ES are still unclear. METHODS: The purpose of this study was therefore to report a case of primary intracranial ES, whose molecular features included both EWSR1-FLI1 (EWS RNA binding protein 1- Friend leukemia integration 1) gene fusion and EWSR1 gene mutation. It is worth noting that this is the first reported case of ES invading the superior sagittal sinus and mostly causing occlusion. At the same time, there were polymorphisms of four drug metabolism-related enzymes in the tumor. Subsequently, we conducted a literature review to characterize the clinical features, imaging findings, pathological features, treatments, and prognoses of primary intracranial ESs. RESULTS: A 21-year-old female was admitted to the hospital with headache with nausea and vomiting for 2 weeks. An MRI showed a 3.8 × 4.0 cm large heterogeneous mass in the bilateral parietal lobe with peritumoral edema. The tumor invaded the superior sagittal sinus and mostly caused occlusion of the middle segment of the superior sagittal sinus. The mass was successfully removed using a neuromicroscope. Postoperative pathology indicated a primary intracranial ES. High throughput sequencing (next generation sequencing) showed that there was EWSR1-FLI1 gene fusion and EWSR1 gene mutation in the tumor, with polymorphisms of four drug metabolism-related enzymes and low tumor mutational burden. Subsequently, the patient received intensity modulated radiation therapy. The patient has signed an informed consent form. CONCLUSIONS: The diagnosis of primary intracranial ES depended on histopathology, immunohistochemistry staining, and genetic testing. At present, total tumor resection combined with radiotherapy and chemotherapy is the most effective treatment. We report the first case of primary intracranial ES invading the superior sagittal sinus and causing middle segment occlusion, accompanied by EWSR1-FLI1 gene fusion and EWSR1 gene mutation.