Can FDG-PET assess the response to chemotherapy and predict tissue necrosis in osteosarcoma and Ewing sarcoma?

INTRODUCTION: Osteosarcoma (OS) and Ewing sarcoma (ES) represent the pediatric population's most common malignant bone tumors. 18-Fluorodeoxyglucose positron emission tomography has been shown to be effective in both the diagnostic and staging phases of cancer treatment. In recent years, some studies have also explored the possibility that FDG-PET could have a prognostic role.

Hip Preservation and Capanna Reconstruction for Pediatric Proximal Femur Ewing Sarcoma: A Report of 2 Cases.

CASE: This is a first report describing preservation of the femoral head by transcervical resection of proximal femoral Ewing sarcoma in 2 pediatric patients. A unique Capanna reconstruction supported joint salvage. At 1 year, Pediatric Outcomes Data Collection Instrument and Pediatric Toronto Extremity Salvage Score outcomes were excellent. Surveillance magnetic resonance imaging was without evidence of recurrence or impaired perfusion to the femoral head. CONCLUSION: We demonstrate the feasibility of hip joint preservation and maintenance of femoral head viability after transcervical resection of pediatric proximal femur bone sarcomas while preserving the medial circumflex femoral artery. This technique may be a preferred option over joint sacrifice and endoprosthetic replacement in young patients when tumor margins permit.

Malignant tumour in pregnancy: Ewing-like sarcoma of the gluteal region.

We report a case of an Ewing-like sarcoma of the gluteal region with ongoing growth during the second trimester of pregnancy and noted during the third trimester. This lesion was consequently studied to infer its malignant potential. Several examinations were conducted to characterise this lesion, such as ultrasound and MR, which showed signs of tumourous invasion of the deep tissues of the gluteal region.Given that the pregnancy was at the end of the third trimester, the decision was made to schedule the delivery at 37 weeks of gestation and treat the tumour afterwards to balance maternal and fetal health.This case illustrates the need for a detailed investigation and guidance by a multidisciplinary team to provide prenatal counselling regarding a malignant tumour during pregnancy.

Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature.

BACKGROUND: Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management. CASE PRESENTATION: We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted. CONCLUSIONS: Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.

Recurrent Metastatic Ewing Sarcoma Involving Only in the Muscles of Extremities Shown on FDG PET/CT.

ABSTRACT: Ewing sarcoma is the second most common osseous malignancy in pediatric patient. Metastasis is common due to its aggressive nature, with 25% of patients with metastasis at diagnosis, commonly to the lungs, bone, or bone marrow. Muscle metastasis is uncommon. We report FDG PET/CT findings of multifocal muscle metastases of recurrent Ewing sarcoma in the extremities without lung and bone involvement in a 6-year-old boy.

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.

Ewing sarcoma of the cervix: an unusual site of presentation.

Ewing sarcoma is an exceedingly rare form of cancer that affects the cervix. It falls within the spectrum of neoplastic diseases known as Ewing's family of tumours, typically observed in osseous tissues. A woman in her 40s, experiencing symptoms of leucorrhoea and transvaginal bleeding that commenced 3 months before her consultation, was referred to our gynaecological oncology clinic with a preliminary diagnosis of ovarian teratoma. A colposcopy procedure was conducted unveiling a complete loss of cervical anatomy with friable and malodorous tissue. Pelvic ultrasound identified a lesion of uncertain origin in the cervix, suggestive of malignancy. Histopathological assessment of cervical biopsy specimens confirmed the presence of a small, round, blue cell neoplasm consistent with Ewing sarcoma. She underwent chemotherapy and pelvic radiotherapy, achieving complete remission 9 months after diagnosis, without experiencing any systemic adverse effects or sequelae.

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.

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.

A 13-Year-Old Boy with Chest Wall Actinomycosis Mimicking Ewing Sarcoma on Imaging.

BACKGROUND Actinomyces, a filamentous, branching, anaerobic gram-positive bacillus, typically found as a commensal organism in the oral cavity, can lead to rare chronic bacterial infections in various anatomical regions. Chest wall involvement represents an uncommon presentation, posing significant diagnostic challenges. This report focuses on the case of a 13-year-old boy presenting with chest wall actinomycetoma that closely resembled Ewing sarcoma on imaging. CASE REPORT We present the case of a 13-year-old male with no previous medical history who presented to the Emergency Department with progressive left-sided chest pain following a sports-related fall. Physical examination revealed mild swelling and tenderness on the left anterior chest wall. A chest computed tomography (CT) scan revealed a large tumor involving the lower left chest wall, suggesting Ewing sarcoma. However, a histopathological examination unexpectedly confirmed actinomycosis of the chest wall. Intravenous penicillin G was promptly initiated for 4 weeks. A follow-up CT scan after 4 weeks of therapy demonstrated a significant response with notable reduction in the size of the chest wall mass. The patient then continued with maintenance therapy using oral amoxicillin for 12 months. Throughout this period, complete resolution of the chest wall mass occurred, with no significant adverse events or complications observed. CONCLUSIONS This case highlights the importance of considering uncommon differential diagnoses like chest wall actinomycosis in patients presenting with chest wall masses. The diagnostic complexities associated with this rare condition emphasize the need for a comprehensive evaluation strategy, incorporating histopathological examination and imaging.

Radiomics analysis based on CT for the prediction of pulmonary metastases in ewing sarcoma.

OBJECTIVES: This study aimed to develop and validate radiomics models on the basis of computed tomography (CT) and clinical features for the prediction of pulmonary metastases (MT) in patients with Ewing sarcoma (ES) within 2 years after diagnosis. MATERIALS AND METHODS: A total of 143 patients with a histopathological diagnosis of ES were enrolled in this study (114 in the training cohort and 29 in the validation cohort). The regions of interest (ROIs) were handcrafted along the boundary of each tumor on the CT and CT-enhanced (CTE) images, and radiomic features were extracted. Six different models were built, including three radiomics models (CT, CTE and ComB models) and three clinical-radiomics models (CT_clinical, CTE_clinical and ComB_clinical models). The area under the receiver operating characteristic curve (AUC), and accuracy were calculated to evaluate the different models, and DeLong test was used to compare the AUCs of the models. RESULTS: Among the clinical risk factors, the therapeutic method had significant differences between the MT and non-MT groups (P＜0.01). The six models performed well in predicting pulmonary metastases in patients with ES, and the ComB model (AUC: 0.866/0.852 in training/validation cohort) achieved the highest AUC among the six models. However, no statistically significant difference was observed between the AUC of the models. CONCLUSIONS: In patients with ES, clinical-radiomics model created using radiomics signature and clinical features provided favorable ability and accuracy for pulmonary metastases prediction.

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.

Pediatric presacral tumors with intraspinal extension: a rare entity with diagnostic challenges.

The presacral space is a potential space located between the rectum and the lumbosacral spine. It contains various primitive germ cell types that serve as the origin for a range of tumors. Imaging is crucial in characterizing, assessing the extent of and evaluating the treatment response to these tumors. We report a series of six cases of pediatric presacral tumors with intraspinal extension, including an immature sacrococcygeal teratoma (Altman type II), a malignant sacrococcygeal teratoma (Altman type IV), a neuroblastoma, a rhabdomyosarcoma, a clear cell sarcoma and an Ewing's sarcoma of the ilium. These tumors can be broadly categorized as tumors of germ cell, neuroblastic, mesenchymal and osteogenic origin. Despite overlapping imaging features, a review of the existing literature and careful retrospective observation revealed several distinctive features that aid in the optimal characterization of tumors. These include the tumor's epicenter, the pattern and degree of bone involvement, the status of sacral foramina and neural elements, and internal tumor characteristics such as the presence of fat, calcification, hemorrhage and necrosis.

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma.

With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as 'ultra-rare' sarcomas, with an incidence of ≤1-5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing's sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.

Primary Extraskeletal Ewing Sarcoma of the Duodenum Revealed by 18 F-FDG PET/CT.

ABSTRACT: Extraskeletal Ewing sarcoma of the duodenum is a relatively uncommon entity tumor. We report a case of extraskeletal Ewing sarcoma in a 21-year-old woman. She complained of abdomen pain and melena. 18 F-FDG PET/CT showed an intense uptake by the duodenum mass along with multiple FDG-avid enlarged lymph nodes in the mesentery, which was pathologically proven as extraskeletal Ewing sarcoma.