Primary Ewing's Sarcoma of the Sternum in an Adult Male: A Rare Case Report.

Ewing's sarcoma, a rare primary bone malignancy primarily affecting adolescents and young adults, typically manifests in the pelvic bones and femur. Primary Ewing's sarcoma of the sternum is exceptionally rare, constituting less than 1% of cases. We present a case of a 34-year-old man with a 2-month history of anterior chest wall pain initially attributed to muscular spasm. Subsequently, the patient developed a palpable mass and imaging demonstrated a mid-lower sternal lesion with cortical destruction and soft tissue involvement, confirmed as Ewing's sarcoma on biopsy. In addition, a suspicious lesion was identified in the left distal tibia, which was histologically confirmed as a metastasis from the primary sternal sarcoma. Neoadjuvant chemotherapy preceded partial sternotomy with rib resection and reconstruction, achieving clear surgical margins. Postoperative evaluation showed shrinkage in the sternal lesion and near-resolution of the tibial metastasis. Subsequent chemotherapy cycles resulted in no evidence of the disease on the follow-up positron emission tomography scan. This case underscores the diagnostic challenges of primary sternal Ewing's sarcoma and emphasizes the importance of early recognition and comprehensive evaluation in managing such rare presentations.

Monostotic femoral Caffey disease masquerading as Ewing sarcoma.

We describe a rare case of monostotic infantile cortical hyperostosis (Caffey disease) involving the left femur of an infant, who presented with recent onset left thigh swelling, following vaccination. Radiological workup showed a lamellated periosteal reaction involving the left femoral diaphysis on radiographs masquerading as a bone tumour. The child underwent MRI of the left thigh, which showed extensive muscle oedema without any abnormal soft-tissue proliferation, marrow signal alteration, cortical breach or collection. The follow-up radiograph showed exuberant new bone formation in the second week. The patient was given symptomatic treatment and the parents were counselled. The child recovered well with gradual resolution of symptoms and bony remodelling on a 6-month follow-up radiograph. Here, we describe the serial changes on the radiographs in Caffey disease with monostotic involvement and the role of MRI in difficult cases to differentiate it from other common mimickers, such as infections and neoplasia.

Let it glow: Intraoperative visualization of pulmonary metastases using pafolacianine, a next-generation fluorescent agent, for young adults undergoing pulmonary metastasectomy.

A new generation of disease-specific molecular imaging agents is poised to revolutionize fluorescence-guided surgery. Pafolacianine has been approved for adult lung and ovarian cancers. We demonstrate a proof of concept for pediatric surgeons treating young adults with pulmonary metastatic sarcomas. Five successful fluorescence-guided pulmonary metastasectomy operations were performed in young adult patients with metastatic osteosarcoma or Ewing sarcoma following administration of pafolacianine. All osteosarcoma lesions identified using standard techniques were also markedly fluorescent in patients. Novel fluorescent molecular agents targeted to tumor-specific receptors have promise of increased sensitivity and specificity for detecting metastatic nodules and enhancing surgical clearance of disease.

Ewing sarcoma presenting in the lung: a case report.

BACKGROUND: Ewing sarcoma is a malignant round-cell tumor that primarily affects bones in children. It can also arise in extraosseous tissues, such as the lung, kidneys, and liver. The presentation symptoms of Ewing sarcoma may include cough, dyspnea, and chest pain. CASE PRESENTATION: This report details the history of a 15-year-old Syrian boy with a previous diagnosis of Hodgkin lymphoma who presented with chronic shoulder pain. Imaging studies revealed an 80 mm mass in the apex of the left lung, which was confirmed through histopathological examination to be Ewing sarcoma following a computed-tomography-guided biopsy. The patient received multiple cycles of chemotherapy and subsequently underwent surgical resection of the remaining mass. CONCLUSIONS: This case highlights the rare occurrence of Ewing sarcoma in the lung and the unusual clinical presentation of shoulder pain without other accompanying symptoms.

Ewing Sarcoma of the Larynx with Thyroid Cartilage Destruction: A Case Report.

Ewing sarcoma of the larynx is extremely rare, only a few number of cases have been reported. In this report, we describe a case of extraskeletal Ewing sarcoma of the larynx with thyroid cartilage destruction. Laryngoscope, 134:4577-4581, 2024.

[Ewing sarcoma/primitive hepatic neuroectodermal tumor]. / Sarcoma de Ewing/tumor neuroectodérmico primitivo hepático.

Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) belong to the group of neoplasms called small round cell tumors. PNETs have been divided into central and peripheral. ES and peripheral PNETs arise from bones, soft tissues, or peripheral nerves. We present a case of hepatic ES/PNET in a healthy man that began four months before consultation with abdominal symptoms and weight loss. Upper gastrointestinal endoscopy and laboratory tests revealed no notable findings. The abdominal tomography revealed an enlarged liver due to a solid lesion that involved all its segments with intravenous contrast enhancement and large areas of necrosis. It compressed and displaced neighboring structures. Core needle biopsy of the liver lesion was performed: small round cell neoplasm. Immunohistochemistry revealed negativity for CD45, CKA1/A3, chromogranin, synaptophysin, and cytokeratins CK7 and CK20. Dim CD56 expression and CD99, FLI-1, and NKX2 positivity. He underwent chemotherapy treatment with carboplatin and etoposide for 6 cycles with clinical improvement and tolerance. Control images showed reduction of the mass with involvement of the right hepatic lobe, involvement of the inferior vena cava, infiltration of the right adrenal gland and upper pole of the right kidney. He was referred to hepatobiliary surgery for surgical resection of the residual lesion. The patient rejected the proposed surgical procedure. Our objective is to highlight the clinical and histological diagnostic challenge of this entity that requires ruling out other clinical entities.

El sarcoma de Ewing (ES) y el tumor neuroectodérmico primitivo (PNET) pertenecen al grupo de neoplasias denominadas tumores de células pequeñas y redondas. Los PNET se dividen en centrales y periféricos. El ES y los PNET periféricos surgen del tejido óseo, de los tejidos blandos o nervios periféricos. Presentamos un caso de ES/PNET hepático en un hombre sano que inició cuatro meses antes de la consulta con síntomas abdominales y pérdida de peso. La endoscopia digestiva alta y la analítica no revelaron hallazgos relevantes. En la tomografía de abdomen se evidenció hígado aumentado de tamaño a expensas de lesión sólida que comprometía todos sus segmentos con realce al contraste endovenoso y grandes áreas de necrosis. Comprimía y desplazaba estructuras vecinas. Se realizó biopsia con aguja gruesa de la lesión hepática: neoplasia de células pequeñas y redondas. La inmunohistoquímica reveló negatividad para CD45, CKA1/A3, cromogranina, sinaptofisina y citoqueratinas CK7 y CK20. Expresión tenue de CD56 y positividad de CD99, FLI-1 y NKX2. Realizó tratamiento quimioterápico con carboplatino y etopósido por 6 ciclos con mejoría clínica y tolerancia al mismo. En imágenes de control se evidenció reducción de la masa con afección del lóbulo hepático derecho, compromiso de la vena cava inferior, infiltración de la glándula suprarrenal y polo superior del riñón derechos. Se remitió a cirugía hepatobiliar para resección quirúrgica de la lesión residual. El paciente rechazó el procedimiento quirúrgico. Nuestro objetivo es destacar el desafío diagnóstico clínico e histológico de esta entidad que obliga a descartar otras entidades clínicas.

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.

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.

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.

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.

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.

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.

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.

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.