Primitive neuroectodermal tumours (PNETs) are rare, malignant tumours arising from primitive nerve cells. PNET of the chest wall is rare and is observed in children and young adults. Askin defined Askin\'s tumour as a PNET of the thoracopulmonary area. It develops from the soft tissues of the chest wall, particularly in the paravertebral region. Here, we report a case of Askin\'s tumour, a rare neoplasm occurring in the thoracopulmonary region in a 13-year-old girl. She came with complaints of fever, cold, cough with mucoid expectoration, breathlessness for 15 days, and generalized weakness for three months. A high-resolution computed tomography (HRCT) thorax scan was also done, which suggested a large right pleural-based mass with vertebral metastasis. Through diligent diagnostic evaluation involving imaging studies and histopathological examination, the tumour was accurately identified.

BACKGROUND: Ewing\'s sarcoma is a primary bone tumor predominantly observed in children and adolescents, necessitating a multidisciplinary treatment approach. While localized cases have a 5-year survival rate of 60-70%, the prognosis is significantly worse in pelvic advanced cases with metastasis. Moreover, pelvic Ewing\'s sarcoma has the unique problem of leading to high rates of postoperative infection.
METHODS: We present the case of a Japanese 14-year-old boy with left iliac Ewing\'s sarcoma and multiple metastases. At the initial visit, imaging revealed a large tumor in the left iliac bone with extraosseous extension and metastasis to multiple sites. Neoadjuvant chemotherapy resulted in significant tumor reduction. Surgical resection was performed without pelvic ring reconstruction to enable early postoperative chemotherapy and minimize postoperative infection risk. Despite complete abductor muscle removal, the patient achieved a stable gait postoperatively by centering the load axis.
CONCLUSIONS: Our case highlights the successful management of a left iliac Ewing\'s sarcoma with multiple metastases, with a focus on functional preservation and infection risk reduction. Pelvic ring reconstruction was not performed to avoid postoperative complications, emphasizing the importance of early postoperative chemotherapy. The patient achieved a stable gait postoperatively, demonstrating the potential benefits of this approach in similar cases.

Ewing sarcoma is one of the small round blue cell tumors of childhood that typically affects bone. Recently, a subgroup of undifferentiated round-cell sarcomas has been genetically identified as BCOR (B-cell Line 6 Corepressor)-altered sarcomas (BAS). We present a case of a six-year-old male child who presented with a chief complaint of shortness of breath and tachypnea and was found to have a large mediastinal mass concerning sarcoma. Preliminary biopsy results were positive for small round blue cells, possibly Ewing sarcoma. After six cycles of chemotherapy, with subsequent shrinkage of mediastinal mass, the patient was able to undergo wedge resection and excision of the mass with en bloc resection of the fifth and sixth rib, preserving his right lung. Final tissue pathology was positive for BAS. There have been only four reported cases of BAS of the chest wall and zero reported cases of primary tumor presentation of the lung, making this a rare presentation of the disease.

UNASSIGNED: Ewing\'s sarcoma is the second most common primary malignant bone tumor in children and adolescents, after osteosarcoma. It is a rare tumor, with the axial skeleton being the preferred site of development, followed by the long bones. Diagnosis is evoked by imaging and confirmed by histology. Treatment is based on intensive chemotherapy with local surgical treatment in operable forms, and in some cases, radiotherapy. There are only a few sporadic cases in the literature describing distal fibular localization.
UNASSIGNED: We report the case of a 7-year-old child who presented with pain on the lateral aspect of the left ankle without local inflammatory signs. Radiological findings revealed a metaphyseal-diaphyseal osteolytic tissue process of the left fibula, and histology concluded that it was Ewing\'s sarcoma. We proceeded with neoadjuvant polychemotherapy followed by segmental resection of the distal fibula with an anteroexternal tibial rod, plus tibiotalar, and talocalcaneal arthrodesis without recourse to radiotherapy.
UNASSIGNED: The management of Ewing\'s sarcoma is constantly evolving. Its distal fibular location in a growing limb makes it even more difficult. It must be personalized, multidisciplinary, and carried out in specialized centers.

BACKGROUND: Small round cell tumor (SRCT) is a group of malignancy with similar optical microscopic morphology. Despite its low incidence, SRCT has a high malignant degree and poor prognosis. Besides, atypical clinical symptoms make it difficult in preoperative diagnosis.
METHODS: A 67-year-old man was presented to the outpatient service with dysuria and weak urine stream lasting for 3 months. After oral treatment with tamsulosin and finasteride for 2 months, the symptoms worsen. Transurethral prostate holmium laser enucleation was operated and postoperative pathology result revealed small blue round cell malignant tumor. Further immunohistochemistry and fluorescence in situ hybridization examination indicated Ewing-like SRCT. So a Da Vinci Robotic prostatectomy was performed further and whole-genome sequencing was conducted. Several gene mutations including RAF1, ARID1A, SMARCA4, and BCL2L11 were found but no FDA-approved drug could treat specifically. Then the patient received Ewing-type therapeutic regimens treatment and has been followed up to date (over 24 months).
CONCLUSIONS: Because of its non-elevated serum PSA level, prostate SRCT is often ignored as a possibility of malignant tumor and regarded as benign prostatic hyperplasia (BPH). The possibility of prostate SRCT need to be considered if dysuria symptoms could not alleviate significantly after a period of oral treatment.

Ewing\'s sarcoma, characterized by small round cell morphology, is a rare malignancy, with mediastinal Ewing\'s sarcoma being even less common. This case describes a distinctive presentation of primary mediastinal Ewing\'s sarcoma in a 32-year-old male presenting with sudden and severe chest pain. Initial evaluation excluded cardiac and pulmonary emergencies, revealing a posterior mediastinal mass through advanced imaging. The patient\'s clinical symptoms significantly improved following the complete resection of the tumor via thoracoscopy. Subsequent analysis incorporating imaging, histological, immunohistochemical and genetic findings led to the conclusive diagnosis of primary mediastinal Ewing\'s sarcoma.

Extraosseous Ewing sarcoma is a rare and aggressive malignancy belonging to the Ewing sarcoma family of tumors, primarily affecting soft tissues such as the pelvis, retroperitoneum, and chest wall. Although it predominantly involves these soft tissues, extraosseous Ewing sarcoma can also occur in solid organs, including the pancreas. Here, we present a rare case of a 4-year-old girl diagnosed with primary extraosseous Ewing sarcoma of the pancreas.

BACKGROUND: Ewing\'s sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing\'s sarcoma can present as an intradural extramedullary mass mimicking more common tumors.
METHODS: A 32-year-old female had a left L3 nerve root-associated lesion identified in the setting of recent-onset radiculopathy. Contrast-enhanced magnetic resonance imaging of the lumbar spine was favored to demonstrate a schwannoma or neurofibroma. Hemilaminectomy, facetectomy, and resection of the mass led to improved radiculopathy and a tissue diagnosis of Ewing\'s sarcoma. Immediate referral to medical oncology facilitated expeditious initiation of adjuvant chemotherapy and radiation.
CONCLUSIONS: The differential diagnosis for newly identified nerve root-associated tumors should remain broad, including common benign pathologies and rare malignant entities. Tissue remains the gold standard for diagnosis, as preoperative imaging suggested a nerve sheath tumor. Malignant pathologies such as Ewing\'s sarcoma must be considered, especially in the setting of rapidly progressive symptoms or interval growth on serial imaging. Early diagnosis allows for the timely initiation of comprehensive oncological care. Long-term multidisciplinary follow-up is necessary for the surveillance of disease progression.

Ewing\'s sarcoma (ES) is a bone and soft tissue tumor that mainly occurs at a young age. The underlying cause of Ewing\'s sarcoma is the formation of fusion proteins between FET family genes and ETS family genes. Tumors with FET/ETS fusion genes can have defects in the DNA damage response and are sensitive to PARP inhibitors (PARPi). However, several studies have shown that PARPi alone is not sufficient to induce a meaningful antitumor response and that combinations of DNA-damaging agents with PARPi are required to achieve efficacy. Accordingly, preclinical studies have reported dramatic responses to PARPi treatment in combination with DNA-damaging agents such as temozolomide or irinotecan. Similarly, it has been previously reported that by generating reactive oxygen species, high-dose intravenous vitamin C (IVC) can induce DNA damage. This suggests that the combination of IVC with PARPi may increase genotoxic stress and enhance the antitumor response. In addition, unlike chemotherapeutic agents, IVC induces DNA damage selectively in cancer cells, and the side effects are significantly milder than those of chemotherapy. As ETS fusion-positive ES is deficient in faithful DNA repair, partly due to the interaction between ETS fusion products and PARP1, a PARPi plus IVC seems to be a logical and effective combination for the treatment of ETS fusion-positive ES. This paper reports significant responses to IVC (1-1.5 g/kg) in combination with PARPi (olaparib 300 mg BID or talazoparib 1 mg/day) in two patients with metastatic Ewing\'s sarcoma. The observations highlight an unmet therapeutic need for patients with advanced metastatic ES. The combination of PARPi with a selective DNA-damaging agent was effective in these cases. This case experience suggests that IVC may be incorporated into PARPi-based therapeutic strategies. Further studies are needed to confirm the efficacy of this combination in the treatment of Ewing sarcoma with ETS fusions.
Combining vitamin C with PARP inhibitors for Ewing sarcoma treatment: mechanistic insights and 2 case studies Ewing’s sarcoma is a type of bone and soft tissue tumor that commonly affects young people and it is often resistant to conventional therapy. In this study, clinical cancer scientists and oncologists investigated a new approach to treating this cancer by combining high-dose vitamin C with PARP inhibitors. High-dose vitamin C can damage the DNA of cancer cells and PARP inhibitors block the damaged DNA sites so they can’t be repaired and eventually this leads to cancer cells dying. The researchers found that when these two treatments were used together, there were significant improvements in two patients with advanced Ewing’s sarcoma. Importantly, the combination led to fewer side effects compared to standard chemotherapy, suggesting it might be a more tolerable treatment option. These findings suggest that combining high-dose intravenous vitamin C with PARP inhibitors could be a promising treatment for Ewing’s sarcoma. More research is needed to confirm these results, but this approach shows potential for helping patients with advanced forms of this type of cancer. This is the first clinical report demonstrating the benefits of using high-dose vitamin C with PARP inhibitors and the study emphasizes the importance of exploring more treatment options for this aggressive type of cancer and suggests that further investigations into this combined approach could lead to more effective and tolerable treatments for Ewing’s sarcoma.

Treating Ewing\'s Sarcoma of the thorax (Askin\'s tumor) with antineoplastic therapy in a malnourished cystic fibrosis patient colonized with Pseudomonas aeruginosa and Staphylococcus aureus may carry a significant potential for complications. We present the case of a known cystic fibrosis patient, diagnosed with Askin\'s tumor 5 years ago. Despite facing severe neutropenia, exacerbations of cystic fibrosis with Pseudomonas aeruginosa infections, and challenges in maintaining adequate caloric intake during the oncological treatment, the patient\'s outcome has been favorable. Chemotherapy doses had to be adjusted, and continuous antibiotic treatment was introduced throughout the course of therapy to reduce the frequency and intensity of exacerbations. Approximately 5 years after the cancer diagnosis, with no signs of relapse, the patient was started on CFTR (Cystic fibrosis transmembrane conductance regulator) modulator treatment. This intervention has successfully corrected the weight deficit. The coincidence of Ewing\'s sarcoma of the chest wall and cystic fibrosis in a single patient is 2.857 × 10-5% and to the best of our knowledge, this scenario has not been documented before.