Malignant tumors
Archana Singal, Shekhar Neema, Piyush Kumar in Nail Disorders, 2019
Ewing sarcoma is a rare tumor almost exclusively seen in persons under 20 years of age. Only about 1% occur in the small bones of the hands and feet. Pain and swelling with low-grade fever are the leading symptoms. Two forms are distinguished: skeletal and extraskeletal Ewing sarcoma. The extraskeletal Ewings sarcoma is cytogenetically and molecular genetically identical to peripheral primitive neuroectodermal tumor (PNET), but is less well differentiated histologically. Its clinical presentation is a neoplasm with a diameter of 5–10 cm. One case of probable skeletal Ewing sarcoma was observed in the tip of a toe radiographically causing lytic lesions.120 The pulp was swollen and ulcerated. A subungual Ewing sarcoma was observed by F. Facchetti (unpublished 2012). Treatment was successful by amputation.121 Another recent case showed complete destruction of the nail unit.122 The clinical differential diagnosis includes osteomyelitis, tuberculosis, enchondroma, and a variety of benign tumors. Treatment is by generous local excision and chemotherapy.
Long-Term Outcomes and Prognostic Factors in Patients with Indications for Particle Therapy in Sarcomas
Manjit Dosanjh, Jacques Bernier in Advances in Particle Therapy, 2018
In a recent clinical trial on PBT for Ewing’s sarcoma, an estimated five-year OS, LC and distant-metastasis-free survival (DMFS) of 83.0%, 81.5% and 76.4%, respectively, was reported. Metastatic status at diagnosis was found to be a significant prognostic factor for five-year LC (p = 0.003). A tumour volume larger than 200 millilitres was associated with decreased DMFS (p = 0.03) [89]. These outcomes after PBT are comparable with analyses on treatment with RT for Ewing’s sarcoma reporting a three-year LC of 61% for patients presenting with metastatic disease and 84% for patients without metastases [90]. A higher risk of local relapse for patients with tumours larger than 200 millilitres compared to small tumours (subdistribution HR of 1.8) was also reported for patients treated with conventional RT [91].
Integrative hyperthermia treatments for different types of cancer
Clifford L. K. Pang, Kaiman Lee in Hyperthermia in Oncology, 2015
Surgical resection is the primary means for the treatment of osteosarcomas. Amputation and disarticulation are the most common methods. However, with the progress of chemotherapy in recent years, some researchers have begun applying resection of tumor segment or total femur resection, with artificial prosthetic for replacement. Limb salvage has become a major operation. Conduct preoperative standardized chemotherapy for 6–8 weeks, and then implement tumor resection. Resection margin is required to be radical or extensive. Artificial joint replacement is commonly used for bone defects. If limb salvage is not suitable or in the case that there is no condition for limb salvage, amputation should be decisively implemented, but postoperative chemotherapy has to be applied. Chondrosarcoma treatment focuses on surgery, and the efficacy depends on the breadth of tumor resection and malignancy of histological grade. Ewing’s sarcoma is very sensitive to radiation and chemotherapy. In current treatment protocols, the most advocated is neoadjuvant chemotherapy combined with extensive or radical resection of tumor. The therapeutic principle of treatment for bone malignant fibrous histiocytoma is similar to that of osteosarcoma.
Primary tumor volume measurements in Ewing sarcoma: MRI inter- and intraobserver variability and comparison with FDG-PET
Published in Acta Oncologica, 2018
Ömer Kasalak, Jelle Overbosch, Andor W. J. M. Glaudemans, Ronald Boellaard, Paul C. Jutte, Thomas C. Kwee
Ewing sarcoma is a high grade primary tumor of bone, most commonly with soft tissue extension [1]. In rare cases the lesion is purely in the soft tissue [1]. Its peak incidence is during adolescence and young adulthood [1]. The overall annual incidence of Ewing sarcoma is approximately 2.93 cases/1,000,000 [2]. Metastatic status at diagnosis is the strongest prognostic factor across different treatment strategies [1]. Five-year overall survival remains 30% for patients with initially metastatic disease [1]. Accurate staging, for which imaging plays an important role, is thus important for correct prognostication and treatment planning. Primary tumor volume has also been recognized as an important and independent prognostic factor, both in localized disease and primary disseminated multifocal Ewing sarcoma [1]. In Europe, primary tumor volume is even used to tailor maintenance chemotherapy in patients with localized disease [1]. The optimal cutoff value for prognostic stratification is still underinvestigated, although a primary tumor volume of more than 200 mL has been proposed by authoritative experts to identify those patients with a worse outcome [1], which is based on several previous studies [3–6]. The imaging modality at which these measurements should be done is also unclear, although they are currently usually performed at magnetic resonance imaging (MRI), which is regarded as the optimal radiological modality for local evaluation of Ewing sarcoma, thanks to its high contrast resolution [7].
Targeting the DNA damage response in pediatric malignancies
Published in Expert Review of Anticancer Therapy, 2022
Jenna M Gedminas, Theodore W Laetsch
Ewing sarcoma is the second most common primary bone tumor of childhood and adolescence [80]. The tumor is defined by a chromosomal translocation fusing the EWSR1 gene to a member of the ETS family of transcription factors, most commonly FLI1. Outside of this translocation, the tumor has a very low mutational burden; however, the EWS-FLI1 transcription factor leads to the dysregulation of several genes involved in the response to DNA damage including CHK2, MAP4K2, ABL1, and ATM [81–83]. Ewing sarcoma is also known to have high levels of endogenous replication stress, making the cells highly reliant on the ATR pathway for the repair of the resultant DNA damage and maintenance of cell viability [29,84]. Gorthi et al. has shown that Ewing sarcoma cells have an increased accumulation of R-loops which are known to be associated with high levels of DNA damage [84]. This was associated with increased RNAPII binding at the same site as the R-loops, consistent with the knowledge that both EWSR1 and EWS-FLI1 regulate RNAPII elongation. Unresolved R-loops can lead to stalling of the replication fork, furthering the reliance on DNA damage repair [84].
Diverse outcomes in extra-cranial rhabdoid tumors: A single institute experience
Published in Pediatric Hematology and Oncology, 2022
Yoshiki Katsumi, Tomoko Iehara, Yasumichi Kuwahara, Kunihiko Tsuchiya, Eiichi Konishi, Hajime Hosoi
Case 4: A 10-month-old female presenting with fever and recurrent vomiting was found to have a tumor surrounding the ureter and iliac artery and vein by MRI (Supplemental Figure S1E). Complete resection was impossible because of the adhered vessels. After partial resection, the patient was initially diagnosed with Ewing sarcoma. Subsequent RT diagnosis was based on the pathological findings of prominent nucleoli, oval nuclei with mitotic figures, and SMARCB1-negative expression (Supplemental Figure S2D). Five cycles of alternative therapy with vincristine/doxorubicin/cyclophosphamide and ifosfamide/carboplatin/etoposide, concurrent with local radiotherapy were administered. Complete remission was eventually achieved. The patient has survived for over 11 years without recurrence.
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