The Other Site of Rhabdomyosarcoma.

Background: Rhabdomyosarcoma (RMS) is a rare malignant soft tissue sarcoma (STS), accounting for almost 50% of pediatric STSs. Due to its heterogeneity, RMS presents challenges in diagnosis and treatment, with prognosis varying depending on multiple factors. Tumors localized in the other site (OTH)-including the paraspinal, perianal, thoracic, abdominal, pelvic, and perineal regions-are generally classified as unfavorable.

Desmoid-type fibromatosis in an uncommon location: A case report of shoulder involvement misdiagnosed as rhabdomyosarcoma.

Introduction And Importance: Desmoid-type fibromatosis is an uncommon tumor characterized by its local invasiveness, with shoulder involvement being notably infrequent. The optimal treatment strategy for this tumor remains a topic of ongoing debate.

Case Presentation: A 47-year-old Tunisian woman with a history of hypothyroidism, presented with pain and swelling in her left shoulder for a year.

Evaluating Circulating Biomarkers for Diagnosis, Prognosis, and Tumor Monitoring in Pediatric Sarcomas: Recent Advances and Future Directions.

Pediatric sarcomas present a significant challenge in oncology. There is an urgent need for improved therapeutic strategies for high-risk patients and better management of long-term side effects for those who survive the disease. Liquid biopsy is emerging as a promising tool to optimize treatment in these patients by offering non-invasive, repeatable assessments of disease status.

Dependence of PAX3-FOXO1 chromatin occupancy on ETS1 at important disease-promoting genes exposes new targetable vulnerability in Fusion-Positive Rhabdomyosarcoma.

Rhabdomyosarcoma (RMS), a malignancy of impaired myogenic differentiation, is the most common soft tissue pediatric cancer. PAX3-FOXO1 oncofusions drive the majority of the clinically more aggressive fusion-positive rhabdomyosarcoma (FP-RMS). Recent studies have established an epigenetic basis for PAX3-FOXO1-driven oncogenic processes.

Metastatic-Site Radiation Therapy for Ewing Sarcoma and Rhabdomyosarcoma: Consensus Guidelines From the National Pediatric Cancer Foundation.

Purpose: Despite the urgent need for improved outcomes in patients with metastatic Ewing sarcoma (EWS) and rhabdomyosarcoma (RMS), it is unknown how to best approach metastatic-site radiation therapy for these patients and whether such treatment provides a significant oncologic benefit that outweighs the toxicities.

Methods And Materials: We gathered a panel of pediatric radiation oncologists from academic hospitals to identify and discuss current controversies regarding the role of radiation in the management of metastatic EWS and RMS. The panel reviewed existing clinical data and ongoing trials to address 5 key questions: (1) the role of whole lung irradiation (WLI) in treating lung metastases; (2) the number of metastatic sites warranting radiation therapy and the radicality of such an approach; (3) radiation techniques, including stereotactic body radiation therapy (SBRT); (4) the timing of metastatic-site radiation therapy; and (5) the utility of metastatic-site radiation therapy for relapsed metastatic disease.

The Australian paediatric brachytherapy experience: A pathway to a national programme.

Introduction: Paediatric cancers are rare, and most children requiring radiation therapy receive external beam radiation (EBRT). Although EBRT may offer organ preservation compared to surgery, it can be associated with significant late effects. Image-guided brachytherapy is a highly specialised technique offering both organ preservation and dose conformity to minimise late toxicity.

Primary Gastric Alveolar Rhabdomyosarcoma: A Potent Mimicker of Neuroendocrine Carcinoma.

Alveolar rhabdomyosarcoma (ARMS) is a malignant tumor with skeletal muscle differentiation that usually occurs in soft tissues of the extremities or trunk. To date, only a few cases of primary gastric ARMS have been reported. Herein, we describe a case of ARMS in a man in his 80s and present a literature review.

Embryonal rhabdomyosarcoma of prostate combined with prostatic abscess in an adult patient: a case report and literature review.

Background: Embryonal rhabdomyosarcoma (ERMS) of the prostate is a rare and aggressive malignant tumor in adults with a poor prognosis in general. The main presenting symptoms are dysuria and acute urinary retention. Prostate ERMS is easily misdiagnosed, which leads to delays in treatment.

Amputation and limb-sparing surgery in childhood sarcoma: Post-operative imaging appearances and complications.

Limb-sparing surgery and amputation are common surgical techniques used to achieve local tumour control in childhood primary bone and soft tissue malignancy of the limbs. The interpretation of post-operative limb imaging in these frequently complex cases is assisted by knowledge of the surgical techniques employed. This review discusses the rationale underpinning the most common surgical techniques used for these patients as well as their expected post-operative imaging appearance and complications.

Cardiac embryonal rhabdomyosarcoma in a domestic shorthair cat.

Rhabdomyosarcomas (RBMs) are rare neoplasms arising from striated muscle tissues in domestic animals and are exceptionally uncommon, affecting the heart of domestic cats. A case of cardiac embryonal RBM was detected in a two-year-old female domestic shorthair cat. The cat exhibited dyspnea, anorexia, pericardial and pleural effusions, ultimately succumbing to severe respiratory arrest.

The role of radiotherapy in multimodal treatment of non-rhabdomyosarcoma soft tissue sarcomas in children: A real life report from a tertiary center.

Objective: The current treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is a multimodal risk-based approach. Today, smaller fields and lower doses of radiotherapy (RT) have become standard. In this study, it was aimed to evaluate the treatment outcomes and toxicity profile in children with NRSTS that received RT as a part of multimodal therapy.

Correction: Chisholm et al. Frontline and Relapsed Rhabdomyosarcoma (FaR-RMS) Clinical Trial: A Report from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG). 2024, , 998.

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Primary Mast Cell Sarcoma of the Maxillary Sinus and Gingiva Mimicking Malignant Neuroendocrine Tumor: A Case Report.

Mast cell sarcoma (MCS) is an extremely rare and aggressive malignancy primarily affecting bones, with limited literature associating it with neuroendocrine marker expression. This report presents a rare case of MCS arising in the maxillary sinus and gingiva. A 74-year-old man presented with a progressively enlarging ulcer on the right-sided upper gingiva.

A High-Throughput Drug Repurposing Strategy to Treat TBX2 and/or TBX3 Dependent Cancers.

Background: The highly homologous T-box transcription factors TBX2 and TBX3 are critical for embryonic development, and their overexpression in postnatal tissues contributes to a wide range of malignancies, including melanoma and rhabdomyosarcoma. Importantly, when TBX2 and TBX3 are depleted in cancers where they are overexpressed, the malignant phenotype is inhibited, and they have therefore been regarded as druggable targets. However, the time and costs associated with de novo drug development are challenging and result in drugs that are costly, especially for patients in low- and middle-income countries.

Metronomic chemotherapy for paediatric extracranial solid tumours: a systematic review and meta-analysis of randomised clinical trials.

Background: Metronomic chemotherapy ('less is more, regularly') could be an alternative to the maximum tolerated dose ('the more, the better') in the chemotherapeutic cancer treatment of high-risk malignant solid extracranial tumours in children or young adults.

Objective: To evaluate the efficacy of metronomic chemotherapy compared with placebo or stop treatment in paediatric patients with extracranial malignant solid tumours.

Methods: We searched the databases MEDLINE and CENTRAL on 8 September 2023 and included randomised clinical trials (RCTs).