Staufen1 controls mitochondrial metabolism via HIF2 α in embryonal rhabdomyosarcoma and promotes tumorigenesis
We report the novel and differential role of STAU1 in mitochondrial metabolism in cancerous versus non-malignant skeletal muscle cells (NMSkMCs). Specifically, our data show that STAU1 depletion reduces OXPHOS and inhibits proliferation of ERMS cells. Our findings further reveal the binding of STAU1 to several OXPHOS mRNAs which affects their stability. Indeed, STAU1 depletion reduced the stability of OXPHOS mRNAs, causing inhibition of mitochondrial metabolism. In parallel, STAU1 depletion impacted negatively the HIF2α pathway which further modulates mitochondrial metabolism. Exogenous expression of HIF2α in STAU1-deple...
Source: Cellular and Molecular Life Sciences : CMLS - October 17, 2023 Category: Cytology Authors: Shekoufeh Almasi Sahar SarmastiEmami Stephen Baird Zhibin Ning Daniel Figeys Jocelyn C ôté Kyle N Cowan Bernard J Jasmin Source Type: research

Staufen1 controls mitochondrial metabolism via HIF2 α in embryonal rhabdomyosarcoma and promotes tumorigenesis
We report the novel and differential role of STAU1 in mitochondrial metabolism in cancerous versus non-malignant skeletal muscle cells (NMSkMCs). Specifically, our data show that STAU1 depletion reduces OXPHOS and inhibits proliferation of ERMS cells. Our findings further reveal the binding of STAU1 to several OXPHOS mRNAs which affects their stability. Indeed, STAU1 depletion reduced the stability of OXPHOS mRNAs, causing inhibition of mitochondrial metabolism. In parallel, STAU1 depletion impacted negatively the HIF2α pathway which further modulates mitochondrial metabolism. Exogenous expression of HIF2α in STAU1-deple...
Source: Cellular and Molecular Life Sciences : CMLS - October 17, 2023 Category: Cytology Authors: Shekoufeh Almasi Sahar SarmastiEmami Stephen Baird Zhibin Ning Daniel Figeys Jocelyn C ôté Kyle N Cowan Bernard J Jasmin Source Type: research

Rhabdomyosarcoma of middle ear: No longer a radiological surprise!
Shilpa Sankhe, Ayush Jain, Subhash YadavJournal of Pediatric Neurosciences 2022 17(1):82-84An eight-year-old girl presented to the outpatient department with a history of decreased hearing from the right ear, bloody ear discharge, and facial deviation to the left side. On clinical examination, she was afebrile with conductive right-sided hearing loss. With a primary diagnosis of unsafe type of chronic suppurative otitis media in mind, computed tomography scan of the temporal bone was performed which showed an aggressive destructive lesion in the petrous temporal bone. Magnetic resonance imaging was then performed to look f...
Source: Journal of Pediatric Neurosciences - October 17, 2023 Category: Neuroscience Authors: Shilpa Sankhe Ayush Jain Subhash Yadav Source Type: research

GSE245495 A MYOD-SKP2 axis boosts tumorigenesis in fusion negative rhabdomyosarcoma by preventing differentiation through p57Kip2 targeting [Hi-ChIP]
Contributors : Silvia Pomella ; Matteo Cassandri ; Berkley E Gryder ; Rossella RotaSeries Type : Genome binding/occupancy profiling by high throughput sequencing ; OtherOrganism : Homo sapiensRhabdomyosarcoma (RMS) is a pediatric mesenchymal-derived malignancy encompassing Fusion Positive (FP)-RMS expressing PAX3/7-FOXO1 and Fusion Negative (FN)-RMS often mutated in the RAS pathway. RMS expresses the master myogenic transcription factor MYOD that, paradoxically, is unable to support differentiation while essential for tumor cell survival. We identify here SKP2, an oncogenic E3-ubiquitin ligase, as a critical driver of t...
Source: GEO: Gene Expression Omnibus - October 16, 2023 Category: Genetics & Stem Cells Tags: Genome binding/occupancy profiling by high throughput sequencing Other Homo sapiens Source Type: research

Viruses, Vol. 15, Pages 2094: Umbilical Cord Mesenchymal-Stem-Cell-Derived Exosomes Exhibit Anti-Oxidant and Antiviral Effects as Cell-Free Therapies
This study provides a promising therapeutic strategy for ucMSC-EXO in anti-oxidative stress and antiviral effects, which provides insight into extending the function of ucMSC-EXO in cell-free therapy. (Source: Viruses)
Source: Viruses - October 15, 2023 Category: Virology Authors: Yi Meng Chengcheng Li Yicong Liang Yu Jiang Haonan Zhang Jianhua Ouyang Wen Zhang Rumei Deng Qiuping Tan Xiaolan Yu Zhen Luo Tags: Article Source Type: research

Clinical Insight on Proton Therapy for Paediatric Rhabdomyosarcoma
(Source: Cancer Management and Research)
Source: Cancer Management and Research - October 10, 2023 Category: Cancer & Oncology Tags: Cancer Management and Research Source Type: research

Hedgehog pathway in sarcoma: from preclinical mechanism to clinical application
AbstractSarcomas are a diverse group of malignant neoplasms of mesenchymal origin. They develop rarely, but due to poor prognosis, they are a challenging and significant clinical problem. Currently, available therapeutic options have very limited activity. A better understating of sarcomas ’ pathogenesis may help develop more effective therapies in the future. The Sonic hedgehog (Shh) signaling pathway is involved in both embryonic development and mature tissue repair and carcinogenesis. Shh pathway inhibitors are presently used in the treatment of basal cell carcinoma. Its increase d activity has been demonstrated in ma...
Source: Journal of Cancer Research and Clinical Oncology - October 10, 2023 Category: Cancer & Oncology Source Type: research

Diagnosis of uterine sarcomas and rare uterine mesenchymal tumours with malignant potential. Guidelines of the French Sarcoma Group and Rare Gynaecological Tumours
Bull Cancer. 2023 Oct 6:S0007-4551(23)00348-X. doi: 10.1016/j.bulcan.2023.08.002. Online ahead of print.ABSTRACTThe landscape of uterine sarcomas is becoming more complex with the description of new entities associated with recurrent driver molecular alterations. Uterine sarcomas, in analogy with soft tissue sarcomas, are distinguished into complex genomic and simple genomic sarcomas. Leiomyosarcomas and undifferentiated uterine sarcomas belong to complex genomic sarcomas group. Low-grade and high-grade endometrial stromal sarcomas, other rare tumors associated with fusion transcripts (such as NTRK, PDGFB, ALK, RET ROS1) a...
Source: Bulletin du Cancer - October 8, 2023 Category: Cancer & Oncology Authors: Sabrina Croce Mojgan Devouassoux-Shisheboran Patricia Pautier Isabelle Ray-Coquard Isabelle Treilleux Agn ès Neuville Laurent Arnould Pierre-Alexandre Just Marie Aude Le Frere Belda Gerlinde Averous Agn ès Leroux Guillaume Bataillon Eliane Mery Delphine Source Type: research

Trans-Orbital EUS (with videos)
A 31-year-old male presented with acute cholecystitis and history of metastatic alveolar rhabdomyosarcoma. He previously underwent left orbital exenteration and facial radiation. Given the patient ’s malnutrition, he was deemed a non-surgical candidate. The patient chose to pursue EUS-guided gallbladder drainage. Due to radiation-induced trismus, he was not able to open his mouth more than 5mm. Bougie dilation of an existing gastrostomy was considered, however there was concern for poor hea ling and gastrostomy leak. (Source: Gastrointestinal Endoscopy)
Source: Gastrointestinal Endoscopy - October 6, 2023 Category: Gastroenterology Authors: Joshua L. Hudson, Judy A. Trieu, Andrew J. Gilman Source Type: research

Transorbital endoscopic ultrasonography (with video)
A 31-year-old man presented with acute cholecystitis and a history of metastatic alveolar rhabdomyosarcoma. He had previously undergone left orbital exenteration and facial radiation. Given the patient ’s malnutrition, he was deemed a nonsurgical candidate. The patient chose to pursue EUS-guided gallbladder drainage. Owing to radiation-induced trismus, he was not able to open his mouth more than 5 mm. Bougie dilatation of an existing gastrostomy was considered; however, there was concern about poor healing and gastrostomy leak. (Source: Gastrointestinal Endoscopy)
Source: Gastrointestinal Endoscopy - October 6, 2023 Category: Gastroenterology Authors: Joshua L. Hudson, Judy A. Trieu, Andrew J. Gilman Tags: At the focal point Source Type: research

Impact of Consolidative Radiation on Overall and Progression Free Survival in Pediatric, Adolescent and Young Adult Metastatic Bone and Soft Tissue Sarcoma
CONCLUSION: Consolidative RT was independently associated with improved OS and PFS and decreased risk of distant failure in children and AYA patients with metastatic sarcoma. Future work should evaluate biomarkers to optimize patient selection, timing and dose for consolidative RT.PMID:37797747 | DOI:10.1016/j.ijrobp.2023.09.007 (Source: Health Physics)
Source: Health Physics - October 5, 2023 Category: Physics Authors: Leslie Chang Anjali D'Amiano Rohini Bhatia Gayane Yenokyan Nicolas J Llosa Brian H Ladle Christian F Meyer Adam S Levin Christine A Pratilas Matthew Ladra Sahaja Acharya Source Type: research

GSE218274 PAX3-FOXO1 expression in endothelial progenitors dictates myogenic reprogramming and rhabdomyosarcoma identity
Series Type : Genome binding/occupancy profiling by high throughput sequencing ; Other ; Expression profiling by high throughput sequencingOrganism : Homo sapiens ; Mus musculusThis SuperSeries is composed of the SubSeries listed below. (Source: GEO: Gene Expression Omnibus)
Source: GEO: Gene Expression Omnibus - September 29, 2023 Category: Genetics & Stem Cells Tags: Genome binding/occupancy profiling by high throughput sequencing Other Expression profiling by high throughput sequencing Homo sapiens Mus musculus Source Type: research

Patient-derived organoids facilitating individual therapy in an adolescent with embryonal rhabdomyosarcoma of the cervix: a case report and literature review
Rhabdomyosarcoma (RMS) is a highly aggressive pediatric neoplasm that originates from striated muscle or undifferentiated mesenchymal cells. Based on its histopathological characteristics, the World Health Organization categorizes RMS into four distinct subtypes: embryonal RMS, alveolar RMS, pleomorphic RMS, and sclerosing/spindle cell RMS. Embryonal RMS represents the predominant subtype and primarily manifests in the head and neck region, with the genitourinary system being the subsequent most frequent site of occurrence. Embryonal rhabdomyosarcoma of the cervix (cERMS) is more insidious in the reproductive tract, and th...
Source: Frontiers in Oncology - September 29, 2023 Category: Cancer & Oncology Source Type: research

Pediatric presacral tumors with intraspinal extension: a rare entity with diagnostic challenges
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 includ...
Source: Acta Radiologica - September 27, 2023 Category: Radiology Authors: Ebinesh A Anjali Prakash Aanchal Ashta Anju Garg Abhishek Verma Prerna Padaliya Source Type: research