embryonal rhabdomyosarcoma
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2022 ◽  
pp. 107815522210740
Author(s):  
Dr. Arul Janani ◽  
Dr. Anandkumar Shruthilaya ◽  
Dr. Jayaraman Dhaarani ◽  
Dr. Scott X Julius

Introduction: Febrile episodes in oncology is common are mostly of infectious etiology requiring repeated investigations and escalation of antibiotics. But, drug induced fever occur more often than we think in oncological set-up. Case Report: A 5 year old male child with rhabdomyosarcoma, developed high grade fever spikes following Vincristine monotherapy. Infective etiology work up was negative and the fever responded to corticosteroids. Management and Outcome: He was treated with corticosteroids as premedication considering vincristine induced fever. The further courses of VCR- monotherapy were uneventful with steroids as premedication. Discussion: We present the case of vincristine induced fever in a child with embryonal rhabdomyosarcoma. Clinician’s should consider drug induced fever at appropriate conditions, to avoid leading to antibiotic resistance.


Author(s):  
Charles Odongo ◽  
raymond atwine ◽  
Martin Situma ◽  
ambrose okello ◽  
eugene ogwang ◽  
...  

Introduction: Cavernous hemangioma accounts for 0.6% of bladder tumors. We present a rare case Case Presentation: A 3-year-old girl presented with intravaginal swelling, dysuria, and hematuria. She received 26 cycles of VAC for embryonal rhabdomyosarcoma. Histopathology confirmed CH. Conclusion: CH should be considered in the differentials of childhood genitourinary masses.


2021 ◽  
Vol 22 (24) ◽  
pp. 13377
Author(s):  
Nada Ragab ◽  
Julia Bauer ◽  
Dominik S. Botermann ◽  
Anja Uhmann ◽  
Heidi Hahn

In the Ptch+/- mouse model for embryonal rhabdomyosarcoma (ERMS), we recently showed that oncogenic (onc) H-, K- or NRAS mutations do not influence tumor growth when induced at the advanced, full-blown tumor stage. However, when induced at the invisible ERMS precursor stage at 4 weeks of age, tumor development was enforced upon oncHRAS and oncKRAS but not by oncNRAS, which instead initiated tumor differentiation. These data indicate that oncRAS-associated processes differ from each other in dependency on the isoform and their occurrence during tumor development. Here, we investigated the outcome of oncNRAS induction at an earlier ERMS precursor stage at 2 weeks of age. In this setting, oncNRAS accelerates tumor growth because it significantly shortens the ERMS-free survival and increases the ERMS incidence. However, it does not seem to alter the differentiation of the tumors. It is also not involved in tumor initiation. Together, these data show that oncNRAS mutations can accelerate tumor growth when targeting immature ERMS precursors within a specific time window, in which the precursors are permissive to the mutation and show that oncNRAS-associated processes differ from each other in dependency on their occurrence during tumor development.


Author(s):  
Esha Jain ◽  
Ali Al-Tarbsheh ◽  
Jozef Oweis ◽  
Hala Abdelwahab ◽  
Boris Shkolnik

Rhabdomyosarcoma is an uncommon soft tissue sarcoma that rarely presents in adults. Clinical presentation is dependent on site and size. We present the case of a woman who presented with acute-onset dyspnoea and whose pathology report confirmed embryonal rhabdomyosarcoma (ERMS) seen as an incidental finding on chest computed tomography. We also describe the clinical, laboratory and radiological work-up conducted to diagnose and manage ERMS in the critical care setting.


2021 ◽  
Author(s):  
Reshma Taneja ◽  
Hsin Yao Chiu ◽  
Amos Hong Pheng Loh

Embryonal rhabdomyosarcoma (ERMS) is characterized by a failure of cells to complete skeletal muscle differentiation. Although ERMS cells are vulnerable to oxidative stress, the relevance of mitochondrial calcium homeostasis in oncogenesis is unclear. Here, we show that ERMS cell lines as well as primary tumours exhibit elevated expression of the Mitochondrial Calcium Uniporter (MCU). MCU knockdown resulted in impaired mitochondrial calcium uptake and a reduction in mitochondrial reactive oxygen species (mROS) levels. Phenotypically, MCU knockdown cells exhibited reduced cellular proliferation and motility, with an increased propensity to differentiate in vitro and in vivo. RNA-sequencing of MCU knockdown cells revealed a significant reduction in genes involved in TGF? signalling that play prominent roles in oncogenesis and inhibition of myogenic differentiation. Interestingly, modulation of mROS production impacted TGF? signalling. Our study elucidates mechanisms by which mitochondrial calcium dysregulation promotes tumour progression and suggests that targeting the MCU complex to restore mitochondrial calcium homeostasis could be a therapeutic avenue in ERMS.


Author(s):  
Narasimhan P. Agaram ◽  
Shih‐Chiang Huang ◽  
William D. Tap ◽  
Leonard H. Wexler ◽  
Cristina R. Antonescu

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Maniraj Jeyaraju ◽  
Regina Ann Macatangay ◽  
Ashley Taylor-King Munchel ◽  
Teresa Anne York ◽  
Elizabeth A. Montgomery ◽  
...  

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically include a range of differentiation in rhabdomyoblasts from primitive to terminally differentiated forms, and the latter become more prominent after chemotherapy-induced cytodifferentiation. Several reports have shown therapy-related cytodifferentiation to portend a good prognosis in ERMS. We discuss the case of a pediatric patient who presented with ERMS of the orbit. Although her tumor showed extensive posttreatment cytodifferentiation and several other good prognostic clinicopathologic factors, it pursued an aggressive course, resulting in early metastasis and death. This case represents an unusual course and may be instructive as to the clinicopathologic features impacting prognostication, and ultimately the biology, of this aggressive family of tumors.


2021 ◽  
pp. 101953
Author(s):  
Jeffrey Johnson ◽  
Aaron Brant ◽  
Christina Sze ◽  
Ananias Motta Burgos ◽  
Natallia Sheuka ◽  
...  

Cells ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 2956
Author(s):  
Simona Camero ◽  
Giulia Vitali ◽  
Paola Pontecorvi ◽  
Simona Ceccarelli ◽  
Eleni Anastasiadou ◽  
...  

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood. Recently, we demonstrated the overexpression of both DNA methyltransferase 3A (DNMT3A) and 3B (DNMT3B) in RMS tumour biopsies and cell lines compared to normal skeletal muscle. Radiotherapy may often fail due to the abnormal expression of some molecules able to drive resistance mechanisms. The aim of this study was to analyse the involvement of DNMT3A and DNMT3B in radioresistance in RMS. RNA interference experiments against DNMT3A/3B were performed in embryonal RMS cells, upon ionizing radiation (IR) exposure and the effects of the combined treatment on RMS cells were analysed. DNMT3A and DNMT3B knocking down increased the sensitivity of RMS cells to IR, as indicated by the drastic decrease of colony formation ability. Interestingly, DNMT3A/3B act in two different ways: DNMT3A silencing triggers the cellular senescence program by up-regulating p16 and p21, whilst DNMT3B depletion induces significant DNA damage and impairs the DNA repair machinery (ATM, DNA-PKcs and Rad51 reduction). Our findings demonstrate for the first time that DNMT3A and DNMT3B overexpression may contribute to radiotherapy failure, and their inhibition might be a promising radiosensitizing strategy, mainly in the treatment of patients with metastatic or recurrent RMS tumours.


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