This report presents a case of embryonal rhabdomyosarcoma(e RMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital.The images pro...This report presents a case of embryonal rhabdomyosarcoma(e RMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital.The images provided from the computed tomography showed a heterogeneous mass with soft-tissue density,occupying part of the left half of the face inside the maxillary sinus,and infiltrating and destroying the bone structure of the maxillary sinus,left orbit,ethmoidal cells,nasal cavity,and sphenoid sinus.An analysis of the histological sections revealed an undifferentiated malignant neoplasm infiltrating the skeletal muscle tissue.The immunohistochemical analysis was positive for the antigens:MyoD 1,myogenin,desmin,and Ki67(100% positivity in neoplastic cells),allowing the identification of the tumour as an eR MS.The treatment protocol included initial chemotherapy followed by radiotherapy and finally surgery.The total time of the treatment was nine months,and in 18-mo of follow-up period did not show no local recurrences and a lack of visual impairment.展开更多
Aural polyps are a common clinical entity encountered by otorhinolaryngologist in daily practice. Polyps are frequently seen in paediatric patients, usually inflammatory in nature. In children rhabdomyosarcomas (RMS) ...Aural polyps are a common clinical entity encountered by otorhinolaryngologist in daily practice. Polyps are frequently seen in paediatric patients, usually inflammatory in nature. In children rhabdomyosarcomas (RMS) can mimic all the cinical features of chronic suppurative otitis media which usually present as external auditory canal mass or polyp. Here we present a case where a male child presented with recurrence of polyp in left ear which was finally diagnosed as embryonal rhabdomyosarcoma which is a rare and invariably fatal disease in children. Through this article we intend to highlight the failures and delay committed in attaining diagnosis in this patient in spite of multispecialty evaluation involving repeated imaging and histopathological correlation. Our experience with embryonal rhabdomyosarcoma throws light on the high vigilance required in handling the aural polyps in pediatric population as early diagnosis and treatment are the key elements for successful outcomes.展开更多
Prostate embryonal rhabdomyosarcoma(ERMS) is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethra obstruction, and ...Prostate embryonal rhabdomyosarcoma(ERMS) is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethra obstruction, and systemic spread, commonly to the lungs, liver, and bone. ERMS of the prostate is a commonly occurring tumor in infants and children. It is rarely seen in adults. Here, we report on a case of the prostate ERMS in a 27-year-old man.展开更多
Introduction:?Embryonal rhabdomyosarcoma (RMS) of the uterine cervix is a rare and extremely aggressive malignant entity. However, prognosis seems to be improved with the use of multimodality approach treatment. Cases...Introduction:?Embryonal rhabdomyosarcoma (RMS) of the uterine cervix is a rare and extremely aggressive malignant entity. However, prognosis seems to be improved with the use of multimodality approach treatment. Cases: We report the cases of 50- and 51-year-old women, presenting with vaginal bleeding and mass. The two patients underwent radical surgery. Histological examination revealed RMS of uterine cervix. Treatment was effective in the first case but despite the chemotherapy, the second patient died two months later. Discussion and Conclusions: The prognosis of the cervical embryonal RMS depends on clinical and histological features. The current treatment protocols are based on trials done on pediatric patients. Studies on embryonal RMS treatment and outcomes are limited in women over forty years. In patients with unfavorable prognosis characteristics, the multimodality approach including surgery, adjuvant chemo and radiotherapy can be effective. Otherwise, surgery alone can effectively be proposed.展开更多
目的:探讨survivin基因在儿童胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)中的生物学作用。方法:采用survivin免疫组织化学检测ERMS肿瘤组织和正常骨骼肌组织的表达情况,小干扰RNA(siRNA)介导的survivin抑制对ERMS RD细胞凋亡...目的:探讨survivin基因在儿童胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)中的生物学作用。方法:采用survivin免疫组织化学检测ERMS肿瘤组织和正常骨骼肌组织的表达情况,小干扰RNA(siRNA)介导的survivin抑制对ERMS RD细胞凋亡的影响,原位末端标记法(TUNEL)检测RD细胞凋亡率,Western印迹法检测RD细胞caspase-3和caspase-9蛋白表达水平。结果:Sur vivin在ERMS中高表达而在正常骨骼肌组织中不表达,特异性siRNA干扰sur v iv in表达导致RD细胞凋亡显著增加,survivin表达的下调导致更高的凋亡率,survivin表达的下调增加了caspase-3的活性,对caspase-9的活性无影响。结论:Survivin过表达可能通过抑制细胞凋亡而与ERMS发病机制有关。展开更多
文摘This report presents a case of embryonal rhabdomyosarcoma(e RMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital.The images provided from the computed tomography showed a heterogeneous mass with soft-tissue density,occupying part of the left half of the face inside the maxillary sinus,and infiltrating and destroying the bone structure of the maxillary sinus,left orbit,ethmoidal cells,nasal cavity,and sphenoid sinus.An analysis of the histological sections revealed an undifferentiated malignant neoplasm infiltrating the skeletal muscle tissue.The immunohistochemical analysis was positive for the antigens:MyoD 1,myogenin,desmin,and Ki67(100% positivity in neoplastic cells),allowing the identification of the tumour as an eR MS.The treatment protocol included initial chemotherapy followed by radiotherapy and finally surgery.The total time of the treatment was nine months,and in 18-mo of follow-up period did not show no local recurrences and a lack of visual impairment.
文摘Aural polyps are a common clinical entity encountered by otorhinolaryngologist in daily practice. Polyps are frequently seen in paediatric patients, usually inflammatory in nature. In children rhabdomyosarcomas (RMS) can mimic all the cinical features of chronic suppurative otitis media which usually present as external auditory canal mass or polyp. Here we present a case where a male child presented with recurrence of polyp in left ear which was finally diagnosed as embryonal rhabdomyosarcoma which is a rare and invariably fatal disease in children. Through this article we intend to highlight the failures and delay committed in attaining diagnosis in this patient in spite of multispecialty evaluation involving repeated imaging and histopathological correlation. Our experience with embryonal rhabdomyosarcoma throws light on the high vigilance required in handling the aural polyps in pediatric population as early diagnosis and treatment are the key elements for successful outcomes.
文摘Prostate embryonal rhabdomyosarcoma(ERMS) is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethra obstruction, and systemic spread, commonly to the lungs, liver, and bone. ERMS of the prostate is a commonly occurring tumor in infants and children. It is rarely seen in adults. Here, we report on a case of the prostate ERMS in a 27-year-old man.
文摘Introduction:?Embryonal rhabdomyosarcoma (RMS) of the uterine cervix is a rare and extremely aggressive malignant entity. However, prognosis seems to be improved with the use of multimodality approach treatment. Cases: We report the cases of 50- and 51-year-old women, presenting with vaginal bleeding and mass. The two patients underwent radical surgery. Histological examination revealed RMS of uterine cervix. Treatment was effective in the first case but despite the chemotherapy, the second patient died two months later. Discussion and Conclusions: The prognosis of the cervical embryonal RMS depends on clinical and histological features. The current treatment protocols are based on trials done on pediatric patients. Studies on embryonal RMS treatment and outcomes are limited in women over forty years. In patients with unfavorable prognosis characteristics, the multimodality approach including surgery, adjuvant chemo and radiotherapy can be effective. Otherwise, surgery alone can effectively be proposed.
文摘目的:探讨survivin基因在儿童胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)中的生物学作用。方法:采用survivin免疫组织化学检测ERMS肿瘤组织和正常骨骼肌组织的表达情况,小干扰RNA(siRNA)介导的survivin抑制对ERMS RD细胞凋亡的影响,原位末端标记法(TUNEL)检测RD细胞凋亡率,Western印迹法检测RD细胞caspase-3和caspase-9蛋白表达水平。结果:Sur vivin在ERMS中高表达而在正常骨骼肌组织中不表达,特异性siRNA干扰sur v iv in表达导致RD细胞凋亡显著增加,survivin表达的下调导致更高的凋亡率,survivin表达的下调增加了caspase-3的活性,对caspase-9的活性无影响。结论:Survivin过表达可能通过抑制细胞凋亡而与ERMS发病机制有关。
