Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these canc...Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these cancers are diagnosed in children under 6 years of age, with a slight male predominance. Materials and Methods: This is a retrospective descriptive study of 10 cases of RMS collected in the pediatric hematology and oncology department of the Oujda university hospital, over a 5-year period, running from January 2018 to December 2022. Results: The median age at diagnosis was 3 years, with a sex ratio of 1. The mean time to diagnosis was 2 months. The most common site was the head and neck (50%), followed by the genitourinary tract (20%), the extremities (20%) and finally the abdomen (10%). The most frequent mode of discovery was a mass or swelling found in 90% of patients (all sites included), followed by exophthalmos in 30% of cases. At the diagnostic stage, CT scans were performed in 70% of cases and MRI in 5 patients (50%). Histological diagnosis was determined by immunohistochemical pathology in all our patients, with a predominance of embryonal (70%) versus alveolar (20%) and spindle cell types (10%). All patients underwent an extension workup, and a cervico-thoraco-abdominopelvic CT was performed in all patients (100%);MRI was performed in 2 patients (20%);lymph node involvement was present in 5 patients (50%). Metastases at the time of diagnosis were noted in only 1 patient (10%), who simultaneously presented with two metastatic sites;testicular and abdominal wall. Sixty percent of patients presented with advanced disease (high risk) and 40% with standard risk. Chemotherapy was used in all patients (100%), with upfront tumor resection performed in 40%. Fifty percent of patients received radiotherapy at a mean dose of 43 Gy, with the orbit the most frequently irradiated area (30%). All patients underwent CTscan and/or MRI and/or ultrasound surveillance. Follow-up during and after treatment was marked by complete remission in 8patients, loss of sight in one patient, and one patient died as a result of progressive disease. Conclusion: RMS is a malignant tumor of striated muscle. The epidemiological and clinical features of this tumor in our study are generally similar to those described in the literature. Management of these tumors requires multidisciplinary collaboration involving oncopediatric, radiologist, pediatric surgeon, pathologist and radiotherapist.展开更多
BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchyma...BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchymal cells.It was first re-ported in postmenopausal women in 1970,and fewer than 50 postmenopausal patients have been reported to date.CASE SUMMARY A 68-year-old multiparous female was admitted to the hospital on October 11,2023,with the chief complaint of a mass causing vaginal prolapse with incomplete urination that had persisted for 4 months.The vaginal mass was approximately the size of a pigeon egg;after lying down,the vaginal mass retracted.Complete resection was performed,and vaginal pleomorphic RMS was diagnosed based on pathology and immunohistochemical staining features.The patient is currently undergoing chemotherapy.The present study also reviewed the clinical,histolo-gical,and immunohistochemical features and latest treatment recommendations for vaginal RMS.Any abnormal vaginal mass should be promptly investigated through pelvic examination and appropriate imaging.The current initial treat-ment for vaginal RMS is biopsy and primary chemotherapy.CONCLUSION When surgery is planned for vaginal RMS,an organ-preserving approach should be considered.展开更多
BACKGROUND Rhabdomyosarcoma is a tumor of mesenchymal origin.Secondary leukemia is a complication of previous transformation to other hematologic disorders or is a treatment-related acute myeloid leukemia secondary to...BACKGROUND Rhabdomyosarcoma is a tumor of mesenchymal origin.Secondary leukemia is a complication of previous transformation to other hematologic disorders or is a treatment-related acute myeloid leukemia secondary to cytotoxic chemotherapy or radiation therapy for other malignancies.CASE SUMMARY We present the case of a 36-year-old female patient who was diagnosed with rhabdomyosarcoma and acute myeloid leukemia.Further disease progression was observed after multiline chemotherapy.Eventually,the patient suffered cerebral hemorrhage,which resulted in death.CONCLUSION The incidence of rhabdomyosarcoma in adults is extremely low,and secondary leukemia caused by rhabdomyosarcoma is even rarer.Secondary leukemia has a very poor prognosis and a low overall survival rate.展开更多
Malignant mesenchymal tumors (MTM) in children represent 5% to 10% of malignant tumors in children. They constitute a heterogeneous group of tumors of various differentiations depending on their supposed tissue of ori...Malignant mesenchymal tumors (MTM) in children represent 5% to 10% of malignant tumors in children. They constitute a heterogeneous group of tumors of various differentiations depending on their supposed tissue of origin. They mainly include tumors of muscular origin, those derived from connective, vascular, nervous, or adipose tissue. Rhabdomyosarcoma (RMS) is the most common mesenchymal tumor in children and adolescents (60% to 70% of them). And it accounts for 5.8% of all malignant solid tumors in children. Almost half of rhabdomyosarcomas occur in the head and neck. The prognosis for this type of tumor is particularly poor. A case of rhabdomyosarcoma in the mandible with extension to the abdominal wall and unilateral testis in a 6-month-old infant is reported with evolution since birth. It is a purplish lesion at the level under the right chin which was initially taken for vascular malformation, evolving very quickly towards a mandibular mass deforming the painful face with inflammatory signs, followed by the appearance of a hard swelling under the skin on the left flank taking on the same aspect of the mandibular mass. This observation illustrates the need to know how to systematically think about tumor causes in the face of atypical aspects and to carry out an anatomopathological examination.展开更多
目的:观察人结直肠癌组织磷酸化哺乳动物雷帕霉素靶蛋白(phosphorylated mammalian target of rapamycin,p-mTOR)、表皮生长因子受体(EGFR)、生长因子受体结合蛋白2(Grb2)和血管内皮生长因子(VEGF)等的表达,并探讨其可能的临床意义。方...目的:观察人结直肠癌组织磷酸化哺乳动物雷帕霉素靶蛋白(phosphorylated mammalian target of rapamycin,p-mTOR)、表皮生长因子受体(EGFR)、生长因子受体结合蛋白2(Grb2)和血管内皮生长因子(VEGF)等的表达,并探讨其可能的临床意义。方法:构建含有185例结直肠癌标本的组织芯片,免疫组化法检测结直肠癌组织及癌旁组织中EGFR、Grb2、p-mTOR和VEGF的表达,并分析不同结直肠癌临床病理学特征下(如年龄、性别、浸润深度、淋巴转移、临床分期和分化程度)各自的表达情况,探讨可能的临床意义。结果:EGFR、Grb2、p-mTOR和VEGF在结直肠癌旁组织中呈少量表达或不表达,在结直肠癌组织中的表达率分别为21.1%、44.9%、42.2%和54.1%,明显高于癌旁组织(P<0.05)。结直肠癌患者不同性别、年龄、肿瘤分化程度下EGFR、Grb2、p-mTOR和VEGF表达率无统计学差异;不同浸润深度和临床分期下EGFR的表达率有统计学差异(P<0.05);不同浸润深度、淋巴转移和临床分期下p-mTOR、VEGF表达率均有统计学差异(P<0.05)。结直肠癌组织EGFR/Grb2/p-mTOR/VEGF蛋白两两间均有一定的相关性(r=0.245～0.567,P<0.05)。结论:EGFR、Grb2、p-mTOR和VEGF表达与结直肠癌的发生、发展相关,值得进一步研究以作为结直肠癌肿瘤靶向治疗新的作用靶点。展开更多
文摘Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these cancers are diagnosed in children under 6 years of age, with a slight male predominance. Materials and Methods: This is a retrospective descriptive study of 10 cases of RMS collected in the pediatric hematology and oncology department of the Oujda university hospital, over a 5-year period, running from January 2018 to December 2022. Results: The median age at diagnosis was 3 years, with a sex ratio of 1. The mean time to diagnosis was 2 months. The most common site was the head and neck (50%), followed by the genitourinary tract (20%), the extremities (20%) and finally the abdomen (10%). The most frequent mode of discovery was a mass or swelling found in 90% of patients (all sites included), followed by exophthalmos in 30% of cases. At the diagnostic stage, CT scans were performed in 70% of cases and MRI in 5 patients (50%). Histological diagnosis was determined by immunohistochemical pathology in all our patients, with a predominance of embryonal (70%) versus alveolar (20%) and spindle cell types (10%). All patients underwent an extension workup, and a cervico-thoraco-abdominopelvic CT was performed in all patients (100%);MRI was performed in 2 patients (20%);lymph node involvement was present in 5 patients (50%). Metastases at the time of diagnosis were noted in only 1 patient (10%), who simultaneously presented with two metastatic sites;testicular and abdominal wall. Sixty percent of patients presented with advanced disease (high risk) and 40% with standard risk. Chemotherapy was used in all patients (100%), with upfront tumor resection performed in 40%. Fifty percent of patients received radiotherapy at a mean dose of 43 Gy, with the orbit the most frequently irradiated area (30%). All patients underwent CTscan and/or MRI and/or ultrasound surveillance. Follow-up during and after treatment was marked by complete remission in 8patients, loss of sight in one patient, and one patient died as a result of progressive disease. Conclusion: RMS is a malignant tumor of striated muscle. The epidemiological and clinical features of this tumor in our study are generally similar to those described in the literature. Management of these tumors requires multidisciplinary collaboration involving oncopediatric, radiologist, pediatric surgeon, pathologist and radiotherapist.
文摘BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchymal cells.It was first re-ported in postmenopausal women in 1970,and fewer than 50 postmenopausal patients have been reported to date.CASE SUMMARY A 68-year-old multiparous female was admitted to the hospital on October 11,2023,with the chief complaint of a mass causing vaginal prolapse with incomplete urination that had persisted for 4 months.The vaginal mass was approximately the size of a pigeon egg;after lying down,the vaginal mass retracted.Complete resection was performed,and vaginal pleomorphic RMS was diagnosed based on pathology and immunohistochemical staining features.The patient is currently undergoing chemotherapy.The present study also reviewed the clinical,histolo-gical,and immunohistochemical features and latest treatment recommendations for vaginal RMS.Any abnormal vaginal mass should be promptly investigated through pelvic examination and appropriate imaging.The current initial treat-ment for vaginal RMS is biopsy and primary chemotherapy.CONCLUSION When surgery is planned for vaginal RMS,an organ-preserving approach should be considered.
文摘BACKGROUND Rhabdomyosarcoma is a tumor of mesenchymal origin.Secondary leukemia is a complication of previous transformation to other hematologic disorders or is a treatment-related acute myeloid leukemia secondary to cytotoxic chemotherapy or radiation therapy for other malignancies.CASE SUMMARY We present the case of a 36-year-old female patient who was diagnosed with rhabdomyosarcoma and acute myeloid leukemia.Further disease progression was observed after multiline chemotherapy.Eventually,the patient suffered cerebral hemorrhage,which resulted in death.CONCLUSION The incidence of rhabdomyosarcoma in adults is extremely low,and secondary leukemia caused by rhabdomyosarcoma is even rarer.Secondary leukemia has a very poor prognosis and a low overall survival rate.
文摘Malignant mesenchymal tumors (MTM) in children represent 5% to 10% of malignant tumors in children. They constitute a heterogeneous group of tumors of various differentiations depending on their supposed tissue of origin. They mainly include tumors of muscular origin, those derived from connective, vascular, nervous, or adipose tissue. Rhabdomyosarcoma (RMS) is the most common mesenchymal tumor in children and adolescents (60% to 70% of them). And it accounts for 5.8% of all malignant solid tumors in children. Almost half of rhabdomyosarcomas occur in the head and neck. The prognosis for this type of tumor is particularly poor. A case of rhabdomyosarcoma in the mandible with extension to the abdominal wall and unilateral testis in a 6-month-old infant is reported with evolution since birth. It is a purplish lesion at the level under the right chin which was initially taken for vascular malformation, evolving very quickly towards a mandibular mass deforming the painful face with inflammatory signs, followed by the appearance of a hard swelling under the skin on the left flank taking on the same aspect of the mandibular mass. This observation illustrates the need to know how to systematically think about tumor causes in the face of atypical aspects and to carry out an anatomopathological examination.
文摘目的:观察人结直肠癌组织磷酸化哺乳动物雷帕霉素靶蛋白(phosphorylated mammalian target of rapamycin,p-mTOR)、表皮生长因子受体(EGFR)、生长因子受体结合蛋白2(Grb2)和血管内皮生长因子(VEGF)等的表达,并探讨其可能的临床意义。方法:构建含有185例结直肠癌标本的组织芯片,免疫组化法检测结直肠癌组织及癌旁组织中EGFR、Grb2、p-mTOR和VEGF的表达,并分析不同结直肠癌临床病理学特征下(如年龄、性别、浸润深度、淋巴转移、临床分期和分化程度)各自的表达情况,探讨可能的临床意义。结果:EGFR、Grb2、p-mTOR和VEGF在结直肠癌旁组织中呈少量表达或不表达,在结直肠癌组织中的表达率分别为21.1%、44.9%、42.2%和54.1%,明显高于癌旁组织(P<0.05)。结直肠癌患者不同性别、年龄、肿瘤分化程度下EGFR、Grb2、p-mTOR和VEGF表达率无统计学差异;不同浸润深度和临床分期下EGFR的表达率有统计学差异(P<0.05);不同浸润深度、淋巴转移和临床分期下p-mTOR、VEGF表达率均有统计学差异(P<0.05)。结直肠癌组织EGFR/Grb2/p-mTOR/VEGF蛋白两两间均有一定的相关性(r=0.245～0.567,P<0.05)。结论:EGFR、Grb2、p-mTOR和VEGF表达与结直肠癌的发生、发展相关,值得进一步研究以作为结直肠癌肿瘤靶向治疗新的作用靶点。
