Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Me...Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.展开更多
Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed durin...Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.展开更多
We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated ...We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated with neoadjuvant chemotherapy followed by wide excision of the patella and reconstruction of the extensor mechanism using split tendoachilles auto graft. The patella is an uncommon site for primary or metastatic tumors of the bone. ES, though rare, should be included in the differential diagnosis of swellings arising from the patella. Auto graft from the tendoachilles is a good alternative for reconstructing the extensor mechanism of the knee.展开更多
尤文肉瘤是一种罕见的小圆细胞恶性肿瘤,软组织与骨肿瘤世界卫生组织(World Health Organizition,WHO)分类第五版(2020年)将其归类于未分化小圆细胞肉瘤。尤文肉瘤由形态单一的小圆形细胞构成,免疫组织化学检测提示细胞膜CD99阳性,伴有...尤文肉瘤是一种罕见的小圆细胞恶性肿瘤,软组织与骨肿瘤世界卫生组织(World Health Organizition,WHO)分类第五版(2020年)将其归类于未分化小圆细胞肉瘤。尤文肉瘤由形态单一的小圆形细胞构成,免疫组织化学检测提示细胞膜CD99阳性,伴有不同程度的神经外胚层分化,并具有特征性的FET家族-ETS家族融合基因。手术联合化疗的治疗方式已将尤文肉瘤患者的5年生存率提高到约70%,但如患者诊断时已出现肿瘤转移,其5年生存率仍不到30%。随着分子病理学的飞速发展,尤文肉瘤的分子机制(特征性FET-ETS家族融合突变及常见伴随突变)也成为了研究热点。在诊断方面,研究者对尤文肉瘤特征性基因重排的不断深入了解,如罕见的TAF15-ETV4融合基因尤文肉瘤病例的发现,提示全基因组测序用于诊断尤文肉瘤的重要性已不容忽视。在治疗方面,许多针对特征性FET家族-ETS家族融合基因的药物正在临床试验中。2022年,尤文肉瘤的第一个重复出现的可作为分子治疗靶点的伴随突变——FGFR1突变被发现,提示伴随突变可能是尤文肉瘤分子治疗的突破点之一。2023年最新研究指出ETV6可能成为未来尤文肉瘤治疗的新靶点之一。本文总结尤文肉瘤的分子病理研究进展,并探讨其在尤文肉瘤的临床诊断、治疗和预后中的价值。展开更多
Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the an...Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the anatomopathological examination. The treatment is based on the combination of chemotherapy surrounding complete surgical removal of the tumor. Radiotherapy should remain reserved for a few specific indications because of its complications. We report the case of three children who presented with a pleural effusion revealing Ewing’s sarcoma affecting the ribs.展开更多
Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy al...Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome,more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting.Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.展开更多
尤文肉瘤(Ewing’s sarcoma)是一种常见于儿童和青少年的恶性肿瘤。1921年,由Ewing最先报道。尤文肉瘤患者的平均年龄为15岁,发病率约为2.93/百万人,占原发骨肿瘤的16%,与骨肉瘤、软骨肉瘤一起被称为三种最常见的骨肿瘤[1]。尤文肉瘤的...尤文肉瘤(Ewing’s sarcoma)是一种常见于儿童和青少年的恶性肿瘤。1921年,由Ewing最先报道。尤文肉瘤患者的平均年龄为15岁,发病率约为2.93/百万人,占原发骨肿瘤的16%,与骨肉瘤、软骨肉瘤一起被称为三种最常见的骨肿瘤[1]。尤文肉瘤的起源尚不清楚,根据目前的研究,尤文肉瘤被归类为"尤文肉瘤家族"(Ewing’s sarcoma family of tumors,ESFT)[2]。展开更多
Ewing’s sarcoma, a group of primary neuroectodermal tumor (PNET), is an uncommon rare highly malignant tumor mostly affecting the bones of male patients, and accounts for approximately 10% of the primary malignant bo...Ewing’s sarcoma, a group of primary neuroectodermal tumor (PNET), is an uncommon rare highly malignant tumor mostly affecting the bones of male patients, and accounts for approximately 10% of the primary malignant bone tumors, and occupies the second place after osteosarcoma in children’s less than 20 years. Primary intramedullary Ewing’s sarcoma is an extremely rare condition. Here, we present a case of a 16-year-old girl with progressive weakness of lower extremities, inability to walk, progressive low back pain, bilateral leg pain, paresthesia and urinary retention. Spinal MRI showed intramedullary abnormal signal focus from T11 to L3 vertebrae with moderate heterogeneous enhancement on T1 weighted sequences. After surgery, histological examination found a lot of small round-cell tumors with a high nuclear-cytoplasmic ratio, frequent mitoses and apoptotic nuclei suggesting a diagnosis of Ewing’s sarcoma and confirmed by CD99 reactivity. She had undergone adjuvant chemotherapy and irradiation and was free of symptoms since 02 years. Within this single pure case of intramedullary Ewing’s sarcoma, perhaps the 1st in the English literature, the authors described this extremely rare uncommon localization with a brief review of the literature.展开更多
文摘Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.
文摘Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.
文摘We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated with neoadjuvant chemotherapy followed by wide excision of the patella and reconstruction of the extensor mechanism using split tendoachilles auto graft. The patella is an uncommon site for primary or metastatic tumors of the bone. ES, though rare, should be included in the differential diagnosis of swellings arising from the patella. Auto graft from the tendoachilles is a good alternative for reconstructing the extensor mechanism of the knee.
文摘尤文肉瘤是一种罕见的小圆细胞恶性肿瘤,软组织与骨肿瘤世界卫生组织(World Health Organizition,WHO)分类第五版(2020年)将其归类于未分化小圆细胞肉瘤。尤文肉瘤由形态单一的小圆形细胞构成,免疫组织化学检测提示细胞膜CD99阳性,伴有不同程度的神经外胚层分化,并具有特征性的FET家族-ETS家族融合基因。手术联合化疗的治疗方式已将尤文肉瘤患者的5年生存率提高到约70%,但如患者诊断时已出现肿瘤转移,其5年生存率仍不到30%。随着分子病理学的飞速发展,尤文肉瘤的分子机制(特征性FET-ETS家族融合突变及常见伴随突变)也成为了研究热点。在诊断方面,研究者对尤文肉瘤特征性基因重排的不断深入了解,如罕见的TAF15-ETV4融合基因尤文肉瘤病例的发现,提示全基因组测序用于诊断尤文肉瘤的重要性已不容忽视。在治疗方面,许多针对特征性FET家族-ETS家族融合基因的药物正在临床试验中。2022年,尤文肉瘤的第一个重复出现的可作为分子治疗靶点的伴随突变——FGFR1突变被发现,提示伴随突变可能是尤文肉瘤分子治疗的突破点之一。2023年最新研究指出ETV6可能成为未来尤文肉瘤治疗的新靶点之一。本文总结尤文肉瘤的分子病理研究进展,并探讨其在尤文肉瘤的临床诊断、治疗和预后中的价值。
文摘Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the anatomopathological examination. The treatment is based on the combination of chemotherapy surrounding complete surgical removal of the tumor. Radiotherapy should remain reserved for a few specific indications because of its complications. We report the case of three children who presented with a pleural effusion revealing Ewing’s sarcoma affecting the ribs.
文摘Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome,more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting.Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.
文摘尤文肉瘤(Ewing’s sarcoma)是一种常见于儿童和青少年的恶性肿瘤。1921年,由Ewing最先报道。尤文肉瘤患者的平均年龄为15岁,发病率约为2.93/百万人,占原发骨肿瘤的16%,与骨肉瘤、软骨肉瘤一起被称为三种最常见的骨肿瘤[1]。尤文肉瘤的起源尚不清楚,根据目前的研究,尤文肉瘤被归类为"尤文肉瘤家族"(Ewing’s sarcoma family of tumors,ESFT)[2]。
文摘Ewing’s sarcoma, a group of primary neuroectodermal tumor (PNET), is an uncommon rare highly malignant tumor mostly affecting the bones of male patients, and accounts for approximately 10% of the primary malignant bone tumors, and occupies the second place after osteosarcoma in children’s less than 20 years. Primary intramedullary Ewing’s sarcoma is an extremely rare condition. Here, we present a case of a 16-year-old girl with progressive weakness of lower extremities, inability to walk, progressive low back pain, bilateral leg pain, paresthesia and urinary retention. Spinal MRI showed intramedullary abnormal signal focus from T11 to L3 vertebrae with moderate heterogeneous enhancement on T1 weighted sequences. After surgery, histological examination found a lot of small round-cell tumors with a high nuclear-cytoplasmic ratio, frequent mitoses and apoptotic nuclei suggesting a diagnosis of Ewing’s sarcoma and confirmed by CD99 reactivity. She had undergone adjuvant chemotherapy and irradiation and was free of symptoms since 02 years. Within this single pure case of intramedullary Ewing’s sarcoma, perhaps the 1st in the English literature, the authors described this extremely rare uncommon localization with a brief review of the literature.
