Turner syndrome (TS) is a female chromosomal disorder caused by the lack of an X chromosome. The loss of this chromosome may result in the deficiency of tumorsuppressive or DNA repair genes, leading to tumorigenesis. ...Turner syndrome (TS) is a female chromosomal disorder caused by the lack of an X chromosome. The loss of this chromosome may result in the deficiency of tumorsuppressive or DNA repair genes, leading to tumorigenesis. Recombinant human growth hormone (GH) has been popularly used for treatment in TS patients for growth promotion. Although treatment with GH has been correlated with precancerous and cancerous lesions in TS children, its associations with gastric or colonic tumors, especially ileal tubular adenomas, have not been reported frequently. We here report a case of a 16-year-old patient with TS and tubular adenoma of the small intestine. Whether the ileal adenoma was caused by TS itself or GH therapy was discussed.展开更多
Background This multi-center analysis of data from China reviews the management and long-term outcomes of patients with bilateral Wilms tumors(BWT),and explores prognostic risk factors.Methods We retrospectively analy...Background This multi-center analysis of data from China reviews the management and long-term outcomes of patients with bilateral Wilms tumors(BWT),and explores prognostic risk factors.Methods We retrospectively analyzed a cohort of pediatric patients with synchronous BWT treated at 18 pediatric oncology centers in China between 2006 and 2023.The overall survival(OS)and event-free survival(EFS)rates were calculated using Kaplan-Meier methodology.Prognostic risk factors were determined using univariable and multivariable analysis.Results A total of 167 patients with BWT and a median age at diagnosis of 13 months(range 0-78 months)were included in the study.Neoadjuvant chemotherapy was administered to 149 index cases;tumor biopsy was performed before initiating chemotherapy in 70 patients.One hundred and three children underwent bilateral nephron-sparing surgery(NSS)and two hundred fifty-two of the three hundred one kidneys underwent NSS.The four-year OS and EFS rates in the study cohort were 86.5%and 77.8%.After a median follow-up of 50 months,four patients developed renal failure requiring dialysis and a single patient received a kidney transplant 26 months postoperatively.Conclusions Regarding prognostic factors,the results of the multifactor analysis indicate that distant metastasis and positive surgical margins have negative impacts on OS and distant metastasis had a negative effect on EFS.Distant metastasis and positive surgical margins affect the long-term prognosis of BWT.展开更多
Background Low-risk neuroblastomas have favorable biologic characteristics.Children Oncology Group (COG) proposed that surgical resection of the primary tumor was sufficient.We evaluated the long-term prognosis of sur...Background Low-risk neuroblastomas have favorable biologic characteristics.Children Oncology Group (COG) proposed that surgical resection of the primary tumor was sufficient.We evaluated the long-term prognosis of surgery alone for patients with low-risk neuroblastoma in China.Methods A total of 34 patients with low-risk neuroblastoma were treated in our center between Jan 2009 and Dec 2013.The medical records of these patients were reviewed.Results The primary lesion was located in the adrenal gland in 19 patients,the retroperitoneum in 5,the posterior mediastinum in 9 and the neck in 1.The tumor diameters and volumes were 1.80-10.0 cm (average 5.5 ±2.3 cm) and 1.28-424.10 cm3 (average 58.81 ± 92.00 cm3),respectively.The stages of the patients were as follows:stage Ⅰ in 25,stage Ⅱ in 7,and stage Ⅳs in 2.All patients were in the low-risk group according to COG risk stratification criteria.No patients showed MYCN amplification.The primary tumors of all patients were completely resected.Nine adrenal tumors were completely resected by laparoscopy.All patients were successfully followed for 66-115 (average 89.71 ± 16.17) months.Recurrence was observed in 4 patients.In addition to one local recurrence,another three recurrences were metastases.The lesions were effectively controlled in all patients with recurrences.All patients survived,including 28 cases of tumor-free survival;the 4-year overall and event-free survival rates were both 100%.Conclusions Surgery alone is a safe and effective treatment strategy for low-risk neuroblastoma.Recurrent lesions may be controlled and treated by rescue chemotherapy and surgery.展开更多
基金Supported by The 2011 Ministry of Health Key Clinical Speciality Center Project
文摘Turner syndrome (TS) is a female chromosomal disorder caused by the lack of an X chromosome. The loss of this chromosome may result in the deficiency of tumorsuppressive or DNA repair genes, leading to tumorigenesis. Recombinant human growth hormone (GH) has been popularly used for treatment in TS patients for growth promotion. Although treatment with GH has been correlated with precancerous and cancerous lesions in TS children, its associations with gastric or colonic tumors, especially ileal tubular adenomas, have not been reported frequently. We here report a case of a 16-year-old patient with TS and tubular adenoma of the small intestine. Whether the ileal adenoma was caused by TS itself or GH therapy was discussed.
基金supported by the high-level clinical specialty construction project of public hospital in capital region of Inner Mongolia Autonomous Region(2023SGGZ0038).
文摘Background This multi-center analysis of data from China reviews the management and long-term outcomes of patients with bilateral Wilms tumors(BWT),and explores prognostic risk factors.Methods We retrospectively analyzed a cohort of pediatric patients with synchronous BWT treated at 18 pediatric oncology centers in China between 2006 and 2023.The overall survival(OS)and event-free survival(EFS)rates were calculated using Kaplan-Meier methodology.Prognostic risk factors were determined using univariable and multivariable analysis.Results A total of 167 patients with BWT and a median age at diagnosis of 13 months(range 0-78 months)were included in the study.Neoadjuvant chemotherapy was administered to 149 index cases;tumor biopsy was performed before initiating chemotherapy in 70 patients.One hundred and three children underwent bilateral nephron-sparing surgery(NSS)and two hundred fifty-two of the three hundred one kidneys underwent NSS.The four-year OS and EFS rates in the study cohort were 86.5%and 77.8%.After a median follow-up of 50 months,four patients developed renal failure requiring dialysis and a single patient received a kidney transplant 26 months postoperatively.Conclusions Regarding prognostic factors,the results of the multifactor analysis indicate that distant metastasis and positive surgical margins have negative impacts on OS and distant metastasis had a negative effect on EFS.Distant metastasis and positive surgical margins affect the long-term prognosis of BWT.
文摘Background Low-risk neuroblastomas have favorable biologic characteristics.Children Oncology Group (COG) proposed that surgical resection of the primary tumor was sufficient.We evaluated the long-term prognosis of surgery alone for patients with low-risk neuroblastoma in China.Methods A total of 34 patients with low-risk neuroblastoma were treated in our center between Jan 2009 and Dec 2013.The medical records of these patients were reviewed.Results The primary lesion was located in the adrenal gland in 19 patients,the retroperitoneum in 5,the posterior mediastinum in 9 and the neck in 1.The tumor diameters and volumes were 1.80-10.0 cm (average 5.5 ±2.3 cm) and 1.28-424.10 cm3 (average 58.81 ± 92.00 cm3),respectively.The stages of the patients were as follows:stage Ⅰ in 25,stage Ⅱ in 7,and stage Ⅳs in 2.All patients were in the low-risk group according to COG risk stratification criteria.No patients showed MYCN amplification.The primary tumors of all patients were completely resected.Nine adrenal tumors were completely resected by laparoscopy.All patients were successfully followed for 66-115 (average 89.71 ± 16.17) months.Recurrence was observed in 4 patients.In addition to one local recurrence,another three recurrences were metastases.The lesions were effectively controlled in all patients with recurrences.All patients survived,including 28 cases of tumor-free survival;the 4-year overall and event-free survival rates were both 100%.Conclusions Surgery alone is a safe and effective treatment strategy for low-risk neuroblastoma.Recurrent lesions may be controlled and treated by rescue chemotherapy and surgery.
