Desmoplastic small round cell tumor （DSRCT） is a rare, highly aggressive malignancy with distinctive histological features： a nesting pattern of cellular growth within dense desmoplastic stroma, occurring in young ...Desmoplastic small round cell tumor （DSRCT） is a rare, highly aggressive malignancy with distinctive histological features： a nesting pattern of cellular growth within dense desmoplastic stroma, occurring in young population with male predominance. The mean survival period is only about 1.5-2.5 years. The tumor has co-expressed epithelial, muscle, and neural markers in immunohistochemical studies. This work reports a 27-year-old man presenting with hematemesis and chronic constipation. Serial studies including endoscopy, upper gastrointestinal series, abdominal computed tomography and barium enema study showed disseminated involvement of visceral organs. The patient underwent aggressive surgery and received postoperative adjuvant chemotherapy consisting of 5-fluorouracil, cyclophosphamide, etoposide, doxorubicin, and cisplatin. He survived without any disease for 20 mo after the surgery. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome in this advanced DSRCT young patient.展开更多
The prognosis of patients with hepatocellular cardnorna （HCC） accompanied by portal vein tumor thrombus （PVTT） is generally poor if leo untreated： a median survival time of 2.7-4.0 mo has been reported. Furthermo...The prognosis of patients with hepatocellular cardnorna （HCC） accompanied by portal vein tumor thrombus （PVTT） is generally poor if leo untreated： a median survival time of 2.7-4.0 mo has been reported. Furthermore, while transcatheter arterial chemoembolization （TACE） has been shown to be safe in selected patients, the median survival time with this treatment is still only 3.8-9.5 mo. Systemic single-agent chemotherapy for HCC with PVTT has failed to improve the prognosis, and the response rates have been less than 20%. While regional chemotherapy with low-dose cisplatin and 5-fluorouracil or interferon and 5-fluorouracil via hepatic arterial infusion has increased the response rate, the median survival time has not exceeded 12 （range 4.5-11.8） mo. Combined treatment consisting of radiation for PVTT and TACE for liver tumor has achieved a high response rate, but the median survival rates have still been only 3.8-10.7 mo. With hepatic resection as monotherapy, the 5-year survival rate and median survival time were reportedly 4%-28.5% and 6-14 mo. The most promising results were reported for combined treatments consisting of hepatectomy and TACE, chemotherapy, or internal radiation. The reported 5-year survival rates and median survival times were 42% and 31 mo for TACE followed by hepatectomy; 36.3% and 22.1 mo for hepatectomy followed by hepatic arterial infusion chemotherapy; and 56% for chemotherapy or internal radiation followed by hepatectomy.展开更多
AIM: To evaluate the efficacy of different treatment strategies for hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) and investigate factors influencing prognosis.METHODS: One hundred and sevent...AIM: To evaluate the efficacy of different treatment strategies for hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) and investigate factors influencing prognosis.METHODS: One hundred and seventy-nine HCC patients with macroscopic PVTT were enrolled in this study. They were divided into four groups and underwent different treatments: conservative treatment group (n = 18),chemotherapy group (n = 53), surgical resection group (n = 24) and surgical resection with postoperative chemotherapy group (n = 84). Survival rates of the patients were analyzed by the Kaplan-Meier method. A log-rank analysis was performed to identify group differences. Cox's proportional hazards model was used to analyze variables associated with survival.RESULTS: The mean survival periods of the patients in four groups were 3.6, 7.3, 10.1, and 15.1 mo respectively.There were significant differences in the survival rates among the groups. The survival rates at 0.5-, 1-, 2-, and 3-year in surgical resection with postoperative chemotherapy group were 55.8%, 39.3%, 30.4%, and 15.6% respectively, which were significantly higher than those of other groups (P＜0.001). Multivariate analysis revealed that the strategy of treatment (P＜0.001) and the number of chemotherapy cycles (P = 0.012) were independent survival predictors for patients with HCC and PVTT.CONCLUSION: Surgical resection of HCC and PVTT combined with postoperative chemotherapy or chemoembolization is the most effective therapeutic strategy for the patients who can tolerate operation.Multiple chemotherapeutic courses should be given postoperatively to the patients with good hepatic function reserve.展开更多
文摘Desmoplastic small round cell tumor （DSRCT） is a rare, highly aggressive malignancy with distinctive histological features： a nesting pattern of cellular growth within dense desmoplastic stroma, occurring in young population with male predominance. The mean survival period is only about 1.5-2.5 years. The tumor has co-expressed epithelial, muscle, and neural markers in immunohistochemical studies. This work reports a 27-year-old man presenting with hematemesis and chronic constipation. Serial studies including endoscopy, upper gastrointestinal series, abdominal computed tomography and barium enema study showed disseminated involvement of visceral organs. The patient underwent aggressive surgery and received postoperative adjuvant chemotherapy consisting of 5-fluorouracil, cyclophosphamide, etoposide, doxorubicin, and cisplatin. He survived without any disease for 20 mo after the surgery. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome in this advanced DSRCT young patient.
文摘The prognosis of patients with hepatocellular cardnorna （HCC） accompanied by portal vein tumor thrombus （PVTT） is generally poor if leo untreated： a median survival time of 2.7-4.0 mo has been reported. Furthermore, while transcatheter arterial chemoembolization （TACE） has been shown to be safe in selected patients, the median survival time with this treatment is still only 3.8-9.5 mo. Systemic single-agent chemotherapy for HCC with PVTT has failed to improve the prognosis, and the response rates have been less than 20%. While regional chemotherapy with low-dose cisplatin and 5-fluorouracil or interferon and 5-fluorouracil via hepatic arterial infusion has increased the response rate, the median survival time has not exceeded 12 （range 4.5-11.8） mo. Combined treatment consisting of radiation for PVTT and TACE for liver tumor has achieved a high response rate, but the median survival rates have still been only 3.8-10.7 mo. With hepatic resection as monotherapy, the 5-year survival rate and median survival time were reportedly 4%-28.5% and 6-14 mo. The most promising results were reported for combined treatments consisting of hepatectomy and TACE, chemotherapy, or internal radiation. The reported 5-year survival rates and median survival times were 42% and 31 mo for TACE followed by hepatectomy; 36.3% and 22.1 mo for hepatectomy followed by hepatic arterial infusion chemotherapy; and 56% for chemotherapy or internal radiation followed by hepatectomy.
基金Supported by the Foundation of Hundred Outstanding Scholars Project of Shanghai, No. 97BR029 the Science and Technology Development Foundation of Shanghai, No. 984419067
文摘AIM: To evaluate the efficacy of different treatment strategies for hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) and investigate factors influencing prognosis.METHODS: One hundred and seventy-nine HCC patients with macroscopic PVTT were enrolled in this study. They were divided into four groups and underwent different treatments: conservative treatment group (n = 18),chemotherapy group (n = 53), surgical resection group (n = 24) and surgical resection with postoperative chemotherapy group (n = 84). Survival rates of the patients were analyzed by the Kaplan-Meier method. A log-rank analysis was performed to identify group differences. Cox's proportional hazards model was used to analyze variables associated with survival.RESULTS: The mean survival periods of the patients in four groups were 3.6, 7.3, 10.1, and 15.1 mo respectively.There were significant differences in the survival rates among the groups. The survival rates at 0.5-, 1-, 2-, and 3-year in surgical resection with postoperative chemotherapy group were 55.8%, 39.3%, 30.4%, and 15.6% respectively, which were significantly higher than those of other groups (P＜0.001). Multivariate analysis revealed that the strategy of treatment (P＜0.001) and the number of chemotherapy cycles (P = 0.012) were independent survival predictors for patients with HCC and PVTT.CONCLUSION: Surgical resection of HCC and PVTT combined with postoperative chemotherapy or chemoembolization is the most effective therapeutic strategy for the patients who can tolerate operation.Multiple chemotherapeutic courses should be given postoperatively to the patients with good hepatic function reserve.
