目的探讨Ⅰ期肺腺癌沿气腔播散(spread through air space,STAS)与上皮-间质转化(epithelial-mesenchymal transition,EMT)的关系,及其与临床病理特征、预后的相关性。方法回顾性分析412例Ⅰ期肺腺癌的临床病理特征,分析STAS与各临床病...目的探讨Ⅰ期肺腺癌沿气腔播散(spread through air space,STAS)与上皮-间质转化(epithelial-mesenchymal transition,EMT)的关系,及其与临床病理特征、预后的相关性。方法回顾性分析412例Ⅰ期肺腺癌的临床病理特征,分析STAS与各临床病理特征及预后的相关性,采用免疫组化法检测EMT标志蛋白及关键转录因子的表达。结果412例Ⅰ期肺腺癌中,STAS阳性率为36.9%(152/412);微乳头型及实体型肺腺癌中STAS的阳性率明显高于其他组织学形态。STAS的出现与肿瘤坏死、胸膜侵犯、脉管侵犯明显相关。预后分析显示,STAS阳性者5年累积生存率、无复发生存率明显降低,5年复发/死亡累积风险明显升高,是Ⅰ期肺腺癌独立预后因素。结论STAS是Ⅰ期肺腺癌患者独立的预后因素,STAS的形成与EMT相关。展开更多
Background Borderline gastrointestinal stromal tumors （GISTs） are intermediate tumors between benign and malignant variants; however, the clinical and pathological features of borderline GISTs remain poorly defined....Background Borderline gastrointestinal stromal tumors （GISTs） are intermediate tumors between benign and malignant variants; however, the clinical and pathological features of borderline GISTs remain poorly defined. This study aimed to characterize GISTs and to identify a set of borderline criteria for practical use. Methods Medical records and specimens of 840 patients from 12 hospitals were retrospectively examined. Totally 485 and 76 patients with any of the parameters predictive of either malignant or benign tumors were excluded. The Kaplan-Meier method was used to calculate disease-free survival and overall survival rates. Results Among the remaining 279 borderline GIST patients, 223 were followed up for 1 to 31.48 years. Two patients developed local recurrence, and both were cured by subsequent operations alone. The 5-year disease-free survival and overall survival rates were 99% and 100%, respectively. Morphologically, borderline GISTs typically exhibited moderate cellularity, and subsets of them also showed moderate atypia, low mitotic activities, or large tumor size. According to the National Institutes of Health （NIH） consensus criteria, the risk levels of the 279 GISTs were classified to be very low to high. However, the disease-free survival rates were not significantly different among these risk groups （P=0.681）. Conclusions The proposed borderline GIST criteria in the current study may complement the existing NIH criteria, based primarily on tumor size and mitotic count, in the evaluation of the biological behaviors of GISTs. Since a subset of borderline GISTs with high risk level showed favorable outcome, the introduction of the borderline GIST system may avoid overdiagnosis and over therapy.展开更多
Background Lymphangioleiomyomatosis （LAM） is a rare disease that predominantly affects young females. It is considered as an ＂orphan＂ life-threatening disease of unknown etiology, with uncertain clinical prognosis...Background Lymphangioleiomyomatosis （LAM） is a rare disease that predominantly affects young females. It is considered as an ＂orphan＂ life-threatening disease of unknown etiology, with uncertain clinical prognosis, and no effective treatment. LAM can arise sporadically or in association with tuberous sclerosis complex （TSC）, an autosomal inherited syndrome characterized by hamartoma-like tumor growth and pathologic features that are distinct from manifestations of pulmonary LAM. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections. Methods Fourteen cases of LAM from Zhongshan Hospital, Fudan University are reviewed, twelve were confirmed by lung biopsy, one by retroperitoneal lymphangioleiomyoma resection, and one by autopsy. Results All 14 patients were women, aged 18 to 69 years （mean 43.3 years, median 46.5 years）. Haemoptysis （57.1%） and chylothorax （35.7%） were more frequent than those described in previous case series. Extrapulmonary findings such as renal angiomyolipoma （AML）, enlarged abdominal lymph nodes, liver AML and retroperitoneal lymphangioleiomyoma were seen in 21.4%, 14.3%, 7.14% and 7.14% in 14 cases respectively, which is remarkably lower than in the previously reported. Abnormal smooth muscle cells （LAM cells） were found to line the airways, bronchioles, lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts. There were some surprises in the autopsy case as several LAM cell emboli were found in the veins of mediastinum lymph nodes; LAM cells were found to be disseminated in soft tissues adjacent to the ilium. Conclusions Women with unexplained recurrent pneumothorax, tuberous sclerosis, or a diagnosis of primary spontaneous pneumothorax or emphysema in the setting of limited or absent tobacco use should undergo high-resolution computed tomography （HRCT） scan screening for LAM. Routine abdominal and pelvic imaging examinations should be performed to detect extrapulmonary involvement. The autopsy studies histologically suggested that LAM could be a multisystemic disease and LAM cells might possess metastatic potential.展开更多
Background According to the National Institutes of Health consensus criteria, gastrointestinal stromal tumors （GISTs） smaller than 2 cm in diameter with less than 5 mitotic figures per 50 high-power fields are consi...Background According to the National Institutes of Health consensus criteria, gastrointestinal stromal tumors （GISTs） smaller than 2 cm in diameter with less than 5 mitotic figures per 50 high-power fields are considered very-low-risk GISTs but these two indices alone cannot reliably predict a benign outcome during long-term follow-ups. Therefore, identification of additional parameters for predicting the clinical behavior of GISTs is necessary. Methods Eighty-eight patients with tumors that meet the very-low-risk GIST criteria were retrospectively investigated and morphological parameters of tumors associated with the biological behavior of very-low-risk GISTs were evaluated in the present study. The Kaplan-Meier method was used to calculate disease-free survival rates. Results Eighty-one patients were followed up for one to 16.3 years. Five cases of relapses were identified in the patients. Distinctive infiltrative growth patterns such as muscularis propria, muscularis mucosa, or nerve infiltration were identified by microscopy in 4 patients with the relapse, including three patients who experienced multiple recurrences. The infiltrative growth features became more obvious in multiple recurrent tumors compared to the single recurrent tumor, while only one developed relapse in 76 patients without infiltration （P 〈0.0001）. Conclusion Microscopic infiltrative growth patterns of the tumor may have clinical significance in predicting the prognosis of very-low-risk GISTs.展开更多
文摘目的探讨Ⅰ期肺腺癌沿气腔播散(spread through air space,STAS)与上皮-间质转化(epithelial-mesenchymal transition,EMT)的关系,及其与临床病理特征、预后的相关性。方法回顾性分析412例Ⅰ期肺腺癌的临床病理特征,分析STAS与各临床病理特征及预后的相关性,采用免疫组化法检测EMT标志蛋白及关键转录因子的表达。结果412例Ⅰ期肺腺癌中,STAS阳性率为36.9%(152/412);微乳头型及实体型肺腺癌中STAS的阳性率明显高于其他组织学形态。STAS的出现与肿瘤坏死、胸膜侵犯、脉管侵犯明显相关。预后分析显示,STAS阳性者5年累积生存率、无复发生存率明显降低,5年复发/死亡累积风险明显升高,是Ⅰ期肺腺癌独立预后因素。结论STAS是Ⅰ期肺腺癌患者独立的预后因素,STAS的形成与EMT相关。
文摘Background Borderline gastrointestinal stromal tumors （GISTs） are intermediate tumors between benign and malignant variants; however, the clinical and pathological features of borderline GISTs remain poorly defined. This study aimed to characterize GISTs and to identify a set of borderline criteria for practical use. Methods Medical records and specimens of 840 patients from 12 hospitals were retrospectively examined. Totally 485 and 76 patients with any of the parameters predictive of either malignant or benign tumors were excluded. The Kaplan-Meier method was used to calculate disease-free survival and overall survival rates. Results Among the remaining 279 borderline GIST patients, 223 were followed up for 1 to 31.48 years. Two patients developed local recurrence, and both were cured by subsequent operations alone. The 5-year disease-free survival and overall survival rates were 99% and 100%, respectively. Morphologically, borderline GISTs typically exhibited moderate cellularity, and subsets of them also showed moderate atypia, low mitotic activities, or large tumor size. According to the National Institutes of Health （NIH） consensus criteria, the risk levels of the 279 GISTs were classified to be very low to high. However, the disease-free survival rates were not significantly different among these risk groups （P=0.681）. Conclusions The proposed borderline GIST criteria in the current study may complement the existing NIH criteria, based primarily on tumor size and mitotic count, in the evaluation of the biological behaviors of GISTs. Since a subset of borderline GISTs with high risk level showed favorable outcome, the introduction of the borderline GIST system may avoid overdiagnosis and over therapy.
文摘Background Lymphangioleiomyomatosis （LAM） is a rare disease that predominantly affects young females. It is considered as an ＂orphan＂ life-threatening disease of unknown etiology, with uncertain clinical prognosis, and no effective treatment. LAM can arise sporadically or in association with tuberous sclerosis complex （TSC）, an autosomal inherited syndrome characterized by hamartoma-like tumor growth and pathologic features that are distinct from manifestations of pulmonary LAM. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections. Methods Fourteen cases of LAM from Zhongshan Hospital, Fudan University are reviewed, twelve were confirmed by lung biopsy, one by retroperitoneal lymphangioleiomyoma resection, and one by autopsy. Results All 14 patients were women, aged 18 to 69 years （mean 43.3 years, median 46.5 years）. Haemoptysis （57.1%） and chylothorax （35.7%） were more frequent than those described in previous case series. Extrapulmonary findings such as renal angiomyolipoma （AML）, enlarged abdominal lymph nodes, liver AML and retroperitoneal lymphangioleiomyoma were seen in 21.4%, 14.3%, 7.14% and 7.14% in 14 cases respectively, which is remarkably lower than in the previously reported. Abnormal smooth muscle cells （LAM cells） were found to line the airways, bronchioles, lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts. There were some surprises in the autopsy case as several LAM cell emboli were found in the veins of mediastinum lymph nodes; LAM cells were found to be disseminated in soft tissues adjacent to the ilium. Conclusions Women with unexplained recurrent pneumothorax, tuberous sclerosis, or a diagnosis of primary spontaneous pneumothorax or emphysema in the setting of limited or absent tobacco use should undergo high-resolution computed tomography （HRCT） scan screening for LAM. Routine abdominal and pelvic imaging examinations should be performed to detect extrapulmonary involvement. The autopsy studies histologically suggested that LAM could be a multisystemic disease and LAM cells might possess metastatic potential.
文摘Background According to the National Institutes of Health consensus criteria, gastrointestinal stromal tumors （GISTs） smaller than 2 cm in diameter with less than 5 mitotic figures per 50 high-power fields are considered very-low-risk GISTs but these two indices alone cannot reliably predict a benign outcome during long-term follow-ups. Therefore, identification of additional parameters for predicting the clinical behavior of GISTs is necessary. Methods Eighty-eight patients with tumors that meet the very-low-risk GIST criteria were retrospectively investigated and morphological parameters of tumors associated with the biological behavior of very-low-risk GISTs were evaluated in the present study. The Kaplan-Meier method was used to calculate disease-free survival rates. Results Eighty-one patients were followed up for one to 16.3 years. Five cases of relapses were identified in the patients. Distinctive infiltrative growth patterns such as muscularis propria, muscularis mucosa, or nerve infiltration were identified by microscopy in 4 patients with the relapse, including three patients who experienced multiple recurrences. The infiltrative growth features became more obvious in multiple recurrent tumors compared to the single recurrent tumor, while only one developed relapse in 76 patients without infiltration （P 〈0.0001）. Conclusion Microscopic infiltrative growth patterns of the tumor may have clinical significance in predicting the prognosis of very-low-risk GISTs.
