Objective To sum up the clinical experience in diagnosis and treatment of spinal primitive neuroectodermal tumor(PNET). Methods Thirteen patients with spinal PNET were included in the study from 1999 to 2009.There wer...Objective To sum up the clinical experience in diagnosis and treatment of spinal primitive neuroectodermal tumor(PNET). Methods Thirteen patients with spinal PNET were included in the study from 1999 to 2009.There were 8 males and 5展开更多
BACKGROUND Primitive neuroectodermal tumors are rare,highly malignant small round cell tumors belonging to the Ewing sarcoma family.The purpose of this article is to present clinical manifestation,histology,treatment,...BACKGROUND Primitive neuroectodermal tumors are rare,highly malignant small round cell tumors belonging to the Ewing sarcoma family.The purpose of this article is to present clinical manifestation,histology,treatment,and prognosis of two primitive neuroectodermal tumors(PNETs)in extremely rare anatomic locations,the abdominal wall and vulva.CASE SUMMARY Case 1 was a 66-month-old girl with lesions on the abdominal wall;tumor size was about 3.4 cm×6.1 cm×2 cm.The patient underwent radical resection of the tumor.After the operation,an alternating vincristine,doxorubicin,and cyclophosphamide/ifosfamide and etoposide(IE)regimen was given for eight cycles,and the patient survived for 66 mo without progression.Case 2 was a 40-month-old girl,with a vulvar lesion;tumor size was about 3.3 cm×5 cm×2.5 cm.The tumor was partially resected by surgery.The family left treatment after two cycles of vincristine,pirarubicin,and cyclophosphamide/IE chemotherapy,and the patient died at home six months after surgery.CONCLUSION PNET is a rare,fast-growing,highly malignant tumor that requires histologic and molecular analyses for exact diagnosis,and multimodal treatment is required to achieve a good prognosis.展开更多
Gastric neuroendocrine tumors(GNETs)are rare lesions characterized by hypergastrinemia that arise from enterochromaffin-like cells of the stomach.GNETs consist of a heterogeneous group of neoplasms comprising tumor ty...Gastric neuroendocrine tumors(GNETs)are rare lesions characterized by hypergastrinemia that arise from enterochromaffin-like cells of the stomach.GNETs consist of a heterogeneous group of neoplasms comprising tumor types of varying pathogenesis,histomorphologic characteristics,and biological behavior.A classification system has been proposed that distinguishes four types of GNETs;the clinicopathological features of the tumor,its prognosis,and the patient’s survival strictly depend on this classification.Thus,correct management of patients with GNETs can only be proposed when the tumor has been classified by an accurate pathological and clinical evaluation of the patient.Recently developed cancer therapies such as inhibition of angiogenesis or molecular targeting of growth factor receptors have been used to treat GNETs,but the only definitive therapy is the complete resection of the tumor.Here we review the literature on GNETs,and summarize the classification,clinicopathological features(especially prognosis),clinical presentations and current practice of management of GNETs.We also present the latest findings on new gene markers for GNETs,and discuss the effective drugs developed for the diagnosis,prognosis and treatment of GNETs.展开更多
The tumor desmoplastic small round cell in women is rare. Ovarian its location is exceptional. We report a patient of 20 years, the originalNorth Africawho consulted for an increase in abdominal volume with impaired g...The tumor desmoplastic small round cell in women is rare. Ovarian its location is exceptional. We report a patient of 20 years, the originalNorth Africawho consulted for an increase in abdominal volume with impaired general condition. Pelvic ultrasonography and computed tomography were in favor of multiple peritoneal masses with ascites. After surgical exploration, the histological and immunohistochemical data for diagnosis small round cell desmoplastic tumor of ovarian the patient was a candidate for poly chemotherapy but she was died a month later. Diagnosis relies on histological and immunohistochemical data. The demonstration of a nonrandom translocation t (11;22) (p13;q12) is specific for the disease. The management is multidisciplinary and combining surgery, a poly aggressive chemotherapy and radiotherapy. The prognosis remains poor.展开更多
AIM:To sum up the clinical and pathological characte- ristics of solid pseudopapillary tumor (SPT) and the experience with it.METHODS: A total of 553 SPT patients reported in Chinese literature between January 1996 an...AIM:To sum up the clinical and pathological characte- ristics of solid pseudopapillary tumor (SPT) and the experience with it.METHODS: A total of 553 SPT patients reported in Chinese literature between January 1996 and January 2009 were retrospectively reviewed and analyzed. RESULTS: The mean age of the 553 SPT patients included in this review was 27.2 years, and the male to female ratio was 1:8.37. Their symptoms were non-specific, and nearly one third of the patients were asymptomatic. Computed tomography and ultraso-nography were performed to show the nature and location of SPT. Most of the tumors were distributed in the pancreatic head (39.8%), tail (24.1%), body andtail (19.5%). Forty-five patients (9.2%) were diagnosed as malignant SPT with metastasis or invasion. None of the clinical factors was closely related to the malignant potential of SPT. Surgery was the main therapeutic modality for SPT. Local resection, distal pancreatectomy and pancreatoduodenectomy were the most common surgical procedures. Local recurrence and hepatic metastasis were found in 11 and 2 patients, respectively, after radical resection. Four patients died of tumor progression within 4 years after palliative resection of SPT. The prognosis of SPT patients was good with a 5-year survival rate of 96.9%.CONCLUSION: SPT of the pancreas is a rare indolent neoplasm that typically occurs in young females. It is a low-grade malignancy and can be cured with extended resection. The prognosis of such patients is good although the tumor may recur and metastasize.展开更多
BACKGROUND A prostatic stromal tumor is deemed to be a rare oncology condition.Based on the retrospective analysis of clinical data and scientific literature review,a case of prostatic stromal tumor was reported in th...BACKGROUND A prostatic stromal tumor is deemed to be a rare oncology condition.Based on the retrospective analysis of clinical data and scientific literature review,a case of prostatic stromal tumor was reported in this article to explore the diagnosis,treatment and prognosis of this rare disease.CASE SUMMARY The present case involved an older male patient who was admitted to our department for a medical consultation of dysuria.Serum prostate-specific antigen was 8.30 ng/mL,Ultrasound and magnetic resonance imaging suggested evident enlargement of the prostate and multiple cystic developments internally.Considering that the patient was an elderly male with a poor health status,transurethral resection of the prostate was performed to improve the symptoms of urinary tract obstruction.Furthermore,based on histopathologic examination and immunohistochemical staining,the patient was pathologically diagnosed with prostatic stromal tumor.The patient did not receive any further adjuvant therapy following surgery leading to a clinical recommendation that the patient should be followed up on a long-term basis.However,during the recent follow-up assessment,the patient demonstrated recurrence of lower urinary tract symptoms and gross hematuria.CONCLUSION Referring to scientific literature review,we believe that the management of these lesions requires a thorough assessment of the patient.Furthermore,the treatment of prostate stromal tumors should be based on the imaging examination andpathological classification. Active surgical treatment is of great significance to theprognosis of patients, and subsequent surveillance after the treatment iswarranted.展开更多
文摘Objective To sum up the clinical experience in diagnosis and treatment of spinal primitive neuroectodermal tumor(PNET). Methods Thirteen patients with spinal PNET were included in the study from 1999 to 2009.There were 8 males and 5
基金Supported by the Guangxi Natural Science Foundation Project,No.2014GXNSFAA118202
文摘BACKGROUND Primitive neuroectodermal tumors are rare,highly malignant small round cell tumors belonging to the Ewing sarcoma family.The purpose of this article is to present clinical manifestation,histology,treatment,and prognosis of two primitive neuroectodermal tumors(PNETs)in extremely rare anatomic locations,the abdominal wall and vulva.CASE SUMMARY Case 1 was a 66-month-old girl with lesions on the abdominal wall;tumor size was about 3.4 cm×6.1 cm×2 cm.The patient underwent radical resection of the tumor.After the operation,an alternating vincristine,doxorubicin,and cyclophosphamide/ifosfamide and etoposide(IE)regimen was given for eight cycles,and the patient survived for 66 mo without progression.Case 2 was a 40-month-old girl,with a vulvar lesion;tumor size was about 3.3 cm×5 cm×2.5 cm.The tumor was partially resected by surgery.The family left treatment after two cycles of vincristine,pirarubicin,and cyclophosphamide/IE chemotherapy,and the patient died at home six months after surgery.CONCLUSION PNET is a rare,fast-growing,highly malignant tumor that requires histologic and molecular analyses for exact diagnosis,and multimodal treatment is required to achieve a good prognosis.
基金Supported by National Natural Scientific Foundation of China,No.B1070296
文摘Gastric neuroendocrine tumors(GNETs)are rare lesions characterized by hypergastrinemia that arise from enterochromaffin-like cells of the stomach.GNETs consist of a heterogeneous group of neoplasms comprising tumor types of varying pathogenesis,histomorphologic characteristics,and biological behavior.A classification system has been proposed that distinguishes four types of GNETs;the clinicopathological features of the tumor,its prognosis,and the patient’s survival strictly depend on this classification.Thus,correct management of patients with GNETs can only be proposed when the tumor has been classified by an accurate pathological and clinical evaluation of the patient.Recently developed cancer therapies such as inhibition of angiogenesis or molecular targeting of growth factor receptors have been used to treat GNETs,but the only definitive therapy is the complete resection of the tumor.Here we review the literature on GNETs,and summarize the classification,clinicopathological features(especially prognosis),clinical presentations and current practice of management of GNETs.We also present the latest findings on new gene markers for GNETs,and discuss the effective drugs developed for the diagnosis,prognosis and treatment of GNETs.
文摘The tumor desmoplastic small round cell in women is rare. Ovarian its location is exceptional. We report a patient of 20 years, the originalNorth Africawho consulted for an increase in abdominal volume with impaired general condition. Pelvic ultrasonography and computed tomography were in favor of multiple peritoneal masses with ascites. After surgical exploration, the histological and immunohistochemical data for diagnosis small round cell desmoplastic tumor of ovarian the patient was a candidate for poly chemotherapy but she was died a month later. Diagnosis relies on histological and immunohistochemical data. The demonstration of a nonrandom translocation t (11;22) (p13;q12) is specific for the disease. The management is multidisciplinary and combining surgery, a poly aggressive chemotherapy and radiotherapy. The prognosis remains poor.
文摘AIM:To sum up the clinical and pathological characte- ristics of solid pseudopapillary tumor (SPT) and the experience with it.METHODS: A total of 553 SPT patients reported in Chinese literature between January 1996 and January 2009 were retrospectively reviewed and analyzed. RESULTS: The mean age of the 553 SPT patients included in this review was 27.2 years, and the male to female ratio was 1:8.37. Their symptoms were non-specific, and nearly one third of the patients were asymptomatic. Computed tomography and ultraso-nography were performed to show the nature and location of SPT. Most of the tumors were distributed in the pancreatic head (39.8%), tail (24.1%), body andtail (19.5%). Forty-five patients (9.2%) were diagnosed as malignant SPT with metastasis or invasion. None of the clinical factors was closely related to the malignant potential of SPT. Surgery was the main therapeutic modality for SPT. Local resection, distal pancreatectomy and pancreatoduodenectomy were the most common surgical procedures. Local recurrence and hepatic metastasis were found in 11 and 2 patients, respectively, after radical resection. Four patients died of tumor progression within 4 years after palliative resection of SPT. The prognosis of SPT patients was good with a 5-year survival rate of 96.9%.CONCLUSION: SPT of the pancreas is a rare indolent neoplasm that typically occurs in young females. It is a low-grade malignancy and can be cured with extended resection. The prognosis of such patients is good although the tumor may recur and metastasize.
文摘BACKGROUND A prostatic stromal tumor is deemed to be a rare oncology condition.Based on the retrospective analysis of clinical data and scientific literature review,a case of prostatic stromal tumor was reported in this article to explore the diagnosis,treatment and prognosis of this rare disease.CASE SUMMARY The present case involved an older male patient who was admitted to our department for a medical consultation of dysuria.Serum prostate-specific antigen was 8.30 ng/mL,Ultrasound and magnetic resonance imaging suggested evident enlargement of the prostate and multiple cystic developments internally.Considering that the patient was an elderly male with a poor health status,transurethral resection of the prostate was performed to improve the symptoms of urinary tract obstruction.Furthermore,based on histopathologic examination and immunohistochemical staining,the patient was pathologically diagnosed with prostatic stromal tumor.The patient did not receive any further adjuvant therapy following surgery leading to a clinical recommendation that the patient should be followed up on a long-term basis.However,during the recent follow-up assessment,the patient demonstrated recurrence of lower urinary tract symptoms and gross hematuria.CONCLUSION Referring to scientific literature review,we believe that the management of these lesions requires a thorough assessment of the patient.Furthermore,the treatment of prostate stromal tumors should be based on the imaging examination andpathological classification. Active surgical treatment is of great significance to theprognosis of patients, and subsequent surveillance after the treatment iswarranted.
