ABSTRACT
[Abstract ] Objective Nephroblastoma is one of the solid malignancies with fair prognosis in children .The lungs, liver, and bones are the common organs for distant metastasis of the tumor and important factors influencing the therapeutic effects as well .This study was to investigate the clinical characteristics and prognosis of nephroblastoma with distant metastasis in children . Methods We retrospectively analyzed the clinical characteristics , pathological features , and prognosis of 9 cases of nephroblastoma with distant metastasis treated in Nanjing Children Hospital from September 2011 to May 2015 . Results The patients presented with abdominal masses, distention and pain at preliminary diagnosis and confirmed with nephroblastoma , with pulmonary metastasis in 7 cases, liver metastasis in 1, and lung and liver metastases in the other .All were treated by surgery, radiotherapy, and chemotherapy and followed up for a median time of 34 months.By the end of the follow-up, 1 patient died of recurrence , 1 was undergoing chemotherapy , and the other 7 were being recovered, including complete disappearance of lung metastasis in 4, obvious reduction or calcification of lung metastasis in 2, evident disappearance of liver metastasis in 1, but relapse in none. Conclusion Lung and liver are common sites of nephroblastoma metastasis in children .As for the treatment of the disease , relatively good results can be achieved by radiotherapy , chemotherapy , and reoperation .
ABSTRACT
Objective Primitive neuroectodermal tumor ( PNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors, the purpose of the study was to investigate the clinical features, treatment and prognosis of Children PNET. Methods A retrospective study was performed on clinical data of 13 children with PNET hospitalized in our hospital from March 2010 to Octorber 2014.In order to analyze the clinical effects and prognostic results, statistical analysis was made on their clinical manifestations, CT and ultrasonic results, therapeutic schemes, postoperative pathology and immu-nohistochemical staining results, along with telephone and outpaitient follow-up of average 19 months. Results Of the 13 patients, 4 cases were central nervous system PNET(CNS-PNET) and 9 cases were peripheral PNET(pPNET).The clinical manifestations of the former were headache, vomiting, convolsion, movement disorder or decreased muscle strength, while the latter mainly showed mas-ses and abdominal distention.3 cases with metastasis to lung, lymph nodes and bones respectively showed masses on surface or in cavi-ty in imaging.Homer-Wright pseudorosette textures were found in 12 patients.Immunohistochemical results showed 11 cases with CD99(+), 9 cases with VIM(+) and 8 cases Syn( +).Among 11 patients underwent chemotherapy, 2 cases were lost to follow-up, 7 cases were in stable condition without occurrence and the other 2 cases recurred 4-6 months after chemotherapy.3 of 4 cases with CNS-PNET survived with the sequelae of decreased muscle strength, convolsion and movement disorder.1 case with CNS-PNET and 1 case with pPNET died 7 months and 16 months after diagnosis respectively. Conclusion PNET in children is a very highly malignant tumor with low long-term survival rate.Immunohistochemi-cal examination including CD99 and VIM is of important diagnostic value and CNS-PNET is prone to sequelae.