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In the last half-century, the multi-cooperation of conventional therapies including chemotherapy, radiotherapy and bone marrow hematopoietic stem cell transplantation has saved a lot of children with lymphocyte malignancies, but the outcomes of relapsed and/or refractory diseases remain dismal.Thus, it is in urgent need of novel approaches to improve clinical efficiency.After decades of rapid development, immunotherapy has achieved remarkable results from the treatment of pediatric lymphocyte malignancies, especially chimeric antigen receptors T(CAR-T), bi-specific T cell engager(BiTE), and immune checkpoint blockader(ICB). However, a part of patients is still resistant to immunotherapy.The review will focus on CAR-T, BiTE and ICB to elucidate the pre-clinical studies and clinical trials that have been intensively conducted in pediatric leukemia and lymphoma, as well as the brief introduction of their limitations and probably optimal strategies.
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Objective:To investigate the efficacy of microsurgical treatment in cavernous sinus tumors.Methods:The clinical data of 87 patients with cavernous sinus tumor treated by microsurgery from January, 2010 to August, 2019 were analysed retrospectively. The surgical approaches and microsurgical skills for common tumors in Cavernous Sinus region were discussed. The follow-up included outpatient and telephone follow-ups, and the follow-up results were evaluated by KPS score.Results:Among the 87 cases, 57 were totally resected (65.5%), 14 were subtotal resected (16.1%) and 16 were major resected (18.4%). Hospitalisation ranged from 14 to 98 days, with an average of 29 days. Postoperative complications occurred in 30 cases with cranial nerve injury, 2 brain stem injury, 4 postoperative bleeding, 5 cerebrospinal fluid leakage, 4 infection, 1 Pituitary damage and 1 death. Prognosis and follow-up analysis showed 68 cases with KPS>60 and 66 with KPS>80 at 1 month after surgery; 74 with KPS>70 and 72 with KPS>80 at 3 months after surgery; 78 with KPS>80 by 12 months after surgery. During the follow-up period of 6-120 months, 3 cases died. Recurrence: 6 of incomplete resection of meningioma, were in 1-6 years after the surgery, 4 of incomplete resection of schwannoma in 1-8 years, 2 of pituitary adenoma respectively in 13 and 16 months after the surgery. There was no recurrence after reoperation. Two cases of chondrosarcoma, 3 of chordoma and 3 of germinoma were treated with radiotherapy, and during the follow-up, there was no progress of the focus. No tumor progression or recurrence was found in other cases during follow-up.Conclusion:Surgery of cavernous sinus tumor is difficult due to frequent postoperative complications. Reasonable preoperative plan, surgical approach and precise microsurgical techniques are the keys in reduction of postoperative complications and in the improvement of prognosis.
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Objective:To evaluate the value of intraoperative magnetic resonance imaging (iMRI) combined with neuronavigation for the resection of insular gliomas.Methods:From August 2014 to October 2017 in the First Hospital Affiliated to Sun Yat-sen University,clinical data of 41 patients with insular glioma,who underwent the surgery assisted with 3.0T iMRI and neuronavigation,were analyzed retrospectively,and the resection extent,complications and prognosis were evaluated.Results:Subtotal tumor resection was achieved in 21 patients and partial resection was done in 20 after iMRI scanning.After further resection,total tumor resection was achieved in 16 patients,subtotal resection in 18 and partial resection in 7.There was a statistical significant difference in tumor resection between pre-iMRI and post-iMRI according to the Fisher test (P＜0.05).In the follow-up from 3 months to 3 years,the symptoms of the 41 patients had improved.Conclusion:iMRI corrected the shift of brain.Neuronavigation can accurately and timely assess the degree of resecting tumor.The combination of neuronavigation with surgery can maximally and safely resect insular glioma.
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Objective · To investigate the clinical characteristics and multidisciplinary treatment of children with clear cell sarcoma of kidney (CCSK). Methods · Data of seven children with CCSK treated at Xinhua Hospital, Shanghai Jiao Tong University School of Medicine between Jan 2011 and Jan 2016 were collected. The retrospective analysis of clinical manifestations, features of imaging and pathology, treatment, and follow-up was performed. Results · Of 7 children with CCSK, 6 were male and 1 was female with the median age of 28 months (4-59 months), and 3 were at stageⅠ, 2 at stage Ⅲ, and 2 at stage Ⅳ. All cases were discussed and evaluated by multidisciplinary teams, including pediatric hematology/oncology, pediatric surgery, pathology, radiology and radiotherapy. CCCG-WT-2009 protocol was adopted to treat these patients. The median follow-up period was 22 months (8-56 months). Six children survived and one died. Conclusion · The multidisciplinary treatment mode can effectively improve the prognosis of CCSK. CCCG-WT-2009 protocol has good therapeutic effect and high cure rate for children with early stage CCSK, but the treatment of advanced stage CCSK needs to be further explored and perfected.
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Objective · To investigate the changes of neutrophils and lymphocytes during chemotherapy courses in children with high-risk neuroblastoma, as well as severe infection rate and its relationship with combination chemotherapy regimens in order to provide guidance to prevent infection in these children. Methods · A total of 45 cases of children with high-risk neuroblastoma, who were hospitalized in Xinhua Hospital from July 2012 to September 2015 were recruited. Reactions after chemotherapy and the results of routine blood tests on every 5 days during the whole chemotherapy circles were recorded. Follow-up visits were carried out to learn the infection occurrence. Results · The counts of neutrophils and lymphocytes dropped to the minimum at the 10th day or so during chemotherapy, then rised near the 15th day. Infections were negatively correlated with the numbers of neutrophils (r =-0.245) and lymphocytes (r=-0.227) at the 10th day. Persistent lymphopenia occured after the forth course of chemotherapy. Rate of grade Ⅳ bone marrow suppression after chemotherapy was 90%, and severe infection rate was 15.5%. There were significant differences in the rates of grade Ⅲ bone marrow suppression (P=0.008), grade Ⅳ bone marrow suppression (P=0.001) and severe infection (P=0.010) among the 3 combination chemotherapy regimens used in high-risk neuroblastoma children. Conclusion · Children with high-risk neuroblastoma had obviously high rates of bone marrow suppression and severe infection after chemotherapy, especially after cyclophosphamide+doxorubicin+vincristine regimens. More attention should be paid to this group of children and preventive measures were worthy of taking into account.
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Objective To evaluate the clinical application of multimodality treatment of brain arteriovenous malformation (BAVM) with Onyx embolization and microsurgery.Methods There were 48 patients with BAVM treated by combination of Onyx embolization and microsurgery.The clinical features,therapeutic process,complications and discharge status (by MRI,DSA and Glasgow outcome scale,GOS) were recorded in detail.The prognosis was estimated by modified Rankin scale (mRS) with 6-42 months' follow-up.Results The total resection rate was 97.9％.The therapy-related complications happened in 8 patients (16.7％).Among them,4 cases had dysfunction of central nervous system (8.3％),2 cases with intra-cerebral hemorrhage (4.2％),and 2 suffered from severe cerebral edema (4.2％).Good recovery was seen in 38 cases (79.2％) evaluated by GOS when discharged from hospital.The follow-up result mRS ＜ 3 was 97.9％.Conclusion Multimodality treatment of BAVM with Onyx embolization and microsurgery should be a good therapeutic strategy.
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Objective To investigate the clinical features and therapeutic strategies of one patient with meningioangiomatosis ( MA ) complicated with intracranial aneurysm formation. Methods The clinical data of one patient with aneurysm-formed MA admitted to the Department of Neurosurgery,the First Affiliated Hospital of Sun Yat-sen University was analyzed retrospectively. The patient received emergency craniotomy and middle cerebral artery aneurysm clipping and evacuation of intracranial hematoma. The meningeal tissue around the aneurysm was taken for pathological examination. Results Microscopy revealed meningeal thickening in the patient with MA accompanied with intracranial aneurysm. Spindle cells around the meninges and perivascular tissue of cortical hyperplasia formed an annular and fence-like structure. The results of immunohistochemistry showed the hyperplastic vessels and perivascular fibroblast like spindle cells diffuse vimentin-positive;the hyperplastic vascular endothelial cells CD99,CD34 were positive;and the residual glial cells in the lesions showed glial fibrillary acidic protein positive. Conclusion MA complicated with intracranial aneurysm is rare. The vascular condition is poor. The shape of aneurysm is irregular,and the risk of bleeding is high. Excise the involved meningeal tissue and clip the aneurysm with microsurgical operation is the resonable treatment.
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Objective To investigate the clinical features and treatment strategy of brain arteriovenous malforma-tions(AVM) associated with arterial aneurysms. Methods The clinical manifestation, imaging data, treatment and out-come of the patients with AVM were retrospectively analyzed in 38(10.4%) of these 364 patients. Results Hemorrhage was the most common clinical manifestation (60.5%). The hemorrhage risk was higher in AVM associated with arterial an-eurysms than in single AVM(χ2=5.956,P<0.05). The most common type of associated aneurysms was feeding artery an-eurysm (69.4%). Hemorrhage more frequently occurred within the aneurysm.(χ2=8.869,P<0.05). The bleeding lesions and 43 associated aneurysms of the 37 patients were treated effectively by endovascular treatment and/or craniotomy. Thirty-four patients had excellent or good outcomes, six patients had complications, 3 patients had neurological deficits. Postoperative digital subtraction angiography (DSA) examination showed the complete disappearance of arteriovenous malformation and aneurysm in sixteen cases. Conclusions The AVM associated with aneurysms is common in patients with AVM. AVM associated with aneurysms has a higher risk of hemorrhage than single AVM. The aneurysm within AVM has a higher rate of hemorrhage. The treatment priority should be given to the bleeding lesions and aneurysms.
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Objective To investigate the effects of all trans-retinoic acid (ATRA) on the expressions of iron metabolism-related genes and their products in K562 cells and the possible relationship. Methods (1) The characteristics of K562 leukemic cell differentiation induced by ATRA was evaluated by Benzidine, Wright's, NSE and NBT staining.(2) The expression levels of cellular surface antigens (CD71 and CD 13) in K562 cells cultured with ATRA were measured by flow cytometry. (3) IRP/IRE binding activity was assessed by RNA/protein band-shift assay.(4) Ferritin was determined by radioimmunoassay.(5) The mRNA expression levels of H-Fn, TfR and IRP2 in K562 cells cultured with different concentrations of ATRA were delineated by RT-PCR method, confirmed by sequencing of RT-PCR products. Results K562 cells could be induced to differentiate into neutrophils by ATRA, confirmed by cytochemical staining. The expression of CD71 decreased while CD13 increased. The mRNA expression levels of TfR and IRP2 were decreased while mRNA expression level of H-Fn was increased in K562 cells cultured with ATRA, compared to that in control cells. Concomitantly,IRP binding activity was significantly decreased but the level of ferritin was significantly increased in K562 cells cultured with ATRA. Conclusions During the course of K562 cells induction and differentiation to myelocytes by ATRA, the expression level of iron metabolism-related genes and products were changed but the upstream-regulation mechanism still remains unclear.