Residual or recurrent cerebellar low-grade glioma in children after tumor resection： is re-treatment needed？ A single center experience from 1983 to 2003

PURPOSE： The aim of this study was to report on children with cerebellar low-grade glioma （LGG）, who were found to have progressive or nonprogresssive residual tumors or tumor recurrence after tumor resection. PATIENTS AND METHODS： Medical records and magnetic resonance imaging （MRI） studies of children （〈16years） with cerebellar LGG were retrospectively analyzed. RESULTS： Of 289 patients with CNS tumors referred between 1983 and 2003, 28 （9.7%） （15 male, 13 female; median age at diagnosis： 71 months） had cerebellar LGG （pilocytic astrocytoma grade Ⅰ： n=21; fibrillary astrocytoma grade Ⅱ： n=5; mixed hamartoma/pilocytic astrocytoma： n=1; radiographic diagnosis： n=1）. Total resection was initially performed in 16 patients （57.1%）, near total resection in 4 （14.3%）, and partial resection in 6 patients （21.4%）. One patient underwent biopsy. At a median follow-up of 112 months, 25 patients （89.3%） were alive, 18of them being in complete remission. Three patients died, 2 due to symptoms related to brain stem compression/infdtration and 1 patient due to postoperative cerebral edema. Presently 5 patients have nonprogressive residual tumors and 2 patients developed nonprogressive recurrences 10 years and 20 months after initial total resection, respectively. None of them required second surgery and none received additional nonsurgical therapies. Only 1 additional patient had to undergo second surgery due to disease progression. CONCLUSIONS： A ＂wait and see＂ strategy is justified in patients with nonprogressive recurrent or residual cerebellar LGG after primary tumor resection. However,     

Endoscopic options in children： experience with 134 procedures

OBJECT： There are frequent applications for endoscopy in neurosurgery. However, endoscopic surgery in children has peculiar characteristics and is associated with different rates of success. In this study, the authors report on their experience with 134 consecutive endoscopy procedures performed in 126 patients〈18 years of age. METHODS： Between April 1993 and October 2007, 134 endoscopic procedures were performed in 126 children. Indications for surgery included brain tumors in 48 children, cystic lesions in 24, aqueductal stenosis in 23, various malformations in 20, hemorrhage and infarction in 6, and isolated ventricles in 5 children. In this long-term followup study, data were analyzed with respect to clinical and radiological success rates, as well as shunt dependence both in relation to lesion origin, and to the type of endoscopic procedure performed （endoscopic third ventrieulostomy [ETV], septostomy, aqueductoplasty, or cystocistemostomy）. Finally, the influence of patient age on the success rate was evaluated.     

Management and outcome of children with neuroendocrine tumors of the appendix in Spain: Is there room for improvement?

Purpose

Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs.

Patients and methods

Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015.

Results

Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up.

Conclusions

The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines.

     

Fusion genes in solid tumors： an emerging target for cancer diaanosis and treatment

Studies over the past decades have uncovered fusion genes, a class of oncogenes that provide immense diagnostic and therapeutic advantages because of their tumor-specific expression. Originally associated with hemotologic cancers, fusion genes have recently been discovered in a wide array of solid tumors, including sarcomas, carcinomas, and tumors of the central nervous system. Fusion genes are attractive as both therapeutic targets and diagnostic tools due to their inherent expression in tumor tissue alone. Therefore, the discovery and elucidation of fusion genes in various cancer types may provide more effective therapies in the future for cancer patients.     

Structural brain alterations in children an average of 5 years after surgery and chemotherapy for brain tumors

Young children with brain tumors are often treated with high-dose chemotherapy after surgery to avoid brain tissue injury associated with irradiation. The effects of systemic chemotherapy on healthy brain tissue in this population, however, are unclear. Our objective was to compare gray and white matter integrity using MRI procedures in children with brain tumors (n = 7, mean age 8.3 years), treated with surgery and high-dose chemotherapy followed by autologous hematopoietic cell rescue (AuHCR) an average of 5.4 years earlier, to age- and gender-matched healthy controls (n = 9, mean age 9.3 years). Diffusion tensor imaging data were collected to evaluate tissue integrity throughout the brain, as measured by mean diffusivity (MD), a marker of glial, neuronal, and axonal status, and fractional anisotropy (FA), an index of axonal health. Individual MD and FA maps were calculated, normalized, smoothed, and compared between groups using analysis of covariance, with age and sex as covariates. Higher MD values, indicative of injury, emerged in patients compared with controls (p < .05, corrected for multiple comparisons), and were especially apparent in the central thalamus, external capsule, putamen, globus pallidus and pons. Reduced FA values in some regions did not reach significance after correction for multiple comparisons. Children treated with surgery and high-dose chemotherapy with AuHCR for brain tumors an average of 5.4 years earlier show alterations in white and gray matter in multiple brain areas distant from the tumor site, raising the possibility for long-term consequences of the tumor or treatment.     

Combined pancreatoduodenectomy and orthotopic liver transplantation for hepatocellular carcinoma with portal vein tumor thrombus： A case report

Hepatocellular cancer (HCC) is the most common primary malignant hepatic tumor that accounts for over 80% of primary liver tumors. The outlook for HCC is dismal if it is left untreated and the treatment for patients with HCC evolved into a complex task. The treatments for HCC are mainly surgical therapies including hepatic resection (HR) and liver transplantation. Although HR is a well accepted therapy for HCC, it is not suitable for patients with advanced cirrhosis. Orthotopic liver transplantation (OLT) is considered more appropriate in cases with HCC related to cirrhosis, because it may eliminate both the tumor and the underlying liver disease. In this study, we reported a patient with HCC and portal vein tumor thrombus underwent combined pancreatoduodenectomy with OLT and survived 23 months in our center.     

Preoperative functional MR imaging localization of language and motor areas： effect on therapeutic decision making in patients with potentially resectable brain tumors

Purpose： To prospectively evaluate the effect of preoperative functional magnetic resonance （MR） imaging localization of language and motor areas on therapeutic decision making in patients with potentially resectable brain tumors. Materials and Methods： The Institutional Review Board approved this HIPAA-compliant study, and each patient gave written informed consent. Thirty-nine consecutive patients （19 male, 20 female; mean age, 42.2 years） referred for functional MR imaging for possible tumor resection were prospectively evaluated. A preoperative diagnosis of brain tumor was made in all patients.     

The Value of Interferon Combined with Hepatic Artery Chemoembolization and Portal Vein Chemotherapy in Preventing a Recurrence after Radical Resection for Hepatocellular Carcinoma

OBJECTIVE The change of cell immune function after hepatectomy of patients suffering from hepatocellular carcinoma (HCC) is usually neglected. The aim of this study was to explore the change of T cell subsets in HCC patients after hepatectomy, and to study the value of treatment with interferon (INF)combined with hepatic artery chemoembolization (HACE) and portal vein chemotherapy (PVC) to prevent recurrence after radical resection of HCC.METHODS Seventy-five HCC patients were treated with PVC and HACE at the 2nd week and 4th week after radical tumor resection. In the 2nd week after surgery, 33 pationts received INF treatment for one week. Seventy-two patients were followed up over three years. The effect of INF combined with HACE and PVC on the postoperative recurrence rate was compared with that of HACE and PVC treatment. Changes of T cell subsets in the peripheral blood were examined with labeled monoclonal antibodies before and after hepatectomy or with use of interferon. Forty cholecystolithiasis patients who received a cholecystectomy were used as controls.RESULTS CD3^ and CD4^ cells in the peripheral blood were reduced in patients with HCC. After hepatectomy, they declined further with a decrease in the CD4^ /CD8^ ratio. The values returned to pre-operative level at the 4th week after surgery. The CD3^ and CD4^ cells and the CD4^ /CD8^ ratio increased remarkably following the use of INF. The 1-, 2- and 3-year recurrent rates of patients treated with HACE, PVC and INF in combination were 0%, 6.2% and 15.6%, respectively, while those treated only with HACE and PVC were 5.0%, 12.5% and 27.5%, respectively.CONCLUSION Patients with HCC suffer from a marked immunosuppression, which become ever more severe after hepatectomy. The combined use of HACE, PVC and INF is superior in decreasing the recurrent rate to the combination of only HACE and PVC.     

Genetic variants in TGFβ-1 and PAI-1 as possible risk factors for cardiovascular disease after radiotherapy for breast cancer

Background and purpose

It has been established that radiotherapy can increase cardiovascular disease (CVD) risk. Genetic variants, which play a role in the tissue, damage response and angiogenesis regulating TGFβ pathway might give us insight into the mechanisms underlying radiation-induced CVD. We examined the effects of two polymorphisms, TGFβ1 29C > T and PAI-1 5G > 4G, on CVD incidence.

Materials and methods

This retrospective cohort study included 422 10-year breast cancer survivors, aged <50 years at diagnosis, treated between 1977 and 1995. We collected information on treatment, oncological follow-up, CVD, CVD risk factors and genotypes.

Results

During a mean follow-up of 19.4 years, 61 patients developed CVD. Internal mammary chain (IMC) irradiation, exposing a part of the heart to radiation, was associated with a hazard ratio of 2.36 (95% CI: 1.27-4.37, p = 0.01) compared to no IMC irradiation. Compared to the C/C + C/T genotype, the T/T genotype of the TGFβ1 polymorphism was associated with hazard ratios of 1.79 (0.99-3.26, p = 0.06) and 1.74 (0.90-3.34, p = 0.10) in the total and IMC-irradiated group, respectively. We found no evidence for an association between PAI-1 5G > 4G and CVD risk.

Conclusion

Our study suggests there might be an association between the TGFβ1 29C > T polymorphism and CVD risk in long-term breast cancer survivors.     

New risk factors and new tendency for central nervous system relapse in patients with diffuse large B-cell lymphoma：a retrospective study

Background:In patients with diffuse large B?cell lymphoma (DLBCL), central nervous system (CNS) relapse is uncom?mon but is nearly always fatal. This study aimed to determine the risk factors for CNS relapse in DLBCL patients and to evaluate the effcacy of rituximab and intrathecal chemotherapy prophylaxis for CNS relapse reduction. Methods:A total of 511 patients with newly diagnosed DLBCL treated at the Sun Yat?sen University Cancer Center between January 2003 and December 2012 were included in the study. Among these patients, 376 received R?CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) as primary treatment, and 135 received CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) as primary treatment. Intrathe?cal chemotherapy prophylaxis (methotrexate plus cytarabine) was administered to those who were deemed at high risk for CNS relapse. In the entire cohort and in the R?CHOP set in particular, the Kaplan–Meier method coupled with the log?rank test was used for univariate analysis, and the Cox proportional hazards model was used for multivariate analysis. Differences were evaluated using a two?tailed test, andP<0.05 was considered signiifcant. Results:At a median follow?up of 46months, 25 (4.9%) patients experienced CNS relapse. There was a trend of reduced occurrence of CNS relapse in patients treated with rituximab; the 3?year cumulative CNS relapse rates were 7.1% in CHOP group and 2.7% in R?CHOP group (P=0.045). Intrathecal chemotherapy prophylaxis did not confer much beneift in terms of preventing CNS relapse. Bone involvement [hazard ratio (HR)=4.21, 95% conifdence interval (CI) 1.38–12.77], renal involvement (HR=3.85, 95% CI 1.05–14.19), alkaline phosphatase (ALP) >110U/L (HR=3.59, 95% CI 1.25–10.34), serum albumin (ALB) <35g/L (HR=3.63, 95% CI 1.25–10.51), treatment with rituxi?mab (HR=0.34, 95% CI 0.12–0.96), and a time to complete remission≤ 108days (HR=0.22, 95% CI 0.06–0.78) were independent predictive factors for CNS relapse in the entire cohort. Bone involvement (HR=4.44, 95% CI 1.08–18.35), bone marrow involvement (HR=11.70, 95% CI 2.24–60.99), and renal involvement (HR=10.83, 95% CI 2.27–51.65) were independent risk factors for CNS relapse in the R?CHOP set. Conclusions:In the present study, rituximab decreased the CNS relapse rate of DLBCL, whereas intrathecal chemo?therapy prophylaxis alone was not suffcient for preventing CNS relapse. Serum levels of ALB and ALP, and the time to complete remission were new independent predictive factors for CNS relapse in the patients with DLBCL. In the patients received R?CHOP regimen, a trend of increased CNS relapse was found to be associated with extranodal lesions.     

Prognositic factors and clinicopathologic characteristics of small gastrointestinal stromal tumor of the stomach： a retrospective analysis of 31 cases in one center

Objective： To analyze the clinicopathologic characteristics and prognostic factors of small gastrointestinal stromal tumor （GIST） of the stomach. Methods： A total of 31 small gastric GIST patients, including 10 males and 21 females, with a median age of 58 years （37- 81 years）, who underwent surgery at any time from 1999 to 2012 were included in this study. The clinical records of the patients were analyzed retrospectively. Results： Abdominal discomfort and pain （10 cases, 32.3%, respectively） were the two most common complaints among the patients. All patients received surgery, 11 received gastric wedge resection, 11 received subtotal gastrectom）5 5 received laparoscopic gastric wedge resection, and 4 received endoscopic submucosal dissection. No severe adverse complication was observed. A total of 29 patients （93.5%） were followed up. During the follow-up, 2 patients were found to exhibit tumor recurrence, and 1 patient had liver metastases. One patient died of tumor progressionwhile another died of another malignant tumor. Median progression free survival （PFS） time was 120.3 months, and median overall survival （OS） time was 130.4 months. Conclusion： Small gastric GIST has better prognosis. Surgery is the best choice for therapy. Micro-invasive procedures are safe and effective for elective patients. Tumor necrosis, tumor bleeding, and muscle invasion are potential prognostic factors of small gastric GIST.     

Implantation of 125Ⅰ seeds for recurrence cervical node of head and neck tumor after external beam radiotherapy

Objective To summarize the efficacy and the feasibility of 125I seed implantation for recurrence cervical lymph node of head and neck tumor after radiotherapy or radiotherapy plus neck dissection. Methods Thirty-six patients with the recurrence cervical lymphnode of head and neck tumor after radiotherapy (17 patients) or radiotherapy plus neck dissection (19 patients) were treated with 125I seed implantation guided by ultrasound or CT under local anesthesia. The median number of seeds was 27( range from 3 to 78 ). Postoperative quality evaluation were routinely obtained for all patients. The actuarial D90 ranged from 90-160 Gy (median, 130 Gy). Results The follow-up rate was 100%. The number of the patients who were followed up over 1-and 2-year were 11 and 3. The overall response rate was 81%. The 1-and 2-year over local control rates, over survival rates were 69% and 35%, 50% and 22%, respectively.The 1-and 2-year local control rates in patients with recurrence node after radiotherapy plus neck dissection were 72% and 54%, while those were 67% and 50% in patients with recurrence node after radiotherapy,respectively (χ2=00,P=0.965). The 1-and 2-year survival rates in two groups were 48%, 13% , and 51%, 39%, respectively (χ2=0.17, P=0.676). Conclusions 125I seed implantation is a safe,minimal invasive with low morbidity and high efficacy salvage treatment method for cervical lymph node recurrence of head and neck tumor after radiotherapy with or without neck dissection.     

Rituximab in Combination with CHOP, an Effective and Well-tolerated Salvage Regimen for Diffuse Large B-Cell Lymphoma

OBJECTIVE To evaluate the clinical effect of the R-CHOP regimen (rituximab in combination with cyclophosphamide, epirubicin, vincristine and prednisone) in treating refractory or relapsed diffuse large B-cell lymphoma (DLBCL), as a salvage therapy for DLBCL. METHODS Eighteen patients with refractory or relapsed DLBCL who were treated with the R-CHOP regimen from 2001 to 2006 in hospitals in Jilin Province were analyzed retrospectively. The response rate, change of serum lactate dehydrogenase (LDH), time to progression (TTP) and toxicity were observed. RESULTS The R-CHOP regimen can achieve a higher response rate, decrease serum LDH to a larger extent and obtain longer TTP than a con- ventional secondary regimen. The main adverse effects were similar to con- ventional chemotherapy. CONCLUSION The R-CHOP regimen is one of the most effective sec- ondary therapies for DLBCL.     

Predictive factors for the local recurrence and distant metastasis of phyllodes tumors of the breast：a retrospective analysis of 192 cases at a single center

The local recurrence rate of phyllodes tumors of the breast varies widely among different subtypes, and distant metastasis is associated with poor survival. This study aimed to identify factors that are predictive of local recurrence-free survival （LRFS）, distant metastasis-free survival （DMFS）, and overall survival （OS） in patients with phyllodes tumors of the breast. Clinical data of all patients with a phyllodes tumor of the breast （n = 192） treated at Sun Yat-sen University Cancer Center between March 1997 and December 2012 were reviewed. The Pearson X2 test was used to investigate the relationship between clinical features of patients and histotypes of tumors. Univariate and multivariate Cox regression analyses were performed to identify factors that are predictive of LRFS, DMFS, and OS. In total, 31 （16.1%） patients developed local recurrence, and 12 （6.3%） developed distant metastasis. For the patients who developed local recurrence, the median age at the diagnosis of primary tumor was 33 years （range, 17-56 years）, and the median size of primary tumor was 6.0 cm （range, 0.8-18 cm）. For patients who developed distant metastasis, the median age at the diagnosis of primary tumor was 46 years （range, 24-68 years）, and the median size of primary tumor was 5.0 cm （range, 0.8-18 cm）. In univariate analysis, age, size, hemorrhage, and margin status were found to be predictive factors for LRFS （P = 0.009, 0.024, 0.004, and 0.001, respectively）, whereas histotype, epithelial hyperplasia, margin status, and local recurrence were predictors of DMFS （P = 0.001, 0.007, 0.007, and 〈 0.001, respectively）. In multivariate analysis, independent prognostic factors for LRFS included age [hazard ratio （HR） = 3.045, P = 0.005], tumor size （HR = 2.668, P = 0.013）, histotype （HR = 1.715, P= 0.017）, and margin status （HR = 4.530, P〈 0.001）. Histotype （DMFS： HR = 4.409, P= 0.002; OS： HR = 4.194, P= 0.003） and margin status （DMFS： HR = 2.581, P= 0.013;     

A case of successful surgical resection after repeated transcatheter arterial chemoembolization for far advanced multiple hepatocellular carcinomas in both lobes associated with Vp₂ portal vein tumor thrombus

Treatments for hepatocellular carcinoma (HCC) include surgical resection, transcatheter arteral chemoembolization (TACE), percutaneous local therapy and systemic chemotherapy. However, it is difficult to perform a curative treatment for patients with far advanced multiple hepatocellular carcinomas. Here we report a case of successful surgical resection after repeated TACE for far advanced multiple hepatocellular carcinomas in both lobes associated with Vp? portal vein tumor thrombus. A 54-year-old male who had multiple HCC lesions in lateral, median and right lobes with portal vein tumor thrombus was admitted to our hospital. Three attempts of TACE resulted in a successful control of the tumors in the right lobe. Left hepatic lobectomy was therefore performed, and a relapse-free survival was obtained for over 5 years after surgery.