Pattern of recurrence in children with midline posterior fossa malignant neoplasms

BACKGROUND: Surveillance imaging of the brain and spinal neuraxis in patients with posterior fossa malignant tumors is commonly performed, with the assumption that early detection of tumor recurrence will improve outcome. However, the benefit of this imaging has not been proven. PURPOSE: To evaluate the usefulness of spinal surveillance imaging in children with nonmetastatic (at diagnosis, M0) posterior fossa ependymoma and medulloblastoma. MATERIALS AND METHODS: This retrospective study included 65 children (3 months to 16 years, mean 5.7 years) treated between 1985 and 1997 for ependymoma (22) and medulloblastoma (43). Medical records were reviewed for pathology and treatment data. Serial imaging of the head and spine was reviewed for evidence of tumor recurrence. RESULTS: Twenty-four patients (37 %) had tumor recurrence, including 13 with ependymoma and 11 with medulloblastoma. Of the 17/24 recurrent patients initially diagnosed as M0 (6 medulloblastoma and 11 ependymoma), 13 (76 %) had a cranial recurrence only, and 4 (24 %) presented with concomitant cranial and spinal recurrence. No M0 patient presented solely with spinal metastases at recurrence. CONCLUSION: This study suggests that spinal surveillance imaging in patients with posterior fossa ependymoma or medulloblastoma initially staged as M0 may not be useful, as these patients initially recur intracranially. Thus, until an intracranial recurrence is detected, these patients may be spared the time, expense and sedation risk necessary for spinal imaging.     

Surveillance imaging in children with medulloblastoma (posterior fossa PNET)

Background. The use of surveillance imaging in children with medulloblastoma has been criticised. The aim of this study was to determine what proportion of relapses are detected by surveillance and whether these are found at a relatively favourable stage.¶Methods. This study was a retrospective review of the medical charts and imaging studies of 89 patients treated at a single children's cancer centre. Relapse was defined as evidence of an increase in volume of residual tumour of greater than 25 % or the presence of metastases, or new onset of positive CSF cytology. Relapse was termed symptomatic if it was diagnosed by tests performed because of new symptoms that occurred in the interval between surveillance examinations. Asymptomatic relapse was diagnosed solely on the basis of surveillance imaging. Survival time to relapse was calculated from the date of the first surgical procedure.¶Results. Surveillance imaging detected 17 (71 %) of the 24 relapses that occurred later than 6 months after diagnosis. All seven patients who presented with symptoms between scans have died, with a median survival from relapse of 5 months. Median survival from relapse in the patients detected by surveillance was 44 months, and four remain alive at 44–75 months. The patients detected by surveillance tended to have less advanced disease, which was more amenable to salvage therapy.¶Conclusion. This type of study cannot prove that surveillance imaging improves survival in children with medulloblastoma because of the effects of lead time and length biases. Despite this, surveillance does appear to be effective in detecting potentially curable medulloblastoma relapses and should be offered to all patients.     

Timing of radiation in children with medulloblastoma/PNET

BACKGROUND: To evaluate the importance of timing of radiation therapy in children with PNET/medulloblastoma, treated at the Brazilian National Cancer Institute (INCA). PROCEDURE: The records of 101 children with confirmed diagnosis of medulloblastoma were retrospectively reviewed. Patients had a median follow-up of 48 months (0.5-241 months). The age varied from 0.8 to 17.5 years (median: 7.6 years) and 21.7% were 3 years old or younger. RESULTS: According to the data collected from patients that received treatment for medulloblastoma from 1983 to 2001, the overall survival (OS) rate was 53% and the Disease Free Survival (DFS) rate was 40%. Multivariate analysis showed that under age 3 years, presence of neoplasic cells in the cerebrospinal fluid (CSF) at presentation or subtotal tumor resection resulted in a worse OS. The patients that received a biological effective dose (BED) greater than 44 Gy10 had better prognosis. Two-thirds of the patients had complete response after the initial treatment. Among them, 50% (34 patients) recurred, and of those 34 patients, 42% of them (14 patients) had recurrence in the posterior fossa. CONCLUSION: Surgery with total resection of the tumor and absence of neoplasic cells in the CSF are effective predictors of better OS. Radiotherapy was more effective when a BED was greater than 44 Gy10.     

Prognostic factors and secondary malignancies in childhood medulloblastoma

PURPOSE: Little is known of the outcome of long-term survivors of childhood medulloblastoma, one of the most common pediatric malignancies. To determine the potential for secondary malignancies, a retrospective outcome evaluation in 88 consecutive cases of childhood medulloblastoma was performed. PATIENTS AND METHODS: The records of all patients with childhood medulloblastoma diagnosed at Children's National Medical Center in Washington, DC from 1969 through 1997 were reviewed. RESULTS: The median follow-up time was 92 months (range 6-257 months). Overall survival was 59% at 5 years and 52% at 10 years. Univariate analysis showed that age at diagnosis, extent of surgical resection, presence of metastatic disease (M stage), ventriculoperitoneal shunt placement within 30 days from diagnosis, posterior fossa radiation therapy dose, and adjuvant chemotherapy significantly affected survival. Although based on small numbers, the risk of second neoplasms was significantly increased in this cohort. Multiple basal cell carcinomas developed in the areas of radiation therapy in two patients; these patients also had nevoid basal cell carcinoma syndrome (NBCCS) diagnosed. One other patient died of glioblastoma multiforme 8 years after treatment of medulloblastoma. A meningioma developed in another patient 10 years after radiation therapy. CONCLUSION: As survival of medulloblastoma patients improves, increased surveillance regarding secondary malignancies is required, especially because radiation-induced tumors may occur many years after treatment. These two cases of NBCCS also illustrate the importance of considering the concomitant diagnosis of NBCCS in young patients with medulloblastoma. In those patients, alternative therapy should be considered to minimize radiation therapy-related sequelae.     

Patterns of abdominal relapse and role of sonography in Wilms tumor

This study characterizes the patterns of abdominal recurrence of Wilms tumor and describes the role of sonography in its detection. Twelve patients who had initial tumor recurrence in the abdomen were evaluated. Five patients had recurrence in the kidney; all had nephrogenic rests detected by computed tomography (CT) or magnetic resonance (MR) imaging but not by sonography. The remaining 7 patients had recurrence in the peritoneum (4), the nephrectomy site (2), or the regional lymph nodes (1); tumor spillage had occurred in five of these patients. Four recurrences were detected during therapy, and eight within 3 years after completion of therapy. Seven of the 12 recurrences were first detected by sonography. All 11 sonograms obtained at the time of relapse showed tumor recurrence. Nine patients died a median of 10 months after relapse. The results suggest that regular sonographic surveillance for 3 years after therapy is likely to reveal most abdominal recurrences. Supplementation with CT or MR imaging is indicated for detection of nephrogenic rests.     

PATTERNS OF ABDOMINAL RELAPSE AND ROLE OF SONOGRAPHY IN WILMS TUMOR

This study characterizes the patterns of abdominal recurrence of Wilms tumor and describes the role of sonography in its detection. Twelve patients who had initial tumor recurrence in the abdomen were evaluated. Five patients had recurrence in the kidney; all had nephrogenic rests detected by computed tomography (CT) or magnetic resonance (MR) imaging but not by sonography. The remaining 7 patients had recurrence in the peritoneum (4), the nephrectomy site (2), or the regional lymph nodes (1); tumor spillage had occurred in five of these patients. Four recurrences were detected during therapy, and eight within 3 years after completion of therapy. Seven of the 12 recurrences were first detected by sonography. All 11 sonograms obtained at the time of relapse showed tumor recurrence. Nine patients died a median of 10 months after relapse. The results suggest that regular sonographic surveillance for 3 years after therapy is likely to reveal most abdominal recurrences. Supplementation with CT or MR imaging is indicated for detection of nephrogenic rests.     

101例儿童复发髓母细胞瘤的序贯治疗生存分析

目的 分析儿童复发髓母细胞瘤(MB)的临床特征,并探讨进一步治疗方案.方法 回顾性分析2011年8月至2017年7月该院收治的101例复发MB患儿的临床资料,随访截至2020年7月31日.采用Kaplan-Meier法进行生存分析,Cox回归模型进行多因素回归分析.结果 95例患儿给予诱导缓解治疗,51例有效,有效率5...     

Reduction of absolute lymphocyte count in children with recurrent medulloblastoma

The complete blood cell (CBC) count with differential cell counts of 36 children with medulloblastoma showed a substantial reduction of the absolute lymphocyte count at recurrence. The preoperative value of the CBC count with differential cell counts failed to predict the survival of patients with medulloblastoma. A prospective study with serial measurement of the CBC count with differential cell counts will be needed to determine sensitivity and specificity of the CBC count with differential cell counts to clinical application for early detection of recurrence of medulloblastoma.     

Management of hydrocephalus in children with posterior fossa tumors: role of tumor surgery

OBJECTIVE: The majority of children with posterior fossa tumors have hydrocephalus (HC) at the time of presentation. There is no consensus regarding the management of HC in these children. Here, we report the rate of cure of HC with tumor surgery alone. PATIENTS AND METHODS: This is a retrospective study of 87 children with posterior fossa tumors in which 35 patients had medulloblastoma, 38 had astrocytoma and 14 had ependymoma. The mean age at presentation was 7.3 years (range: 0.2-19.7 years). All patients underwent tumor resection and were followed with close clinical and image surveillance to detect persistent HC. HC was treated with endoscopic third ventriculostomy (ETV) or shunt. We have focused on patients who needed permanent cerebrospinal fluid diversion (ETV or shunt) within 30 days of tumor resection. HC presenting after this time period is, in the majority of cases, due to tumor recurrence or progression. In this series, 24/87 (28%) patients had a suboccipital craniectomy and 63/87 (72%) had a craniotomy. RESULTS: At the time of presentation, 69/87 (79%) patients had symptomatic HC. In 41/69 (59%) patients presenting with HC, the HC was cured by tumor resection alone. The HC cure rate for patients with astrocytoma was 83%, whereas it was 47% for patients with medulloblastoma and 54% for patients with ependymoma. The cure rate was equal in the craniectomy and craniotomy groups. CONCLUSIONS: An 87% cure rate of HC by tumor resection alone in children with posterior fossa astrocytoma warrants no change in treatment strategy. However, the low cure rate of HC by tumor resection alone in patients with medulloblastoma and ependymoma raises the issue of whether these patients would benefit from preresection ETV.     

Outcome after relapse in an unselected cohort of children and adolescents with Ewing sarcoma

BACKGROUND: Survival after relapse in patients with Ewing sarcoma is very poor and this retrospective study attempts to identify of prognostic factors predicting survival after relapse. PROCEDURE: A total of 191 patients with localised Ewing sarcoma were registered in the ET-2 trial of the United Kingdom Children's Cancer Study Group (UKCCSG). All patients received standardised primary treatment with chemotherapy and surgery and or radiotherapy as local modality treatment. Sixty-four patients who relapsed are included in this report. Treatment at relapse was variable and included chemotherapy, surgery, radiotherapy and high dose therapy (HDT) or megatherapy with peripheral stem cell transplantation (PBSCT) or autologous bone marrow transplantation (ABMT) in various combinations. A subgroup of patients had only non-specific symptomatic treatment at relapse. Both univariate and multivariate methods were used to investigate variables affecting survival after relapse. RESULTS: The overall actuarial median survival from relapse for all patients was 14 months (95% CI 11-16 months). Univariate analysis showed that males had a longer survival (median, 16 months vs. 11 months); patients who relapsed while on treatment did worse (median, 3 months vs. 16 months) and patients who had a longer disease-free interval (DFI) prior to relapse had a better outcome (DFI <1 year, median survival = 3 months; DFI 1-2 years, survival = 8 months; DFI > 2 years, median survival = 24 months, P < 0.001). Multivariate analysis confirmed that duration of first remission was the only factor associated with longer survival after relapse. CONCLUSIONS: These data suggest that although aggressive therapy may delay disease progression after relapse for some children, the course of the disease after relapse is usually fatal. International co-operative studies are needed to evaluate new strategies.     

Bone and gallium scintigraphy in children with rhabdomyosarcoma: A 10-year review

Technetium-99m bone and Gallium-67 scintigraphy has been widely used in the management of children with solid tumors. A retrospective review of all patients with rhabdomyosarcoma (RMS) from January 1980 to December 1989 was undertaken in order to determine the sensitivity and specificity of scintigraphy in detection of metastatic disease and to determine optimum frequency of serial scans in follow up. Over the 10-year period, 40 patients were diagnosed and treated for RMS: 22 were newly diagnosed from 1980-1984 and of these 16 had a minmum 5-year disease-free period whilst 6 died of the disease, giving a cure rate of 73%. A total of 271 bone and 236 Gallium-67 scans were performed. With respect to detection of metastatic disease in all tissues, Gallium-67 scans had a sensitivity of 84%, specificity 95% and bone scans had a sensitivity of 70% and specificity 95%. Bone scan sensitivity and specificity for skeletal metastases were 100% and 95%, respectively. Considering only patients with Gallium avid primary tumors, the Gallium-67 scan sensitivity was 94%. Ten patients developed new metastatic disease or primary recurrence after starting therapy, 8/10 within 12 months of diagnosis. Frequent surveillance scanning in the treatment phase, 2 years post-diagnosis, is of value in the early detection of metastases and monitoring disease response to therapy. Surveillance scanning after completion of treatment is more difficult to justify.     

Pediatric oncology surveillance imaging: Two recommendations. Abandon CT scanning,and randomize to imaging or solely clinical follow‐up

Radiologic assessments in children with cancer provide information crucial to patient management at diagnosis and during follow‐up. Many studies have now been published, however, questioning the usefulness of off‐therapy surveillance imaging. There is growing concern regarding the hazards from diagnostic irradiation to young patients, most notably from CT scanning. In this paper we advocate abandoning repeated CT surveillance in young patients with a previously treated solid malignancy not arising in the central nervous system. In addition, randomized studies of imaging surveillance versus no imaging surveillance strategies are needed to determine whether earlier detection of recurrence results in improved survival. Pediatr Blood Cancer 2014;61:3–6. © 2013 Wiley Periodicals, Inc.     

ASYMPTOMATIC BACTERIURIA IN SCHOOL GIRLS

ABSTRACT: Lindberg, U. (Department of Paediatrics and Department of Immunology, Institute of Medical Microbiology, University of Goteborg, Goteborg, Sweden). Asymptomatic bacteriuria in school girls. V. The clinical course and response to treatment. Acta Paediatr Scand, 64:718, 1975.–The short-term prognosis of 116 school girls with asymptomatic bacteriuria (ABU) treated or left untreated is reported. In untreated girls a spontaneous remission was obtained in 11% within one year. A short course of nitrofurantoin eliminated the bacteriuria in 93% of the girls in the treatment group. The recurrence rate was 30% in one year in the patients that became abacteriuric spontaneously, after a bladder washout test or after treatment with penicillin or nitrofurantoin. The first recurrence occurred within 3 months in 79% (19/24). Nineteen of the 24 patients with recurrences (79%) had a third infection within 9 months. In patients with parenchymal reduction or reflux the renal concentrating capacity increased significantly after treatment of the bacteriuria. All the girls left untreated had normal renal concentrating capacity on detection and this remained unchanged during the year of follow-up. One of 28 untreated girls attracted a symptomatic pyelonephritis caused by a strain different from that at detection of the ABU. Of 81 girls cured from the bacteriuria 24 recurred, 5 with a' symptomatic pyelonephritis and 3 with cystitis. It is concluded that strains isolated from girls with ABU do not commonly cause symptomatic pyelonephritis.     

Video-assisted thoracoscopic surgery for primary spontaneous pneumothorax in children

Purpose

There is controversy regarding the best way to prevent recurrences of primary spontaneous pneumothorax (PSP) in children. The purpose of this study was to evaluate the efficacy of video-assisted thoracoscopic surgery (VATS) for pediatric PSP.

Methods

We retrospectively reviewed patients under 29 years of age who underwent VATS for PSP between March 2005 and February 2011. Patients were divided into 2 groups: children (under the age of 17 years) and young adults (over the age of 18 years).

Results

Two hundred eighty-one VATS procedures in 257 patients were included in this study. The mean follow-up was 47.1 ± 20.5 months. No mortality was observed. The mean duration of pleural drainage was 3.4 ± 2.2 days. The overall recurrence rate was 6.8 %. The operative outcomes did not differ significantly. However, the recurrence rate was significantly higher in the children’s group than the young adult group (10.6 vs. 3.9 %, P = 0.032). Younger age and postoperative prolonged air leak had a significantly higher risk of postoperative recurrence.

Conclusions

VATS is a safe and effective procedure for PSP in children. However, the risk of recurrence is increased in children and it is related to the formation of new bullae.     

儿童髓母细胞瘤的临床特点及预后相关因素分析

目的通过分析儿童髓母细胞瘤的临床特点、治疗方法和患者预后的关系,探索影响儿童髓母细胞瘤预后相关因素。方法收集2004年至2014年复旦大学附属儿科医院收治且术后病理证实为髓母细胞瘤患儿的病例资料,对所收集数据进行分组,采用单因素Kaplan-Meier法进行生存分析,统计各组病人的总体生存时间及累计生存率,采用Log-Rank检验分析各组病人累计生存率差异,采用Cox回归进行多因素分析,探讨儿童髓母细胞瘤临床因素与生存时间、生存结局的关联性。结果单因素分析结果认为,临床症状阳性数(P=0.039)、M分期(P=0.009)、术后放疗(P=0.001)、术后化疗(P=0.018)、肿瘤复发(P=0.04)是髓母细胞瘤患儿预后的影响因素;Cox多因素分析结果认为术后放疗(OR=0.291,95%置信区间:0.120~0.706,P=0.006)和化疗(OR=0.095,95%置信区间:0.024~0.384,P=0.001)是影响髓母细胞瘤患儿预后的保护因素;性别、年龄、肿瘤位置、T分期、肿瘤切除程度、病理分型等因素未发现与预后有关(P>0.05)。结论术后是否接受放疗、化疗对患儿预后影响较大,而临床症状的个数、M分期、肿瘤复发对患儿预后的判断价值有限,性别、年龄、肿瘤位置、T分期、肿瘤切除程度、病理分型等因素则与预后无关。     

THE LENGTH OF ANTIMICROBIAL THERAPY IN UPPER VS. LOWER URINARY TRACT INFECTION OF CHILDHOOD

Abstract. Pylkkänen, J., Vilska, J. and Koskimies, O. (The Children's Hospital, University of Helsinki, Helsinki, Finland). The length of antimicrobial therapy in upper vs. wer urinary tract infection of childhood. Acta Paediatr Scand, 70: 885, 1981.-235 infants and children were randomized to a 10-day and 42-day treatment group and followed-up for 12 months after their first urinary tract infection. The anatomical level of each symptomatic infection was determined using simple laboratory criteria. The two regimens prescribed were equally effective in eradicating the infection, but after the discontinuation of the 10-day treatment with sulfafurazole, 17 (23%) of 73 patients with their first upper urinary tract infection experienced a recurrence within one month, as compared to only one (1) of 76 subjects in the 42-day therapy group. After the phase of early recurrence, there was no difference in recurrence rate between these groups. The early recurrences were associated with the patient's early age and a short duration of symptoms before therapy. The recurrence rate of first lower UTI after 10-day therapy was significantly lower than that after 42-day treatment. The duration of antimicrobial therapy for childhood urinary tract infection should be adjusted according to the patient's age and the anatomical level of the infection. 10-day treatment may not be sufficient to prevent early recurrence of pyelonephritic infections in infants under 6 months of age.     

Recurrent focal segmental glomerulosclerosis following renal transplantation in Korean pediatric patients

The recurrence of focal segmental glomerulosclerosis (FSGS) in transplants is a well-known problem in pediatric renal transplantation (Tx). Recently, the race of the recipient was recognized as a major variable associated with disease recurrence. In view of this finding, we report on our single-center experience of FSGS recurrence in Korean children, an ethnically homogeneous Far East Asian population. Clinical records and renal biopsy specimens, both native and graft, were reviewed for all pediatric renal Txs (recipient age < or = 18 yr) performed at Seoul National University Hospital from 1984 to 1999. Twenty-two children with primary FSGS received 22 allografts for renal replacement. The mean age of disease onset in these patients was 5.9 yr. The grafts were from 12 living-related, six living-unrelated, and four cadaveric donors, and all recipients were immunosuppressed with cyclosporin A (CsA)-based regimens. Post-transplant recurrence of FSGS was confirmed in nine patients (41%). Long-term graft survival in recurrent and non-recurrent groups was not significantly different. Risk factor analysis showed that patients with a disease duration shorter than 48 months (odds ratio 11.7, 95% CI 1.53-89.1) and a glomerulosclerosis percentage of < 55% by renal biopsy (odds ratio 16.0, 95% CI 1.45-176) were at greater risk of disease recurrence. These results suggest that Korean children are similar to non-African-American youngsters in the USA and Europe with respect to post-transplant recurrence of FSGS. The same may be true of other Far Eastern Asian children.     

显微外科为主的综合治疗对小儿髓母细胞瘤预后的影响

目的 分析显微手术辅以放疗、化疗对小儿髓线细胞瘤治疗效果及其预后的影响。方法 将髓母细胞瘤患儿分为二组。手术全切除组和手术未全切除组。前才术后只接受全神经轴放射治疗，后者术后辅以放疗和化疗，观察二组治疗效果和预后的差异。结果 手术全切除组术后肿瘤复发率２９．３％，１、２、５年生存率分别为１００％、９４．１％、８２．４％；手术未全切出生率且术后肿瘤复发率８５．７％、１、２、５年的生存率分别为７１．４     

Stereotactic radiation therapy with chemotherapy in the management of recurrent medulloblastomas

Medulloblastomas are highly lethal tumors when they recur. Very few patients survive with conventional treatment. This report documents the preliminary study results of a treatment for recurrent medulloblastomas consisting of stereotactic radiation therapy (SRT) with chemotherapy. Four patients had local recurrence without apparent metastases and 8 patients had metastases with or without local recurrence. Twelve patients with 18 lesions underwent SRT as a single session (n=8) or in a hypofractionated manner (n=10) using a gamma knife or modified linear accelerator. All patients then received systemic chemotherapy. Five patients were treated with one to two sequential courses of high-dose chemotherapy with peripheral blood stem cell transplantation. The reduction in tumor size after SRT was often remarkable. Fourteen of 18 lesions treated disappeared 1-6 months after SRT. Two of 4 patients who had local recurrences without apparent metastasis at the time of SRT are alive without evidence of disease 70 and 72 months after SRT, respectively. In contrast, all 8 patients with metastasis had new lesions either in the spinal canal or on the surface of the brain outside the target area of SRT. Median progression-free survival and overall survival from the time of SRT were 9 and 19 months, respectively. The Kaplan-Meier estimates of PFS and overall survival at 3 years were 17 and 25%, respectively. One patient had brainstem edema after SRT causing bulbar palsy and quadriparesis. One patient died of toxicity of chemotherapy. Our experience suggests that local recurrence can be controlled by SRT with chemotherapy but survival of patients with metastases can not be improved effectively by SRT in conjunction with aggressive chemotherapy.     

Asymptomatic bacteriuria in school girls. V. The clinical course and response to treatment.

The short-term prognosis of 116 school girls with asymptomatic bacteriuria (ABU) treated or left untreated is reported. In untreated girls a spontaneous remission was obtained in 11% within one year. A short course of nitrofurantoin eliminated the bacteriuria in 93% of the girls in the treatment group. The recurrence rate was 30% in one year in the patients that became abacteriuric spontaneously, after a bladder washout test or after treatment with penicillin or nitrofurantoin. The first recurrence occurred withn 3 months in 79% (19/24). Nineteen of the 24 patients with recurrences (79%) had a third infection within 9 months. In patients with parenchymal reduction or reflux the renal concentrating capacity increased significantly after treatment of the bacteriuria. All the girls left untreated had normal renal concentrating capacity on detection and this remained unchanged during the year of follow-up. One of 28 untreated girls attracted a symptomatic pyelonephritis caused by a strain different from that at detection of ABU. Of 81 girls cured from the bacteriuria 24 recurred, 5 with a symptomatic pyelonephritis and 3 with cystitis. It is concluded that strains isolated from girls with ABU do not commonly cause symptomatic pyelonephritis.