儿童及青少年卵巢肿瘤治疗进展

：青少年卵巢肿瘤临床上较为少见,但却是青少年人群最常见的妇科肿瘤。其中生殖细胞肿瘤占首位,以畸胎瘤居多,交界性卵巢肿瘤较少见,恶性肿瘤更为少见,约占1％。青少年卵巢肿瘤较之成年人卵巢肿瘤有其特殊性及复杂性,治疗以手术为主,并尽量保留性腺功能及生育功能。随着以铂类为主联合化疗的实施,恶性生殖细胞肿瘤患者的5年生存率超过90％,因此保留生育能力成为临床医生必须面对的另一问题。     

Intraspinal organ in man]

In the ependymal zone of the spinal cord at the LI-SIII level an ependymal glandular organ has been described. Its highest secretory activity coincides with the period of the greatest functional activity of the human reproductive system functioning. Certain stages in development of the organ, its cell composition, blood supply, afferent and efferent innervation have been studied. In secretory cells of the organ peptide with cardio- and vasotonic properties has been identified immunochemically. The action of the organ is considered in connection with role, which the ependymal glands of the brain play in regulation of the organism's function.     

儿童胸腔内巨大肿瘤12例分析

目的:总结儿童胸腔内巨大肿瘤的临床特点与外科治疗方式。方法:回顾性分析12例儿童胸腔内巨大肿瘤患儿的临床、病理学资料,外科处理过程,并对治疗效果进行随访。结果:4例前纵隔肿瘤中2例为梭形细胞瘤,另2例来源于胸腺,分别为良性畸胎瘤和恶性神经鞘瘤,肿瘤均被完整切除;5例后纵隔肿瘤均作肿块切除,最终病理结果4例为神经母细胞瘤,1例为神经节细胞瘤;2例术前诊断为肺肿瘤的患者中,1例作了右全肺切除,1例作右肺中下叶切除,病理报告均为胸膜肺母细胞瘤;1例术前诊断为胸腔内巨大肿块患儿行肿块切除后病理报告为间叶性软骨肉瘤。结论:手术指征、手术径路的把握及术后管理决定了胸腔内巨大肿瘤患儿的治疗效果。     

Pituitary Hormone Dysfunction After Proton Beam Radiation Therapy in Children with Brain Tumors

Objectiveo characterize endocrine dysfunction in pediatric patients with brain tumors who received proton beam (PB) radiation therapy and to compare those treated with PB radiotherapy only versus combined conventional and PB irradiation.MethodsA retrospective review of medical records of patients ≤ 18 years of age who received PB radiation therapy for a brain tumor between 2000 and 2008 was performed. Variables analyzed included patient demographics, tumor type, therapeutic modalities, radiation doses, and types and timing of endocrine dysfunction.ResultsThirty-eight patients were identified, of whom 31 (19 boys and 12 girls; mean age, 11.9 ± 3.3 years) had undergone endocrine evaluation. Of these patients, 19 received PB radiotherapy only and 12 received conventional plus PB irradiation. Before irradiation, a cranial surgical procedure was performed in 28 study subjects, and 22 received chemotherapy. The mean duration of follow-up after radiation therapy was 1.8 ± 0.8 years. Nine patients (47%) in the PB only group and 4 (33%) in the conventional plus PB group developed endocrine dysfunction (no significant difference) after cranial irradiation. Children with endocrine sequelae treated with PB irradiation alone received fewer cobalt gray equivalents than those treated with conventional plus PB irradiation (5,384 ± 268 versus 5,775 ± 226, respectively; P < .02), and pituitary hormone deficiencies were detected later during follow-up in those who received PB radiotherapy only versus conventional plus PB irradiation (1.17 ± 0.4 years versus 0.33 ± 0.11 year, respectively; P < .01).ConclusionA high rate of endocrine sequelae was seen in our study. Children with brain tumors treated with conventional plus PB irradiation developed endocrine dysfunction faster and received a higher radiation dose than those receiving PB radiotherapy only. Prior surgical treatment and chemotherapy were additional risk factors. Large prospective studies are needed to evaluate further the incidence of endocrine sequelae after PB irradiation in children. (Endocr Pract. 2011;17:891-896)     

Outcome for Children with Metastatic Solid Tumors over the Last Four Decades

Background

Outcomes for pediatric solid tumors have significantly improved over the last 30 years. However, much of this improvement is due to improved outcome for patients with localized disease. Here we evaluate overall survival (OS) for pediatric patients with metastatic disease over the last 40 years.

Procedure

The United States Surveillance, Epidemiology, and End Results (SEER) database was used to conduct this study. Patients diagnosed between 0 and 18 years of age with metastatic Ewings sarcoma, neuroblastoma, osteosarcoma, rhabdomyosarcoma or Wilms tumor were included in the analysis.

Results

3,009 patients diagnosed between 1973–2010 met inclusion criteria for analysis. OS at 10 years for patients diagnosed between 1973–1979, 1980–1989, 1990–1999 and 2000–2010 was 28.3%, 37.2%, 44.7% and 49.3%, respectively (p<0.001). For patients diagnosed between 2000–2010, 10-year OS for patients with Ewing sarcoma, neuroblastoma, osteosarcoma, rhabdomyosarcoma and Wilms tumor was 30.6%, 54.4%, 29.3%, 27.5%, and 76.6%, respectively, as compared to 13.8%, 25.1%, 13.6%, 17.9% and 57.1%, respectively, for patients diagnosed between 1973–1979. OS for neuroblastoma significantly increased with each decade. For patients with osteosarcoma and Ewing sarcoma, there was no improvement in OS over the last two decades. There was no improvement in outcome for patients with rhabdomyosarcoma or Wilms tumor over the last 30 years.

Conclusions

OS for pediatric patients with metastatic solid tumors has significantly improved since the 1970s. However, outcome has changed little for some malignancies in the last 20–30 years. These data underscore the importance of continued collaboration and studies to improve outcome for these patients.     

Intraspinal serotonergic signaling suppresses locomotor activity in larval zebrafish

Serotonin (5HT) is a modulator of many vital processes in the spinal cord (SC), such as production of locomotion. In the larval zebrafish, intraspinal serotonergic neurons (ISNs) are a source of spinal 5HT that, despite the availability of numerous genetic and optical tools, has not yet been directly shown to affect the spinal locomotor network. In order to better understand the functions of ISNs, we used a combination of strategies to investigate ISN development, morphology, and function. ISNs were optically isolated from one another by photoconverting Kaede fluorescent protein in individual cells, permitting morphometric analysis as they developed in vivo. ISN neurite lengths and projection distances exhibited the greatest amount of change between 3 and 4 days post‐fertilization (dpf) and appeared to stabilize by 5 dpf. Overall ISN innervation patterns were similar between cells and between SC regions. ISNs possessed rostrally‐extending neurites resembling dendrites and a caudally‐extending neurite resembling an axon, which terminated with an enlarged growth cone‐like structure. Interestingly, these enlargements remained even after neurite extension had ceased. Functionally, application of exogenous 5HT reduced spinally‐produced motor nerve bursting. A selective 5HT reuptake inhibitor and ISN activation with channelrhodopsin‐2 each produced similar effects to 5HT, indicating that spinally‐intrinsic 5HT originating from the ISNs has an inhibitory effect on the spinal locomotor network. Taken together this suggests that the ISNs are morphologically mature by 5 dpf and supports their involvement in modulating the activity of the spinal locomotor network. © 2018 Wiley Periodicals, Inc. Develop Neurobiol, 2018     

Somatic TP53 Mutations Are Detectable in Circulating Tumor DNA from Children with Anaplastic Wilms Tumors

BACKGROUND: Diffuse anaplastic Wilms tumor (DAWT) is a rare, high-risk subtype that is often missed on diagnostic needle biopsy. Somatic mutations in TP53 are associated with the development of anaplasia and with poorer survival, particularly in advanced-stage disease. Early identification of DAWT harboring TP53 abnormalities could improve risk stratification of initial therapy and monitoring for recurrence. METHODS: Droplet digital polymerase chain reaction (ddPCR) was used to evaluate 21 samples from 4 patients with DAWT. For each patient, we assessed TP53 status in frozen tumor, matched germline DNA, and circulating tumor DNA (ctDNA) from plasma, serum, and urine collected throughout treatment. RESULTS: Mutant TP53 was detectable in ctDNA from plasma and serum in all patients. We did not detect variant TP53 in the same volume (200 μl) of urine. One patient displayed heterogeneity of TP53 in the tumor despite both histological sections displaying anaplasia. Concentration of ctDNA from plasma/serum taken prenephrectomy varied significantly between patients, ranging from 0.44 (0.05-0.90) to 125.25 (109.75-140.25) copies/μl. We observed variation in ctDNA throughout treatment, and in all but one patient, ctDNA levels fell significantly following nephrectomy. CONCLUSION: We demonstrate for the first time that ddPCR is an effective method for detection of mutant TP53 in ctDNA from children with DAWT even when there is intratumoral somatic heterogeneity. This should be further explored in a larger cohort of patients, as early detection of circulating variant TP53 may have significant clinical impact on future risk stratification and surveillance.     

人工椎板与椎弓根钉内固定术治疗椎管内神经鞘瘤

目的:探讨人工椎板与椎弓根钉内固定术治疗椎管内神经鞘瘤的可行性,安全性,评价临床疗效.方法:回顾性分析2009年7月-2012年7月入住我院12例椎管内神经鞘瘤患者的临床资料,年龄为45岁-72岁,男9例,女3例,全部病例均采用后路全椎板切除+人工椎板+椎弓根钉内固定术治疗.结果:平均手术时间95分钟,平均出血量为380mL,平均住院时间15天.平均随访12月,全部病例术后症状均得到缓解或部分缓解.结论:人工椎板与椎弓根钉内固定联合应用治疗椎管内神经鞘瘤可取得较好疗效,尤其适用于神经鞘瘤体积较大者.