Results of the JRS-I LRA0401 and LRB0402 Japan Rhabdomyosarcoma Study Group trials for low-risk embryonal rhabdomyosarcoma.

Background: Failure-free survival (FFS) rates of low-risk patients with rhabdomyosarcoma improved in Intergroup Rhabdomyosarcoma Study IV after the escalation of cyclophosphamide total dose to 26.4 g/m. However, this dose may increase the risk of adverse events, including infertility, in some patients.

Correction to: Results of a phase II trial for high-risk neuroblastoma treatment protocol JN-H-07: a report from the Japan Childhood Cancer Group Neuroblastoma Committee (JNBSG).

In the October 2018 issue of International Journal of Clinical Oncology.

Registration-directed phase 1/2 trial of irinotecan for pediatric solid tumors.

Background: Although irinotecan hydrochloride (IRI) is a promising chemotherapeutic agent for pediatric solid tumors, its indications had been off-label in the USA, EU and Japan. Therefore, we conducted a phase 1/2 trial of IRI monotherapy in a registration-directed setting.

Methods: Children aged 2-18 years with solid tumors who were either refractory to or relapsed after standard chemotherapy were enrolled.

Results of a phase II trial for high-risk neuroblastoma treatment protocol JN-H-07: a report from the Japan Childhood Cancer Group Neuroblastoma Committee (JNBSG).

Background: The Japanese Children's Cancer Group (JCCG) Neuroblastoma Committee (JNBSG) conducted a phase II clinical trial for high-risk neuroblastoma treatment. We report the result of the protocol treatment and associated genomic aberration studies.

Methods: JN-H-07 was a single-arm, late phase II trial for high-risk neuroblastoma treatment with open enrollment from June 2007 to February 2009.

Tailor-made treatment combined with proton beam therapy for children with genitourinary/pelvic rhabdomyosarcoma.

Background: Rhabdomyosarcoma (RMS) is one of the most common soft tissue sarcomas among children. Patients who developed genitourinary/pelvic rhabdomyosarcoma (GU/P-RMS) have a higher complication ratio and relatively poorer event free survival, with local therapy being very important. While proton beam therapy (PBT) is expected to reduce co-morbidity, especially for children, this lacks firm evidence and analysis.

Clinical results of proton beam therapy for advanced neuroblastoma.

Purpose: To evaluate the efficacy of proton beam therapy (PBT) for pediatric patients with advanced neuroblastoma.

Methods: PBT was conducted at 21 sites in 14 patients with neuroblastoma from 1984 to 2010. Most patients were difficult to treat with photon radiotherapy.

Successful treatment of infants with localized neuroblastoma based on their MYCN status.

Background: The aim of this study was to evaluate the effectiveness of post-surgical chemotherapy for infants with localized neuroblastoma without MYCN amplification (MNA), and determine whether risk classification using MNA is reasonable.

Methods: Four hundred and fourteen eligible patients were registered between 1998 and 2004. Resectable patients in stage 1 and 2A/2B were treated by surgical resection only.

Enhanced antitumor effect of lower-dose and longer-term CPT-11 treatment in combination with low-dose celecoxib against neuroblastoma xenografts.

Background: We have previously reported that the combination of low-dose (5.9 mg/kg/dose) irinotecan (CPT-11) and simultaneous low-dose (5 mg/kg/dose) celecoxib, a cyclooxygenase-2 inhibitor, administered for 20 consecutive days, had synergistic antitumor activity against human neuroblastoma xenografts in mice. Possible further antitumor efficacy of lower-dose and longer-term CPT-11 combined with simultaneous low-dose celecoxib was investigated for chemosensitive TNB9 and multi-drug resistant TS-N-2nu neuroblastoma xenografts.

Enteric duplication cyst of the pancreas with duplicated pancreatic duct.

Enteric duplication cyst is one of the rarest forms of cystic lesion of the pancreas. We report a unique case of an enteric duplication cyst of the pancreas that was communicating with a duplicated pancreatic duct. A 7-year-old girl with severe acute abdominal pain was found to have a large cyst that was smoothly communicating with the dilated pancreatic duct in the pancreatic tail.

Guidelines for imaging and staging of neuroblastic tumors: consensus report from the International Neuroblastoma Risk Group Project.

Neuroblastoma is an enigmatic disease entity; some tumors disappear spontaneously without any therapy, while others progress with a fatal outcome despite the implementation of maximal modern therapy. However, strong prognostic factors can accurately predict whether children have "good" or "bad" disease at diagnosis, and the clinical stage is currently the most significant and clinically relevant prognostic factor. Therefore, for an individual patient, proper staging is of paramount importance for risk assessment and selection of optimal treatment.

Identification of therapy-sensitive and therapy-resistant neuroblastoma subtypes in stages III, IVs and IV.

The aim was to present a new model of risk stratification with high predictive sensitivity for non-localized neuroblastomas (NBs). "MYCN amplification", "unfavorable histology of the International Neuroblastoma Pathology Classification (INPC) system" and "low Ha-ras/trk A expression" could be defined as an independent predictor for high-risk NBs. A risk stratification flow chart was applied to 103 advanced NBs in which all three factors were examined and 69 were grouped as high-risk NBs of which 38 patients died.

A tissue engineering approach for prenatal closure of myelomeningocele: comparison of gelatin sponge and microsphere scaffolds and bioactive protein coatings.

Myelomeningocele (MMC) is a common and devastating malformation. As an alternative to fetal surgical repair, tissue engineering has the potential to provide a less invasive approach for tissue coverage applicable at an earlier stage of gestation. We have previously evaluated the use of gelatin hydrogel composites composed of gelatin sponges and sheets as a platform for tissue coverage of the MMC defect in the retinoic acid induced fetal rat model of MMC.

Pathogenic implications of remnant vitelline structures in gastroschisis.

Purpose: The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis.

Methods: Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed.

Difference in blood tacrolimus concentration between ACMIA and MEIA in samples with low haematocrit values.

Objectives: The aim was to compare blood tacrolimus concentrations in anaemic patients between affinity column-mediated immunoassay (ACMIA) and microparticle enzyme immunoassay (MEIA).

Methods: Blood concentrations of tacrolimus in 235 whole-blood samples from 64 patients treated with tacrolimus were determined by the two assay methods. Fifty-three samples had low haematocrit (Ht) values (<25%), whereas the other samples had normal Ht values.

Neural differentiation potential of rat amniotic epithelial cells.

Amniotic epithelial cells (AEC) are thought to represent a stem-like cell population and to be an attractive cell source for regenerative medicine, because abundant cells can be obtained noninvasively at delivery. The authors investigated the neural differentiation potential of rat AEC. Rat AEC expressed vimentin and nestin, but not c-kit, oct-4, or nanog.

Tracheal defect repair using a PLGA-collagen hybrid scaffold reinforced by a copolymer stent with bFGF-impregnated gelatin hydrogel.

Purpose: We studied the regenerated cartilage in tracheal defect repair and compared the bio-materials used versus native trachea using basic fibroblast growth factor (bFGF)-impregnated gelatin hydrogel.

Materials And Methods: A full-thickness anterior defect was created in the cervical trachea of 15 experimental rabbits. The defect was implanted with a hybrid scaffold of poly(lactic-co-glycolic acid) (PLGA) knitted mesh and collagen sponge.

Closure of the larynx for intractable aspiration in neurologically impaired patients.

We present three patients with intractable aspiration pneumonia in the setting of permanent neurologic impairment, who had received a tracheostomy and showed a juxtaposition of the innominate artery against the trachea. Neurologically impaired patients often show a juxtaposition or compression of the innominate artery against the trachea by chest deformity in the setting of severe scoliosis, which could result in a trachea-innominate artery fistula. For intractable aspiration, laryngotracheal separation is safely performed and effective in controlling aspiration, but is occasionally complicated by trachea-innominate artery fistula.

A tissue engineering approach for prenatal closure of myelomeningocele with gelatin sponges incorporating basic fibroblast growth factor.

Myelomeningocele (MMC) is a common and devastating malformation. Although fetal surgical closure may improve outcome, a less invasive approach that can be applied earlier in gestation is desirable. The objective of this study was to evaluate the therapeutic feasibility of a tissue engineering approach for prenatal coverage of MMC.

Myoblast transplantation to defecation muscles in a rat model: a possible treatment strategy for fecal incontinence after the repair of imperforate anus.

Purpose: Infants with higher anorectal anomalies often develop fecal incontinence after surgical reconstruction mainly due to the incomplete development of defecation muscles. We investigated the possibility of defecation muscle regeneration by myoblast transplantation to improve fecal continence.

Methods: Myoblasts from F344 female rats at ages of 1 day, 1, 2, 3, 4, 8, and 12 weeks were prepared by a preplating method.

Prolonged low-dose administration of the cyclooxygenase-2 inhibitor celecoxib enhances the antitumor activity of irinotecan against neuroblastoma xenografts.

Cyclooxygenase (COX)-2 is overexpressed in many human tumors including neuroblastoma (NB) and promotes tumor progression. We evaluated the antitumor effect of irinotecan (CPT-11) treatment combined with prolonged very low-dose administration of celecoxib, a selective COX-2 inhibitor, against three human NB xenografts, TNB9, TS-N-2nu, and TS-N-5nu. In addition, the effects of the celecoxib-combined treatment were examined on tumor cell proliferation, apoptosis, angiogenesis, and expression of vascular endothelial growth factor and apoptosis-related proteins in xenografts.