Establishment and Characterization of a Novel Pleuropulmonary Blastoma Cell Line.

Purpose: Pleuropulmonary blastoma (PPB) is an infrequently encountered childhood malignant intrathoracic neoplasm associated with unfavorable clinical behavior. Since a well-characterized preclinical model is essential for developing competent agents for PPB, we aim to establish and characterize the world's first cell line of PPB, and attempt to perform the cytotoxicity assay on the PPB cell line.

Experimental Design: The index case is a 2-year-old female who developed a right thoracic tumor that was surgically removed and treated with multi-agent chemotherapy.

Establishment and characterization of a novel MDM2/MYCN-co-amplified neuroblastoma cell line, NBN-SHIM, established from a late recurrent stage MS tumor.

The biological heterogeneity of neuroblastoma underscores the need for an in vitro model of each molecularly defined subgroup to investigate tumorigenesis and develop targeted therapies. We have established a permanently growing cell line from a 12-year-old girl who developed a late recurrent stage MS, MDM2-amplified neuroblastoma arising in the liver and performed histological, molecular, cytogenetic, exome, and telomere analyses of the recurrent tumor and the cell line. On histology, the recurrent tumor was immunoreactive for TP53, CDKN1A, and MDM2.

A short-term three dimensional culture-based drug sensitivity test is feasible for malignant bone tumors.

The feasibility of a short-term, three-dimensional (3D) culture-based drug sensitivity test (DST) for surgically resected malignant bone tumors, including osteosarcoma (OS), was evaluated utilizing two OS cell line (KCS8 or KCS9)-derived xenograft (CDX) models. Twenty-three (KCS8) or 39 (KCS9) of 60 tested drugs were likely effective in OS cells derived from a cell line before xenografting. Fewer drugs (19: KCS8, 26: KCS9) were selected as effective drugs in cells derived from a CDX tumor, although the drug sensitivities of 60 drugs significantly correlated between both types of samples.

Severe RAS-Associated Lymphoproliferative Disease Case with Increasing αβ Double-Negative T Cells with Atypical Features.

Autoimmune lymphoproliferative syndrome (ALPS) is a disease of lymphocyte homeostasis caused by FAS-mediated apoptotic pathway dysfunction and is characterized by non-malignant lymphoproliferation with an increased number of TCRαβ+CD4-CD8- double-negative T cells (αβDNTs). Conversely, RAS-associated leukoproliferative disease (RALD), which is caused by gain-of-functional somatic variants in KRAS or NRAS, is considered a group of diseases with a similar course. Herein, we present a 7-year-old Japanese female of RALD harboring NRAS variant that aggressively progressed to juvenile myelomonocytic leukemia (JMML) with increased αβDNTs.

Infantile hepatic hemangioma and hepatic mesenchymal hamartoma in an infant associated with placental mesenchymal dysplasia: a case report.

Background: Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma.

A novel risk stratification model based on the Children's Hepatic Tumours International Collaboration-Hepatoblastoma Stratification and deoxyribonucleic acid methylation analysis for hepatoblastoma.

Introduction: Hepatoblastoma (HB) is the most common paediatric liver tumour, and epigenetic aberrations may be important in HB development. Recently, the Children's Hepatic Tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) developed risk stratification based on clinicopathological factors. This study aimed to construct a more accurate model by integrating CHIC-HS with molecular factors based on DNA methylation.

Clinicopathological study of surgery for pulmonary metastases of hepatoblastoma with indocyanine green fluorescent imaging.

Background: The prognosis of metastatic hepatoblastoma remains poor; to improve it, pulmonary metastasis must be controlled. Indocyanine green (ICG) fluorescent imaging has been used recently for lung metastasectomy. The objective of our study was to clarify the usefulness of ICG imaging for lung metastasectomy of hepatoblastoma using detailed clinicopathological analysis.

Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals.

Background/purpose: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades.

Methods: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015.

A MicroRNA Cluster in the Imprinted Region on Chromosome 14q32.2 Is Dysregulated in Metastatic Hepatoblastomas.

Hepatoblastoma (HB) is the most common malignant liver neoplasm in children. Despite progress in HB therapy, outcomes for patients with metastatic disease remain poor. Dysregulation of miRNA expression is one of the potential epigenetic mechanisms associated with pathogenesis of HB.

A novel method for isolating lymphatic endothelial cells from lymphatic malformations and detecting PIK3CA somatic mutation in these isolated cells.

Purpose: Tissue disaggregation and the cell sorting technique by surface markers has played an important role in isolating lymphatic endothelial cells (LECs) from lymphatic malformation (LM). However, this technique may have the drawback of impurities or result in isolation failure because it is dependent on surface marker expressions, the heterogeneity of which has been found in the lymphatic system. We developed a novel method for isolating LM-LECs without using whole tissue disaggregation.

Lymphatic malformations compromising the upper airway in children: ultrasound-guided intralesional focal sclerotherapy with bleomycin targeting culprit lesions.

Purpose: Lymphatic malformations (LMs) compromising the upper airway is a life-threatening and intractable disease. Here, we establish a novel method to perform intralesional focal sclerotherapy targeting the culprit for airway stenosis.

Methods: Between July 2015 and February 2020, 11 patients with airway-compromising LMs were enrolled.

Laparoscopic partial circular myectomy for congenital esophageal stenosis due to tracheobronchial remnant.

Congenital esophageal stenosis (CES) is a rare anomaly, and its appropriate management is not well established. This may be the first report describing laparoscopic partial circular myectomy of the esophageal wall in an infant with CES caused by tracheobronchial remnant (TBR). The narrow segment of the esophagus was laparoscopically detected 1 cm above the esophagogastric junction because the segment was whiter and narrower than the other parts of the esophagus.

The Collagen Gel Droplet-embedded Culture Drug Sensitivity Test in Relapsed Hepatoblastoma.

There are few treatment options for patients with unresectable or refractory hepatoblastoma which has failed to respond to the standard treatment. The rarity of the disease and lack of experimental materials have hampered the development of new treatments. In this study, the collagen gel droplet-embedded culture drug sensitivity test was used to evaluate the effectiveness of the multikinase inhibitors sorafenib and sunitinib, and other drugs, in relapsed hepatoblastoma tumor tissues.

Continuous transanal decompression for infants with long- and total-type Hirschsprung's diseases as a bridge to curative surgery: a single-center experience.

Background: The purpose of this study is to assess the usefulness of continuous bowel decompression using an indwelling transanal tube (ITT) for preoperative management in infants with long-segment (L)- or total (T)-type Hirschsprung's disease (HD).

Case Presentation: Between 2012 and 2015, seven patients with L- or T-type HD underwent preoperative bowel management by continuous bowel decompression using an ITT during waiting period for curative surgery. Continuous bowel decompression was done using an ITT, a 10-12F flexible dual lumen tube placed through the rectum up to the dilated colon under fluoroscopic guidance and secured to the bilateral buttocks.

Usefulness of a recanalized umbilical vein for vascular reconstruction in pediatric hepatic surgery.

Purpose: Pediatric surgeons currently engage in various abdominal vascular surgeries, which sometimes require vascular conduits or grafts. Herein, we report our experience with patients undergoing vascular reconstruction using a recanalized umbilical vein (rUV) and their long-term outcome.

Method: Five patients with extrahepatic portal vein obstruction (EHPVO) underwent mesenterico-/porto-left portal vein (PV) bypass surgery using a short rUV conduit with an interposition vein graft.

Standard values of rapid turnover proteins and zinc in Japanese children.

Measurement of rapid-turnover proteins has an established place in nutrition assessment and is partly dependent on the zinc metallo-enzymes involved. We investigated the reference values of rapid turnover proteins and zinc in Japanese children. This cross-sectional study was conducted at a single center.

Chylothorax associated with child abuse.

We report a case of right chylothorax associated with physical abuse in a 10-month-old boy who presented with respiratory decompensation. Chylothorax was improved by thoracic drainage and nutrition management, such as fasting followed by medium-chain triglyceride milk. Chest computed tomography on admission showed bilateral old rib fractures.

Impact of an external lengthening procedure on the outcome of long-gap esophageal atresia at our hospitals.

Purpose: To demonstrate the outcome of external lengthening for long-gap esophageal atresia (LGEA) at our hospitals.

Methods: Five patients with LGEA underwent external lengthening between 2010 and 2014 (group A), and 11 patients with LGEA underwent other lengthening techniques between 1990 and 2011 (group B). We compared the procedure and outcome between these two groups.

A case of alpha-fetoprotein-producing gastric cancer in a child presenting with rupture of multiple liver metastases.

We report a 14-year-old boy with alpha-fetoprotein-producing gastric cancer (AFPGC) who was found with ruptured metastatic tumor in the liver. AFPGC is exceedingly rare in pediatric age. It often shows metastases to the liver and should be included in differential diagnoses of liver tumors with increased serum AFP.

Navigation using indocyanine green fluorescence imaging for hepatoblastoma pulmonary metastases surgery.

To achieve precise and sensitive detection of chemotherapy-resistant hepatoblastoma pulmonary metastases, we performed surgery using indocyanine green (ICG) fluorescence imaging navigation. Lung metastasectomies were performed in 10 patients aged from 1 to 11 years. ICG (0.

Type IV laryngotracheoesophageal cleft repair by a new combination of lateral thoraco-cervical and laryngoscopic approaches.

Type IV laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that is associated with high morbidity and mortality despite various forms of surgical repair. This article presents our strategy for surgical management of type IV LTECs using a combination of lateral thoraco-cervical and laryngoscopic approaches.