A case of atypical teratoid/rhabdoid tumor in an adult, with long survival

Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that mostly occurs in early childhood and has poor prognosis despite aggressive therapy. Adult cases are rare and, as far as we are aware, only 30 cases have been reported to date. Here we present the case of a 27-year-old female with left parietal AT/RT with the chief complaint of numbness of the right superior limb. First, the tumor was surgically removed and the diagnosis was grade II glioma. With additional radiotherapy, the clinical course after surgery was favorable. After 6 years, she had an operation for recurrence and the diagnosis was grade III glioma. Temozolomide was prescribed, and a disease-free period of 2 years followed. Surgery was performed for a third time for second recurrence with histology of diffuse growth of rhabdoid cells. Immunohistochemistry was partially positive for vimentin and epithelial membrane antigen. Ki-67 labeling index was extremely high and tumor cells showed no staining of INI1 suggestive of diagnosis of AT/RT. We re-evaluated past specimens and none had immunoreactivity of INI1. Ki-67 labeling index and O-6 methylguanine DNA methyltransferase (MGMT) staining were also re-examined and both increased gradually. She is still alive without recurrence for more than 1 year. As far as we are aware, this is the second longest survival of an adult with AT/RT.     

Usage of granulocyte colony-stimulating factor every 2 days is clinically useful and cost-effective for febrile neutropenia during early courses of chemotherapy

Background  

In order to analyze the clinical activity and cost-effectiveness of granulocyte colony-stimulating factors (G-CSF), the prophylactic usage of G-CSF in patients treated with a single chemotherapy regimen during early courses was prospectively evaluated.     

Survival of skin allografts is prolonged in mice with a dominant-negative H-Ras

Ras is a guanine nucleotide-binding protein that plays a major role in regulating the proliferation of T cells. To investigate the mechanism of the Ras/mitogen-activated protein kinase pathway, one of the downstream signal-transduction pathways of T-cell receptors, in the response to alloantigen, we performed full-thickness skin grafting in the major histocompatibility complex (MHC) incompatible strain BALB/c (H-2Kd) (donor) and T-cell-specific H-Ras dominant-negative (dnRas) transgenic (tg) C57BL/6 (H-2Kb) (recipient) male mice. In vitro and in vivo dnRas tg mouse T-cell proliferation and cytotoxic T lymphocyte (CTL) activity assay were also performed. The median graft survival time in control B6/wild type (wt) mouse allografts was seven days. Conversely, the dnRas tg mouse group exhibited a significant (p<0.01) prolongation of graft survival to 15 days. However, all grafts were eventually rejected after one month. Mixed lymphocyte reaction and popliteal lymph node assay revealed that T-cell proliferation was decreased in response to alloantigen, but CTL activity was not changed in the dnRas tg mice. These results suggested that Ras is essential for peripheral T lymphocytes to respond to allo-MHC antigens, and Ras may be a molecular target for controlling transplant rejection.     

Alcohol consumption is associated with an increased risk of distal colon and rectal cancer in Japanese men: the Miyagi Cohort Study

The association between alcohol consumption and the risk of cancer of the proximal or distal colon or rectum remains controversial. We examined this association in a large population-based cohort of Japanese men. In 1990, a self-administered questionnaire on alcohol drinking and other health habits was delivered to 25,279 Japanese men aged 40 to 64 years of age. After exclusion of subjects who gave incomplete responses on alcohol drinking or prevalent cancer cases at the baseline, a total of 21,199 men remained. Of these, 307 men were diagnosed as having colorectal cancer after 11 years of follow-up. Cox proportional hazards regression models were used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs), with adjustments made for potential confounders. Compared with never drinkers, past and current drinkers had multivariate HRs of 1.1 (95% CI, 0.6-1.9) and 1.6 (95% CI, 1.1-2.2) for colorectal cancer, respectively. A dose-response relationship with current volume of alcohol drinkers was observed for cancer of the distal colon and rectum, but not for proximal colon. The multivariate HRs for distal colon and rectal cancer among current heavy drinkers (45.6 g or more ethanol per day) as compared with never drinkers were 4.2 (1.6-10.7; p for trend=0.0002) and 1.8 (1.1-3.2; p for trend=0.04), respectively. In contrast, no significant linear association was found for proximal colon cancer (p for trend=0.2). These data indicate that alcohol consumption in Japanese men is associated with a statistically significant increased risk of cancer of the distal colon and rectum, but not cancer of the proximal colon.     

Prenatal diagnosis of short-rib polydactyly syndrome type 3 (Verma-Naumoff type) by three-dimensional helical computed tomography

We present a case of short-rib polydactyly syndrome (SRPs) type 3 in which accurate prenatal diagnosis was feasible using both ultrasonography and 3D-CT. SRP encompass a heterogeneous group of lethal skeletal dysplasias. However, the phenotypes overlap with those of nonlethal skeletal dysplasias (i.e. Ellis-van Creveld syndrome and Jeune syndrome). As accurate prenatal diagnosis of SRP is helpful for parents, we used 3D-CT in the early third trimester to examine a fetus suggested to have phenotypes of 'short-rib dysplasia group' on ultrasonography. 3D-CT showed mild modification of the vertebral bodies, small ilia with horizontal acetabula and triangular partial ossification defects, and subtle metaphyseal irregularities of the femora. These CT findings and an extensive literature search regarding the phenotypes of various diseases categorized as short-rib dysplasia group led to a correct prenatal diagnosis of SRP type 3. This case exemplified the usefulness of 3D-CT for the precise prenatal diagnosis of skeletal dysplasias.     

Commotio Cordis Presenting as a Temporary Complete Atrioventricular Block in a 2-Year-Old Girl With Congenitally Corrected Transposition of the great Arteries

This report describes a 2-year-old girl with congenitally corrected transposition of the great arteries (ccTGA) who presented with transient complete atrioventricular (AV) block after a mild chest blow. Running around the house with her older sister, she fell to the floor. Her sister also fell and landed on her. The girl became cyanotic and pale and experienced a transient loss of consciousness. At arrival to the emergency department, she had regained consciousness, but she remained pale. An electrocardiogram (ECG) demonstrated complete AV block with a heart rate of 78 beats per minute (bpm). The ECG after admission showed a Wenckebach-type second-degree AV block. Day 2 after admission, a 12-lead ECG showed significant ST and T-wave abnormalities in the precordial leads, but the girl had no chest pain and a normal physical examination. Echocardiography demonstrated normal contractility of the systemic right ventricle. The first-degree AV block and the ST and T-wave abnormalities on the 12-lead ECG improved gradually without abnormal Q-waves. This is the first report of ccTGA in which a transient complete AV block naturally recovered after a presentation with commotio cordis.     

Can Western endoscopists identify the end of the lower esophageal palisade vessels as a landmark of esophagogastric junction?

Background and aim  The endoscopic landmark of esophagogastric junction (EGJ) for diagnosis of Barrett’s esophagus (BE) differs between Japan and Western countries. Japanese endoscopists use the distal end of the lower esophageal palisade vessels to localize EGJ. In the West, endoscopists use the proximal gastric folds because of concerns that palisade vessels may be difficult to recognize. We evaluated whether there were differences between American and Japanese endoscopists in the recognition of palisade vessels. Method  A total of 82 patients were enrolled in this study. Patients were referred for diagnostic esophagogastroduodenoendoscopy (EGD) at the Veterans Affairs Palo Alto Health Care System, from May to July 2008. American and Japanese endoscopists evaluated the EGJ of patients undergoing diagnostic EGD. We analyzed the differences in the recognition of the distal end of palisade vessels. We calculated the kappa statistic to measure interobserver variability. Results  Based on localization using the distal end of the palisade vessels, American and Japanese endoscopists identified the EGJ in 87.8% (72/82) and 89.0% (73/82) of cases, respectively. The kappa statistic for visualization of EGJ was 0.88 [95% confidence interval (CI): 0.73–1.00]. Conclusion  American and Japanese endoscopists similarly recognized the distal end of palisade vessels as EGJ.     

Clinical experience of the newly developed electrohydraulic lithotripter

The electrohydraulic lithotripter (EHL) is a calculus-breaking device used in percutaneous nephrolithotomy (PNL). The conventional EHL has a shortcoming in that its breakability decreases in the saline irrigation environment. The newly developed EHL (Lithotron EL-21 OLYMPUS) is designed to discharge optimum sparks during saline irrigation, without having to dilute the solution, thus providing greater convenience. We used the new EHL in 17 cases of renal calculi, 11 cases of ureteral calculi and 2 cases of reno-ureteral calculi, with a breakup efficiency of 96.7%. As a direct, incidental complication, perforation of the ureter occurred in one case of ureteral calculi (uneventful spontaneous recovery detected 3 days afterward). The new EHL has a breakup capability suitable for the PNL: the EHL provides a safe procedure, provided that one takes sufficient caution to the correct positioning of the probe against the target calculus when activating it. Since the probe shaft is flexible, permitting use with the flexible endoscope, the EHL is particularly effective in treating ureteral and renal calculi in cases where access by the rigid US probe is not feasible. One disadvantage of the EHL is that it lacks the fragment-collecting capability that the ultrasound lithotripter features.     

The prevention of peritoneal adhesions by in situ cross-linking hydrogels of hyaluronic acid and cellulose derivatives

Post-operative peritoneal adhesions can cause pelvic pain, infertility, and potentially lethal bowel obstruction. We have designed and synthesized injectable hydrogels that are formed by mixing hydrazide-modified hyaluronic acid (HA) with aldehyde-modified versions of cellulose derivatives such as carboxymethylcellulose (CMC), hydroxypropylmethylcellulose (HPMC), and methylcellulose (MC). Gelation of these hydrogels occurred in less than 1 min, and had higher shear moduli than that of HA-HA gel (HAX). Hydrogels degraded in the presence of hyaluronidase in vitro, with HA-MC and HA-HPMC degrading more slowly than HAX and HA-CMC. The aldehyde-modified cellulose derivatives showed dose-dependent mild-to-moderate cytotoxicity to mesothelial cells and macrophages in vitro, but all were biocompatible in the murine peritoneum, causing no adhesions for 3 weeks. All the cellulose-derived gels showed efficacy in reducing the area of adhesion formation in a rabbit sidewall defect-bowel abrasion model.     

Clinical efficacy and safety of second line and salvage aflibercept for advanced colorectal cancer in Akita prefecture

BACKGROUNDAngiogenesis inhibitors (AIs) combination with cytotoxic chemotherapy is a promising treatment for patients with colorectal cancer (CRC). Aflibercept (AFL) is an option for second-line treatment of CRC, according to the ‘VELOUR’ trial. Currently, we can choose from three AIs, including bevacizumab, ramucirumab, and AFL. Different AIs can be used in subsequent treatment because of their distinctive mechanisms of action. We addressed the uncertainty regarding AFL efficacy and safety in heavily-treated patients by comparing outcomes of survival treatment with second-line treatment.AIMTo determine and compare the efficacy and safety profiles of AFL in the second-line and salvage therapy settings.METHODSClinical data of 41 patients with advanced CRC who received intravenous AFL combined with the folinic acid-fluorouracil-irinotecan (FOLFIRI) regimen were collected retrospectively from six institutions in Japan, for the period from May 2017 to March 2019. Patient characteristics collected included age, sex, tumor location, RAS and RAF status, metastatic sites, number of previous treatment cycles, therapeutic response, adverse events, duration of previous AI treatment, and survival time. The end points were time to AFL treatment failure (aTTF) and median survival time post-AFL (aMST). Statistical analyses were performed to compare the efficacy and safety in the second-line setting with those of the salvage therapy setting, which was defined as the days since the end of second-line therapy. RESULTSAll 41 patients who received AFL + FOLFIRI for advanced CRC had metastatic or unresectable cancer. Twenty-two patients received AFL in the second-line setting and nineteen in the salvage therapy setting. The patient characteristics were similar in the two groups, except for two factors. The median duration of the previous AI administration was shorter in the second-line patients compared with that in the salvage therapy patients (144 d vs 323 d, P = 0.006). In the second-line and salvage therapy groups, the objective response rates were 11% and 0%, respectively (P = 0.50), and the disease control rates were 53% and 50%, respectively (P = 1.00). In the second-line and salvage therapy groups, the aTTF (123 d vs 71 d, respectively), aMST (673 d vs 396 d, respectively), and incidence of adverse events of grade 3 [8 (36%) vs 9 (47%)] were not significantly different between the two groups.CONCLUSIONAFL can be used to treat advanced CRC patients, with a similar safety and efficacy in the salvage therapy setting as in the second-line setting.