An effective treatment by chemoradiation therapy after stent insertion for advanced esophageal cancer with esophago-pulmonary fistula--report of a case

We report the case of a 56-year-old male who was diagnosed as advanced esophageal cancer with esophago-pulmonary fistula and lung abscess. He received radiation therapy in combination with chemotherapy using cisplatin and 5-FU after insertion of a self-expanding metallic stent. He had sufficient food intake during the chemoradiotherapy (CRT). CRT was very effective for not only primary tumor but also lymph node metastasis, with resulting partial response.We could not detect any relapses and metastases for 8 months after CRT. The CRT after insertion of self-expanding metallic stent is one of the useful and palliative treatments for advance esophageal cancer with esophago-pulmonary fistula.     

Fostamatinib for the treatment of Japanese patients with primary immune thrombocytopenia: A phase 3, placebo-controlled,double-blind,parallel-group study

Fostamatinib, a spleen tyrosine kinase inhibitor, has been approved for the treatment of chronic primary immune thrombocytopenia (ITP) in the United States, Canada and some European countries. We conducted a phase 3, placebo-controlled, double-blind, parallel-group study to evaluate the efficacy and safety of fostamatinib in Japanese patients with primary ITP. Thirty-four patients were randomised to fostamatinib (n = 22) or placebo (n = 12) at 100–150 mg twice a day for 24 weeks. Stable responses (platelet ≥50 000/μl at ≥4 of the 6 visits from weeks 14 to 24) were observed in eight (36%) patients on fostamatinib and in none of the patients on placebo (p = 0.030). Overall responses (platelet ≥50 000/μl at ≥1 of the 6 visits from weeks 2 to 12) were seen in 10 (45%) patients on fostamatinib and in none of the patients on placebo (p = 0.006). Patients on fostamatinib required rescue medication less often and experienced fewer bleeding symptoms than patients on placebo. Adverse events observed were mild or moderate and were manageable. No new safety signals were identified in Japanese patients with ITP.     

An autopsy case of sporadic amyotrophic lateral sclerosis associated with the I113T SOD1 mutation

A 64‐year‐old man noticed weakness in his arms and dyspnea upon exertion. Four months later he was admitted to our hospital, where muscle atrophy and hyperactive deep tendon reflexes in the arms were observed upon examination. A needle electromyograph study revealed acute and chronic denervation in the extremities, and he was diagnosed as having amyotrophic lateral sclerosis (ALS). Seven months after onset of the disease, he died of respiratory failure. Neuropathologically, neuronal cell loss was observed in the motor cortex, hypoglossal nuclei, cervical and lumbar anterior horns and Clarke's nuclei. Some of the remaining neurons contained neurofilamentous conglomerate inclusions (CIs). A small number of Lewy body‐like hyaline inclusions (LBHIs) were also observed. No the Bunina bodies, skein‐like inclusions or basophilic inclusions were detectable. Tract degeneration was moderate in the dorsal and ventral spinocerebellar tracts, mild in the pyramidal tract, but not discerned in the posterior column. Immunohistochemical examinations revealed that the CIs were strongly positive for phosphorylated neurofilament and moderately positive for ubiquitin and Cu/Zn superoxide dismutase 1 (SOD1). Moreover, a number of phosphorylated tau protein‐positive globose neurofibrillary tangles (NFTs) and threads were observed in the periaqueductal gray matter, oculomotor nuclei and trochlear nuclei. Although the family history was negative for neuromuscular diseases, the neuropathological findings indicated features of familial ALS with a SOD1 mutation. In fact, DNA analysis of frozen‐brain tissue revealed the presence of the I113T SOD1 mutation. This case represents the first one of this mutation in a patient who showed CIs as well as LBHIs in the motor neurons at the same time, in addition to the NFTs in the mesencephalic tegmentum.     

Efficacy and safety of prophylactic superselective embolization for angiomyolipoma at the renal hilum

ObjectiveThis study investigated the efficacy and safety of superselective transcatheter arterial embolization for angiomyolipoma at the renal hilum.MethodsBetween August 2012 and January 2015, 13 patients with 16 angiomyolipomas at the renal hilum underwent initial, prophylactic, superselective transcatheter arterial embolization. The patients were followed by computed tomography or magnetic resonance imaging, and volume-reduction ratios after embolization were measured.ResultsThe mean or median post-embolization volume reduction ratios were 23% (follow-up duration, 1–2 months), 55% (3–6 months), 55% (7–12 months), 66% (1–2 years), 67% (2–3 years), and 54% (>3 years). After initial embolization, none of the 16 tumors bled or required surgery; two (13%) tumors recurred; and three (19%) tumors received repeat embolization. Estimated glomerular filtration rates were not decreased at medians of 7 days (near the time of discharge) and 39 days (first clinical follow-up) post-procedure, compared with baseline. Except for post-embolization syndrome, no procedure-related complications occurred.ConclusionsSuperselective embolization for renal hilar angiomyolipoma is safe and kidney-preserving, with good tumor volume reduction and bleeding prevention.     

Histopathological validation of magnifying endoscopy for diagnosis of mixed-histological-type early gastric cancer

BACKGROUND The undifferentiated-type(UDT) component profoundly affects the clinical course of early gastric cancers(EGCs). However, an accurate preoperative diagnosis of the histological types is unsatisfactory. To date, few studies have investigated whether the UDT component within mixed-histological-type(MT) EGCs can be recognized preoperatively.AIM To clarify the histopathological characteristics of the endoscopically-resected MT EGCs for investigating whether the UDT component could be recognized preoperatively.METHODS This was a single-center retrospective study. First, we attempted to clarify the histopathological characteristics of the endoscopically-resected MT EGCs with emphasis on the UDT component. Histopathological examination investigated each lesion's UDT component:(1) Whole mucosal layer occupation of the UDT component;(2) UDT component exposure to the surface of the mucosa; and(3) existence of a clear border between the differentiated-type and UDT components.Then, preoperative endoscopic images with magnifying endoscopy with narrowband imaging(ME-NBI) were examined to identify whether the endoscopic UDT component finding was recognizable within the area where it was present in the histopathological examination. The preoperative biopsy results and comparative relationships between endoscopic and histopathological findings were also examined.RESULTS In the histopathological examination, the whole mucosal layer occupation of the UDT component and exposure of the UDT component to the mucosal surface were observed in 67.3%(33/49) and 79.6%(39/49) of samples, respectively. A clear distinction of the border between the differentiated-type and UDT components could not be drawn in 65.3%(32/49) of MT lesions. In the endoscopic examination, the preoperative endoscopic images showed that only 24.5%(12/49) of MT EGCs revealed the UDT component within the area where it was present histopathologically. Histopathological UDT predominance was the single significant factor associated with the presence of the endoscopic UDT component finding(61.5% vs 11.1%, P = 0.0009). Only 26.5%(13/49) of the lesions were diagnosed from the pretreatment biopsy as having a UDT component. Combined results of the pretreatment biopsy and ME-NBI showed the preoperative presence of the UDT component in 40.8%(20/49) of MT EGCs.CONCLUSION Recognition of a UDT component within MT EGCs is difficult even when pretreatment biopsy and ME-NBI are combined. Endoscopic resection plays a significant role in both treatment and diagnosis.