Pediatric inflammatory myofibroblastic tumor of the bladder with ALK-FN1 fusion successfully treated by alectinib.

An inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm characterized by the proliferation of myofibroblasts and inflammatory cell infiltration. Although radical resection is the only established treatment strategy for IMT, it can cause functional disorders when vital organs are affected. We describe a case of pediatric IMT of the bladder with FN1-ALK (fibronectin 1-anaplastic lymphoma kinase) fusion. Radical resection might lead to urinary disturbance due to the large tumor size at diagnosis. However, the tumor was successfully treated with alectinib, a second-generation ALK inhibitor, followed by transurethral resection of the bladder tumor without any complications.

Presence of identical B-cell clone in both cerebrospinal fluid and tumor tissue in a patient with opsoclonus-myoclonus syndrome associated with neuroblastoma.

Opsoclonus-myoclonus syndrome associated with neuroblastoma (OMS-NB) is a refractory paraneoplastic syndrome which often remain neurological sequelae, and detailed pathogenesis has remained elusive. We encountered a pediatric patient with OMS-NB treated by immunosuppressed therapy who showed anti-glutamate receptor δ2 antibody and increased B-cells in cerebrospinal fluid (CSF), and multiple lymphoid follicles containing abundant Bcells in tumor tissue. Unbiased B-cell receptor repertoire analysis revealed identical B-cell clone was identified as the dominant clone in both CSF and tumor tissue. These identical B-cell clone may contribute to the pathogenesis of OMS-NB. Our results could facilitate the establishment of pathogenesis-based treatment strategies for OMS-NB.

Citrullinated Histone H3: Early Biomarker of Neutrophil Extracellular Traps in Septic Liver Damage.

BACKGROUND: Neutrophil extracellular traps (NETs) play a crucial role in host defense, but excess and prolonged interaction of NETs with platelets can cause severe inflammation and host organ damage. Modification of histone H3 by citrullination is involved in in vitro NET formation. The phosphodiesterase III inhibitor, cilostazol (Ciz), which has a protective effect on liver sinusoidal endothelial cells and inhibits platelet aggregation, may prevent organ damage caused by excess NETosis. In this study, we investigated whether citrullinated histone H3 (H3Cit) could serve as a biomarker for the detection of critical liver damage in sepsis and the efficacy of phosphodiesterase-III inhibition for preventing the liver dysfunction induced by NETosis. MATERIALS AND METHODS: Mice injected with lipopolysaccharide (LPS; 1 mg/kg) were used as a sepsis model with or without treatment with Ciz (200 mg/kg). H3Cit, myeloperoxidase, and neutrophil elastase levels were measured by immunohistochemistry. We evaluated H3Cit-positive neutrophils in the peripheral blood by flow cytometry. RESULTS: Immunohistochemistry revealed that H3Cit-, neutrophil elastase-, and myeloperoxidase-positive cell numbers in the livers peaked at 12 h after LPS administration. However, flow cytometry showed a significant increase in H3Cit-positive neutrophils in the peripheral blood only 4 h after LPS injection. Treatment with Ciz significantly ameliorated all parameters. CONCLUSIONS: H3Cit is a useful biomarker for early detection of NETosis or liver dysfunction, and Ciz may be an effective treatment for septic liver damage.

Risk factors for the recurrence of perineal canal.

PURPOSE: The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC). METHODS: Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated. Regarding the treatment procedure for fistula, either closure of the rectal wall with stitches or ligation of fistula in the rectum was performed. These factors were compared between the two groups. RESULTS: Six of 17 patients with PC who underwent surgical treatment had recurrence. There were no significant differences in the incidence of preoperative infection, age at operation or presence of colostomy (p = 0.60, 0.38, 1.00, respectively). In the recurrence group, all patients were treated by closure of the rectal wall. In the non-recurrence group, five were treated by the closure of the rectal wall with stitches and six by ligation of the fistula. There was a significant association between recurrence and the treatment procedure for fistula (p = 0.04). CONCLUSION: Closure of the rectal wall with stitches is a risk factor for the recurrence of PC.

Repair of type IV laryngotracheoesophageal cleft (LTEC) on ECMO.

PURPOSE: A type IV laryngotracheoesophageal cleft (LTEC) is a very rare congenital malformation. Type IV LTEC that extends to the carina have poor prognosis and are difficult to manage. We present our experience with surgical repair in such a case using extracorporeal membranous oxygenation (ECMO). METHODS: A male infant, who was diagnosed with Goldenhar syndrome, showed severe dyspnea and dysphagia. Laryngoscopy indicated the presence of LTEC. The patient was transferred to our institute for radical operation 26 days after birth. Prior to surgery, a balloon catheter was inserted in the cardiac region of stomach through the lower esophagus to block air leakage, to maintain positive pressure ventilation. We also performed observations with a rigid bronchoscope to assess extent of the cleft, and diagnosed the patient with type IV LTEC. After bronchoscopy, we could intubate the tracheal tube just above the carina. Under ECMO, repair of the cleft was performed by an anterior approach via median sternotomy. RESULTS: The patient was intubated via nasotracheal tube and paralysis was maintained for 2 weeks, using a muscle relaxant for the first 3 days. Two weeks after surgery, rigid bronchoscopy showed that the repair had been completed, and the tracheal tube was successfully extubated without tracheotomy. CONCLUSIONS: Although insertion of a balloon catheter is a very simple method, it can separate the respiratory and digestive tracts. This method allowed for positive pressure ventilation and prevented displacement of the endotracheal tube until ECMO was established. As a result, we safely performed the operation and the post-operative course was excellent.