obJect. The purpose of this study was to evaluate the role of stereotactic radiosurgery (SRS) in the management of intracranial hemangioblastomas. methods. Six participating centers of the North American Gamma Knife Consortium and 13 Japanese Gamma Knife centers identified 186 patients with 517 hemangioblastomas who underwent SRS. Eighty patients had 335 hemangioblastomas associated with von Hippel-Lindau disease (VHL) and 106 patients had 182 sporadic hemangioblastomas. The median target volume was 0.2 cm 3 (median diameter 7 mm) in patients with VHL and 0.7 cm 3 (median diameter 11 mm) in those with sporadic hemangioblastoma. The median margin dose was 18 Gy in VHL patients and 15 Gy in those with sporadic hemangioblastomas. results. At a median of 5 years (range 0.5-18 years) after treatment, 20 patients had died of intracranial disease progression and 9 patients had died of other causes. The overall survival after SRS was 94% at 3 years, 90% at 5 years, and 74% at 10 years. Factors associated with longer survival included younger age, absence of neurological symptoms, fewer tumors, and higher Karnofsky Performance Status. Thirty-three (41%) of the 80 patients with VHL developed new tumors and 17 (16%) of the106 patients with sporadic hemangioblastoma had recurrences of residual tumor from the original tumor. The 5-year rate of developing a new tumor was 43% for VHL patients, and the 5-year rate of developing a recurrence of residual tumor from the original tumor was 24% for sporadic hemangioblastoma patients. Factors associated with a reduced risk of developing a new tumor or recurrences of residual tumor from the original tumor included younger age, fewer tumors, and sporadic rather than VHL-associated hemangioblastomas. The local tumor control rate for treated tumors was 92% at 3 years, 89% at 5 years, and 79% at 10 years. Factors associated with an improved local tumor control rate included VHL-associated hemangioblastoma, solid tumor, smaller tumor volume, and higher margin dose. Thirteen patients (7%) developed adverse radiation effects (ARE) after SRS, and one of these patients died due to ARE.

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ObjectiveTo identify the key diagnostic features and causes of fever of unknown origin (FUO) in Japanese patients.DesignMulticentre prospective study.SettingSixteen hospitals affiliated with the Japanese Society of Hospital General Medicine, covering the East and West regions of Japan.ParticipantsPatient aged ≥20 years diagnosed with classic FUO (axillary temperature≥38.0°C at least twice within a 3-week period, cause unknown after three outpatient visits or 3 days of hospitalisation). A total of 141 cases met the criteria and were recruited from January 2016 to December 2017.InterventionJapanese standard diagnostic examinations.Outcome measuresData collected include usual biochemical blood tests, inflammatory markers (erythrocyte sedimentation rate (ESR), C reactive (CRP) protein level, procalcitonin level), imaging results, autopsy findings (if performed) and final diagnosis.ResultsThe most frequent age group was 65–79 years old (mean: 58.6±9.1 years). The most frequent cause of FUO was non-infectious inflammatory disease. After a 6-month follow-up period, 21.3% of cases remained undiagnosed. The types of diseases causing FUO were significantly correlated with age and prognosis. Between patients with and without a final diagnosis, there was no difference in CRP level between patients with and without a final diagnosis (p=0.121). A significant difference in diagnosis of a causative disease was found between patients who did or did not receive an ESR test (p=0.041). Of the 35 patients with an abnormal ESR value, 28 (80%) had causative disease identified.ConclusionsAge may be a key factor in the differential diagnosis of FUO; the ESR test may be of value in the FUO evaluation process. These results may provide clinicians with insight into the management of FUO to allow adequate treatment according to the cause of the disease.

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Aortoesophageal fistula is a critical and life-threatening disease. The cardiovascular strategy for graft replacement has been widely discussed. However, the surgical strategy of esophageal resection and reconstruction for aortoesophageal fistula has rarely been discussed. The objective of this study is to establish a surgical strategy and procedure of esophageal resection and reconstruction for aortoesophageal fistula. Eleven patients with aortoesophageal fistula who underwent aortic graft replacement and esophagectomy between 2008 and 2015 at Kobe University Hospital were enrolled in this study. Patient characteristics, operative methods, and clinical outcomes were obtained by retrospective chart review. All 11 patients underwent graft replacement, esophagectomy, and omental wrapping. Ten esophagectomies were simultaneously accomplished in the same operative field as aortic graft replacement. Seven patients underwent subtotal esophagectomy from a left thoracotomy, and three patients underwent upper hemiesophagectomy from a median sternotomy. The other patient underwent staged esophagectomy from a right thoracotomy. Seven of 11 patients (63.6%) successfully underwent staged esophageal reconstruction. Pedicled jejunal transfer with supercharge and superdrainage were performed in six patients, and ileocecal reconstruction was performed in one patient. Median survival time in the patients with esophageal reconstruction was 21 months while that in the patients without esophageal reconstruction was 10 months. Six of 7 patients (85.7%) who underwent esophageal reconstructions were alive. Our surgical strategy for aortoesophageal fistula, which includes simultaneous graft replacement and esophagectomy in the same operative field and staged reconstruction by pedicled jejunal transfer to ensure omental wrapping, is feasible and promising.