Aortic aneurysm in a four-year-old child with tuberous sclerosis

We present a case of aortic aneurysm in a four-year-old child complicated with tuberous sclerosis. We used the same general principles as for adult patients and successfully managed our patient. Our methods included the use of isoflurane plus epidural anaesthesia, dopamine to maintain blood pressure, and induced mild hypothermia to reduce brain metabolism and to prevent spinal cord damage during aortic cross-clamping. Intensive monitoring including EEG was beneficial to the anaesthetic management.     

Renal Cell Carcinoma in a Solitary Kidney Treated by Partial Nephrectomy --Case Report and Review of Japanese Literature

Renal cell carcinoma in a solitary kidney is rather rare. Wepresent a case of this condition occurring in a 50-year-oldman. He was treated by partial nephrectomy and temporary hemodialysisfor postoperative acute renal failure. Perusal of the literaturereveals that in situ partial nephrectomy, if possible, seemsto be a reasonable treatment of choice for renal cell carcinomain a solitary kidney. This is the fifth case ever reported inJapan.     

Clinical features of testicular tumors in children

AIM: Testicular tumors are not common pediatric solid tumors, especially in Asian children. There have been few reviews of cases in Japan to date. We present the clinical features of 14 pediatric testicular tumor patients. METHODS: Clinical features of 14 testicular tumor patients, such as chief complaints, age at diagnosis, pathology, stages, treatments and prognosis, were examined from medical records. Two patients had their semen tested at adolescence. RESULTS: Of the 14 prepubescent patients, 12 (85.7%) patients were diagnosed before 3 years of age. Ten cases (71.4%) were diagnosed as yolk sac tumors, three (21.4%) as mature teratomas and one case as an epidermoid cyst. Nine cases (90.0%) among the 10 cases of yolk sac tumor were diagnosed as stage I and one case was stage IV. One stage I yolk sac tumor patient developed lung metastasis later. Eventually, two yolk sac tumor patients died, despite chemotherapy. While all the cases with a diagnosis before 2 years of age survived, 67% (2/3) of cases with a diagnosis after the age of 2 died of tumors. Semen analysis in two patients showed normospermia. CONCLUSION: In the present study, the most common testicular tumors were yolk sac tumors and the patients diagnosed before 2 years of age showed favorable results. Age could be a relapse risk factor in yolk sac tumors. Guidelines for handling testicular tumors in children is not yet well established in Japan. An organized system seems necessary to gather and accumulate the results of the cases in Japan in order to develop better guidelines for treatment.     

Efficacy and morbidity of transrectal ultrasound-guided 12-core biopsy for detection of prostate cancer in Japanese men

BACKGROUND: The objectives of the present study were to determine whether an extensive biopsy scheme contributes to enhanced detection of prostate cancer in Japanese men and to assess the associated pain and morbidity. METHODS: A total of 147 patients were included in this analysis, with 12 biopsy cores being obtained from each patient. Standard systematic sextant biopsy at the apex, mid-prostate and base of the prostate gland was carried out under local anesthesia and this was followed by the acquisition of additional sextant cores at the same levels from the far lateral peripheral zone. Each patient answered a self-administered questionnaire on pain and morbidity during the 5 days following biopsy. RESULTS: Overall, 39 patients (26.5%) received a diagnosis of prostate cancer. Nine patients (23.1%) were positive only at the standard sextant sites, three patients (7.7%) were positive exclusively at the far lateral sites and the remaining 27 patients (69.2%) were positive at both sites. Cancer was found most frequently in cores obtained from the apex (P = 0.009), with this trend being more evident in patients with abnormal rectal findings, positive sonographic findings, gland volume < 40 cm(3) and prostate-specific antigen density > 0.15 ng/mL/cm(3) (P < 0.03). These findings were also true for those with a prostate-specific antigen range from 4.1 to 20.0 ng/mL. A gradual decrease in incidence and grade of pain, hematuria and rectal bleeding was observed during the first 5 days after biopsy (P < 0.0001). CONCLUSIONS: Using this 12-core biopsy scheme, we found cancer most frequently in cores taken at the level of the apex. While the extensive procedure only marginally enhanced overall detection of prostate cancer, it was well tolerated with gradually decreasing pain and morbidity over a brief postbiopsy period. Further efforts to optimize biopsy schemes are warranted.     

Multifocal metastases of recurrent renal cell carcinoma successfully treated with a combination of low dose interleukin-2, α-interferon and radiotherapy

A 59-year-old man presented with a 2-month history of left flank pain and a possibility of gross hematuria. Left renal cell carcinoma stage II was diagnosed and radical left nephrectomy was performed. Twenty-two months postoperatively, lung metastases were demonstrated and 6 x 10(6) units of alpha-interferon (IFN-alpha) were administered for 9 months, only to keep the sizes of the metastases unchanged. Thirty-four months after the operation, liver metastases and bone metastasis in the left sacroiliac joint were revealed. The combination cytokine therapy was performed with 1.4 x 10(6) U of interleukin-2 (IL-2) and 3 x 10(6) U of IFN-alpha for 16 weeks, and the left sacroiliac joint metastasis was treated with radiation therapy of 4 Gy per day for 7 days. Six months after the 16 weeks of immunotherapy, computed tomography and bone scintigraphy revealed that the metastases of the lung, liver and bone substantially disappeared and this complete response is still kept after 16 months.     

Successful management of inferior vena cava thrombus complicating advanced germ cell testicular tumor with temporary inferior vena cava filter

We report a case of right testicular tumor with inferior vena cava (IVC) thrombus. Due to the risk of pulmonary embolization, a temporary IVC filter had been inserted during chemotherapy. There were no complications with the temporary IVC filter during the implantation period. The patient was safely treated with systemic chemotherapy using a temporary IVC filter followed by retroperitoneal lymph node and vena cava dissection.     

Transrectal ultrasound-guided transperineal 14-core systematic biopsy detects apico-anterior cancer foci of T1c prostate cancer

AIM: The optimal biopsy strategy for prostate cancer detection, especially in men with isolated prostate-specific antigen (PSA) elevation, remains to be defined. We evaluated diagnostic yield and safety of transrectal ultrasound (TRUS)-guided transperineal systematic 14-core biopsy and compared the spatial distribution of cancer foci detected with this technique in men with and without abnormality on digital rectal examination (DRE). METHODS: In a prospective study, 289 men aged between 50 and 87 years (median age, 70 years) underwent TRUS-guided transperineal systematic 14-core prostate biopsy because of elevated PSA and/or abnormal DRE findings. Using the fan technique, 12 cores from the peripheral zone and two cores from the transition zone were obtained systematically. To characterize the spatial distribution of cancer positive cores, site-specific overall and unique cancer detection rates were compared between stage T1c and T2 cancers. RESULTS: Prostate cancer was detected in 105 of the 289 patients (36%). Major complications requiring prolonged hospital stay or re-hospitalization during a 4-week postbiopsy period were rare (1.4%). Sixty-seven stage T1c cancers were identified. These cancers were associated with significantly lower PSA and a smaller number of cancer positive cores when compared with stage T2 cancers (n= 38). The overall cancer detection rate was highest at the anterior peripheral zone and the posterior peripheral zone in stage T1c and stage T2 cancers, respectively. The unique cancer detection rate at the anterior peripheral zone was significantly higher in stage T1c cancers than in stage T2 cancers. Therefore, when the prostate is extensively biopsied using the transperineal approach, cancer positive cores are characteristically distributed anteriorly in stage T1c cancers and posteriorly in stage T2 cancers. CONCLUSIONS: TRUS-guided transperineal systematic 14-core biopsy showed an apico-anterior distribution of cancer foci in stage T1c prostate cancers.     

Heart Rate Turbulence as a Predictor of Cardiac Mortality and Arrhythmic Events in Patients with Dilated Cardiomyopathy: A Prospective Study

Background: Few studies have described the clinical usefulness of heart rate turbulence (HRT), an autonomic predictor of mortality, in stratifying patients with dilated cardiomyopathy (DCM) at risk of cardiac mortality and arrhythmic events. We prospectively assessed the utility of HRT for risk stratification in patients with ischemic or nonischemic DCM.
Methods: We enrolled 375 consecutive patients with DCM including ischemic (n = 241) and nonischemic causes (n = 134). HRT was measured using an algorithm based on routine 24-hour Holter electrocardiograms, assessing 2 parameters: turbulence onset (TO) and turbulence slope (TS). HRT was considered positive when both TO was ≥0% and TS was ≤ 2.5 ms/R-R interval. The primary endpoint was defined as cardiac mortality and the secondary endpoint as occurrence of hemodynamically stable sustained ventricular tachyarrhythmias.
Results: Of patients enrolled, 83 patients (22.1%) were not utilized for HRT assessment because there were too few ventricular premature beats, or for other reasons. Eighty-one of 292 patients (27.7%) were HRT-positive. During follow-up of 445 ± 216 days, 30 patients (10.3%) reached the primary endpoint and 17 patients, the secondary endpoint. The hazard ratio (HR) of patients with an HRT-positive outcome was 6.4 (95%CI, 3.0–14.1; P < 0.0001) for the primary endpoint and 5.1 (95%CI, 2.8–9.3; P < 0.0001) for combined endpoints. On subanalysis, HRT positivity was significantly associated in both the ischemic and nonischemic DCM patients with both the primary endpoint (HR = 4.9, P = 0.0006 and HR = 12.3, P = 0.002, respectively) and with combined endpoints.
Conclusions: HRT is a powerful risk stratification marker for cardiac mortality and arrhythmic events in patients with DCM whether ischemia is present or not.     

Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia

Aim To investigate the association between magnetic resonance imaging (MRI) patterns and motor function, epileptic episodes, and IQ or developmental quotient in patients born at term with spastic diplegia. Method Eighty‐six patients born at term with cerebral palsy (CP) and spastic diplegia (54 males, 32 females; median age 20y, range 7–42y) among 829 patients with CP underwent brain MRI between 1990 and 2008. The MRI and clinical findings were analysed retrospectively. Intellectual disability was classified according to the Enjoji developmental test or the Wechsler Intelligence Scale for Children (3rd edition). Results The median ages at diagnosis of CP, assignment of Gross Motor Function Classification System (GMFCS) level, cognitive assessment, and MRI were 2 years (range 5mo–8y), 6 years (2y 8mo–19y), 6 years (1y 4mo–19y), and 7 years (10mo–30y) respectively. MRI included normal findings (41.9%), periventricular leukomalacia, hypomyelination, and porencephaly/periventricular venous infarction. The frequency of patients in GMFCS levels III to V and intellectual disability did not differ between those with normal and abnormal MRI findings. Patients with normal MRI findings had significantly fewer epileptic episodes than those with abnormal ones (p=0.001). Interpretation Varied MRI findings, as well as the presence of severe motor dysfunction and intellectual disability (despite normal MRI), suggest that patients born at term with spastic diplegia had heterogeneous and unidentified pathophysiology.