Long-term survival after bilateral adrenalectomy for metachronous adrenocortical cancer

We report the case of a female patient with bilateral metachronous adrenocortical cancer who survived long-term after adrenalectomy. In 1991, the patient underwent left adrenalectomy to remove a huge adrenal mass (10 x 9 cm) displaying no hormonal abnormality. Histological diagnosis was adrenocortical cancer. A right adrenal mass (7 x 6 cm) was found 4 years after left adrenalectomy. Right adrenalectomy was performed, and histological diagnosis was again adrenocortical cancer. The patient remains alive with no evidence of disease 8 years after last surgery.     

Characteristics of juvenile dermatomyositis in Japan

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM , Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.     

Theophylline metabolism in acute asthma with MxA-indicated viral infection

BACKGROUND: Although viral infection might alter theophylline metabolism in acute asthma, there are some difficulties in detecting infection due to various kinds of viruses in a clinical setting. METHODS: To evaluate the usefulness of assessment of MxA protein in acute asthma exacerbated by viral infection, MxA protein expression in lymphocytes was assayed by flow cytometric analysis in whole peripheral blood in 21 children (aged 0-6 years) receiving continuous theophylline infusion for management of asthma attack. Serum theophylline levels were measured at 24 and 72 h after initiating theophylline infusion. RESULTS: At the beginning of theophylline infusion, 11 children had increased expression of MxA protein, indicating viral infected states. After 24 h continuous infusion, there were no differences in theophylline levels between MxA-negative and MxA-positive groups. After 72 h infusion, the mean theophylline level of MxA-positive children was significantly higher than that of MxA-negative children (9.7 +/- 2.2 microg/mL vs 7.3 +/- 1.6 microg/mL). The ratio of theophylline clearance at 72 h to that at 24 h in the MxA-positive group was significantly lower than that of the MxA-negative group (1.1 +/- 0.2 vs 1.4 +/- 0.1). CONCLUSIONS: Viral infection appeared to affect theophylline metabolism. Flow cytometric assay of lymphoid MxA protein expression in whole blood is an easy and useful method of evaluating viral infection in acute asthma exacerbation.     

Reconstruction of Obliterated Vesicourethral Junction: Use of the Bulbar Urethra as a Continent Valve

Reconstruction of the obliterated vesicourethral junction is both complex and difficult. Here, we report an innovative method using a mobilized bulbar urethra as a continent valve. Three patients with major problems at the vesicourethral junction underwent continent valve reconstruction. In cases 1 and 2, in which there were problems at the anastomosing site after radical prostatectomy, the bladder wall was closed, wedge resection of the midline pubic bone was performed, and a fully mobilized bulbar urethra was implanted submucosally into the anterior bladder wall. In case 2, augmentation cystoplasty using an ileal segment was required due to the small capacity of the bladder. In case 3, in which there was posterior urethra disruption associated with pelvic fracture, the bulbar urethra was implanted into the bladder wall in the same manner as in cases 1 and 2 without pubectomy. The postoperative follow‐up periods were 48, 36, and 12 months, respectively. In all patients, urinary management was achieved by self‐catheterization postoperatively, and the patients were satisfied with their status. This newly devised continent valve construction using a bulbar urethra is effective for reconstruction of the obliterated vesicourethral junction, which markedly improves patients' quality of life.     

A case of granular cell tumor of the bladder successfully managed with extraperitoneal laparoscopic surgery

Granular cell tumor is a benign neoplasm which frequently occurs in the oral cavity, skin, and subcutaneous tissue. Granular cell tumor of the bladder is an extremely rare disease, and only nine cases have been reported. We present here an additional case of granular cell tumor occurring in the bladder. Unlike the other tumors reported, this tumor extruded into the Retzius' cavity. Therefore, the tumor was successfully excised through extraperitoneal laparoscopic surgery. The patient was free from recurrence 40 months after surgery. The small tumor located in Retzius' cavity could be managed with extraperitoneal laparoscopic surgery.     

Utility of immunohistochemical detection of prostate-specific Ets for the diagnosis of benign and malignant prostatic epithelial lesions

BACKGROUND: Human prostate-specific Ets (hPSE) belongs to the Ets family. It regulates the proliferation, differentiation, and development of prostate epithelial cells. A recent study showed that hPSE can be detected in normal glands but not in cell lines established from prostate cancer (PCA), suggesting a translational disorder of hPSE from mRNA to protein in PCA. Immunohistochemical detection of hPSE could therefore be another method of differential diagnosis of PCA from other proliferative conditions in the prostate. METHODS: An immunohistochemical detection of hPSE was carried out on the whole mounted prostatectomy specimen obtained from 19 cases with PCA. RESULTS: Basal and secretory luminar cells showed a diffuse cytoplasmic staining for hPSE in normal glands, hyperplastic glands, and prostate intraepithelial neoplasia lesions. Whereas approximately 30% of PCA lesions showed a negative staining for hPSE, the positive rate for hPSE between PCA and benign glands or prostate intraepithelial neoplasia (PIN), was statistically significant (P < 0.05). Staining intensities in normal glands, hyperplastic glands, and PIN lesions were similar, but generally stronger than those in PCA lesions. CONCLUSIONS: Negative immunoreactivity for hPSE strongly suggests malignancy in the prostate glands. Decreased immunoreactivities of glands for hPSE could suggest PCA.     

Early Gastric Lymphoma Coexisting with Reactive Lymphoreticular Hyperplasia (RLH) -A Case Report-

A 34-year-old female visited our hospital because of epigastralgia. We performed an upper gastrointestinal x-ray examination, and both conventional endoscopy and dye-spraying endoscopy (indigo-carmine contrast method). We diagnosed early gastric lymphoma which simulated Borrmann 3 type gastric cancer with IIb type early gastric cancer on the middle body and reactive lymphoreticular hyperplasia (RLH) of cobble stone-like granular pattern by endoscopic appearance on the lower body. Although dye-spraying endoscopy showed the details of the mucosa, it was very difficult to diagnose the lesions correctly by gross appearance alone. Because gastric lymphoma arises from the mucosal or submucosal layer and spreads in the mucosa diffusely, ultrasonic visualization by echo-endoscopy might be useful in the diagnostic procedure. We report a case of early gastric lymphoma coexisting with RLH; both lesions showed uncommon endoscopic features.