Long-term survival of a pulmonary artery sarcoma produced by aggressive surgical resection and adjuvant chemoradiotherapy.

Malignant pulmonary artery tumors represented by sarcomas are rare, but fatal. Early diagnosis and radical surgical resection offer the only chance for survival. However, surgical intervention has some challenging aspects, and prognosis is poor even after tumor resection. We report a case of a pulmonary artery sarcoma between the right ventricular outflow tract and the pulmonary artery branches. The tumor was aggressively extracted with reconstruction using a cryopreserved pulmonary valved allograft, followed by adjuvant chemoradiotherapy. At 56 months after surgery, the patient is well without any evidence of recurrence, demonstrating that aggressive surgical resection with adjuvant chemoradiotherapy can prolong survival.     

STANDARD IMAGING TECHNIQUES OF ENDOSCOPIC ULTRASOUND‐GUIDED FINE‐NEEDLE ASPIRATION USING A CURVED LINEAR ARRAY ECHOENDOSCOPE

Standard imaging techniques using a curved linear array echoendocope are summarized to facilitate the attainment of expertise in endoscopic ultrasonography and endoscopic ultrasound‐guided fine needle aspiration, and to promote the widespread use of this diagnostic and therapeutic tool. Typical images of the mediastinal organs, the bilio‐pancreatic systems and neighboring organs by scanning from the esophagus, stomach, duodenal bulb, and descending portion of the duodenum, are shown in a sequential manner. The basic techniques of endoscopic ultrasound‐guided fine needle aspiration are also presented.     

Ectopic Ureterocele with a Horseshoe Kidney in an Adult

A rare adult case of a left ectopic ureterocele associated with a duplex horseshoe kidney is reported. To the best of our knowledge, only one pediatric case of horseshoe kidney with an ectopic ureterocele has been reported. The present case was successfully treated by ureteropyelostomy, upper ureterectomy and unroofing of the ureterocele. The patient is currently followed with excretory urograms and renograms.     

Chromosomal Mapping of Genetic Locus Associated with Thymus-size Enlargement in BUF/Mna Rats

The thymoma-prone rat of the BUF/Mna strain is a useful model for human thymoma. In this strain thymoma development is regulated by a single autosomal susceptible gene, Tsr-1. At pre-thymoma age, BUF/Mna rats have extremely large thyrauses, when compared to those of other strains of rats. Genetic studies in crosses between BUF/Mna rats with large thymuses and WKY/NCrj rats with small thymuses suggested the presence of a major autosomal gene, Ten-1 , which contributes to thymus enlargement in a backcross population. Linkage studies between Ten-1 and microsatellite markers in backcross rats of (WKY/NCrj×BUF/Mna)Fl×BUF/Mna have led to the localization of Ten-1 in chromosome 1. This result may provide an approach to clone Tsr-1 , which could be allelic to Ten-1.     

Nerve conduction and microanatomy in the rabbit sciatic nerve after gradual limb lengthening-distraction neurogenesis.

To clarify how the peripheral nerve adapts to elongation during gradual limb lengthening, electrophysiological and histomorphometric examinations were performed on the sciatic nerves in 18 rabbits. External fixators were used to lengthen the right femora by 30 mm (30%), at a daily rate of 0.5 mm (Group 1) or 2.0 mm (Group 2). Examinations were performed immediately after the limb lengthening procedure. Electrophysiologically, mild conduction slowing was observed in Group 1; a conduction block was evident in Group 2. Histologically, the mean diameter of myelinated fibers was unchanged in Group 1, but a significantly decreased diameter was observed in Group 2. Electron microscopy revealed that mild degenerative change of unmyelinated axons occurred sporadically in two cases in Group 2, but neither group showed evidence of thinning of myelin sheath of myelinated fibers. The mean internodal length (between nodes of Ranvier) of teased fibers was 1216+/-295 microm in the control contralateral side, 1484+/-347 microm in Group 1, and 1467+/-322 microm in Group 2. Thus the internodes were lengthened by 22.1% (Group 1) and 20.7% (Group 2) in comparison with those of the controls. Straightening of the geometry of paranodal myelin sheath was significantly correlated with the rate of distraction. These results indicate that myelinated nerve fibers adapt to gradual elongation by lengthening each Schwann cell body, not by proliferation of Schwann cells.     

Gorham massive osteolysis

Gorham syndrome (massive osteolysis) is a very rare tumour-like lesion characterized by progressive osteolysis. The diagnosis must be confirmed by the microscopic finding of intramedullary angioma-like vascular structures. We report a case of a 15-year-old boy with a pathological fracture in his left humerus. Imaging modalities such as magnetic resonance imaging, computed tomography, angiography and bone scintigraphy failed to disclose to tumorous lesion that filled a cavity in the left humerus. After observing the boy's progress for 6 months, a temporary diagnosis of Gorham syndrome was made, and surgical treatment was chosen. After resection of the left humeral head and the proximal one-quarter of the humerus, thorough curettage was performed in the distal humerus and an intramedullary artificial humeral head fixed with adequate success. Pathological examination of the specimen revealed intramedullary haemangioma of the humerus.     

Select types of supporting cell in the inner ear express aquaporin-4 water channel protein

Aquaporins (AQPs) confer a high water permeability on cell membranes and play important parts in secretory and absorptive epithelia in kidney and other organs. Here we investigate whether AQPs are expressed in the sensory epithelia of the inner ear, where a precise volume regulation is crucial. By use of specific antibodies it was found that the inner ear contains AQP1 and 4 while being devoid of detectable levels of AQP2, 3 or 5. Immunofluorescence and postembedding immunogold labelling revealed a strictly non-epithelial distribution of AQP1, confirming previous data. In contrast, AQP4 protein and mRNA (visualized by in situ hybridization) were concentrated in select types of supporting cell, including Hensen's cells and inner sulcus cells. Immunogold particles signalling AQP4 were confined to the basolateral plasma membrane of Hensen's cells and to the basal plasma membrane of Claudius cells and inner sulcus cells. AQP4 was also found in supporting cells of the vestibular end organs, but was absent from transitional epithelial cells and dark cells. Strong labelling for AQP4 and AQP4-mRNA was associated with the central part of the cochlear and vestibular nerves. Hair cells were consistently unlabelled. Our findings indicate that AQP4 may facilitate osmotically driven water fluxes in the sensory epithelia of the inner ear and thus contribute to the volume and ion homeostasis at these sites.     

Successful surgical treatment of secondary kwashiorkor after total gastrectomy: Report of a case

We report herein the case of a 56-year-old woman who developed secondary Kwashiorkor 9 years after undergoing a total gastrectomy for early gastric cancer. Until she began developing the symptoms of Kwashiorkor, including general fatigue, edema of the face and extremities, anemia, alopecia, and weight loss, she had been leading a normal life post-gastrectomy. Her symptoms were alleviated by total parenteral nutrition (TPN) therapy, but reappeared soon after TPN therapy was discontinued. Therefore, she required several subsequent courses of TPN. In an attempt to permanently resolve the ongoing Kwashiorkor symptoms, reconstructive surgery involving transposition of the jejunum from the previous Graham method to the interposition method was performed 10 years after the initial gastrectomy. After the second operation, her malnutrition was completely alleviated, and she has been in good health for the 8 years since. To our knowledge, there has been no other report of the symptoms of secondary Kwashiorkor after total gastrectomy being alleviated by altering the procedure of reconstruction of the intestinal tract. Thus, we recommend surgical treatment to alter the digestive continuity to a more physiological pathway for selected patients with secondary Kwashiorkor syndrome.