A rare cause of distal bile duct stenosis.

INTRODUCTION: In a patient with a distal bile duct stenosis a definite diagnosis could not be made preoperatively. DISCUSSION: The histologic evaluation of the surgical resection specimen revealed infiltration of the pancreatic head and the distal bile duct by breast tumor cells. CONCLUSION: The metastasis was the only tumor manifestation after mastectomy 12 years ago.

Laparoscopic radical prostatectomy with the Heilbronn technique: an analysis of the first 180 cases.

PURPOSE: In 1998 Guillonneau and Vallancien introduced laparoscopic radical prostatectomy with primary access to the seminal vesicle. In 1999 we developed a different laparoscopic technique similar to the classic retropubic radical prostatectomy. We focus on early results and the learning curve of the procedure in the first 180 patients. MATERIALS AND METHODS: A transperitoneal approach is used with immediate access to Retzius' space. After the dorsal vein complex is endoscopically sutured, the urethra is incised and distal pedicles of the prostate with or without the neurovascular bundle transected. The apex is then pulled ventrally followed with incision at the bladder neck, and transvesical access to vas deferens and seminal vesicle. After completing the posterior wall of the urethrovesical anastomosis with 5 interrupted endoscopic sutures, the Foley catheter is placed, bladder neck closed and specimen extracted via the umbilical incision. From March 1999 to December 2000 we have performed 180 procedures, including 3 for stage pT1 tumor, 88 pT2, 82 pT3 and 7 pT4. Mean preoperative PSA was 13.3 ng./ml. (range 1.4 to 148), mean specimen weight 37.4 gm. (10 to 125) and median Gleason score 6 (3 to 9). For evaluation of the learning curve a separate analysis of 3 groups with 60 patients in each was done. Differences between groups 1 (first 60 patients) and 3 (last 60) were analyzed for statistically significant differences. RESULTS: Mean operating time was 271 minutes (range 150 to 500) and transfusion rate 31%. The reintervention rate was 4.4% and complication rate 18.8%. Of the patients 92% did not require any analgesia on postoperative day 2. Positive margins were found in 16% of the patients. The rate of positive margins in pT2 tumors was 2.3%, pT3a 15% and pT3b 34%. After a median followup of 12 months (range 3 to 23) in 9 (5%) patients a prostate specific antigen relapse was observed. The anastomosis was tight after removal of the catheter in 83% of patients, with a median time of 7 days (range 5 to 30). An anastomotic stricture had to be treated with laser incision in 3.3% of patients. On discharge from the hospital 33% of patients were continent, after 6 months 74% and after 12 months 97%. Analysis of the learning curve revealed significant differences in operating time (324 versus 265 minutes), conversion rate to open surgery (8.1% versus 1.7%), complication rate (23.3% versus 11.7%) and rate of prolonged catheterization (31.6% versus 10%, respectively), whereas the percentage of positive margins and continence rates showed no influence. CONCLUSIONS: Laparoscopic radical prostatectomy requires significant laparoscopic expertise with an ongoing learning curve. Morbidity is low, oncological control similar to results of open surgery and functional results are promising. The procedure should be performed only at dedicated centers with adequate training and expertise.

Proteinase-3 mRNA expressed by glomerular epithelial cells correlates with crescent formation in Wegener's granulomatosis.

BACKGROUND: Wegener's granulomatosis (WG) is characterized by systemic vasculitis with crescentic glomerulonephritis (CGN) and circulating autoantibodies directed against neutrophil cytoplasmic antigens (ANCA). Proteinase 3 (PR-3), a neutral serine proteinase in neutrophils implicated in the growth control of myeloid cells, has been identified as the target antigen for ANCA in WG. Since the kidneys are frequently involved in WG, we studied the in situ expression of PR-3 by renal parenchymal cells. METHODS: We assessed the expression of PR-3 in kidney biopsies of 15 patients with WG by immunohistochemistry (IHC) and in situ hybridization (ISH). Normal kidney tissue served as the control. RESULTS: We detected PR-3 mRNA and PR-3 protein in distal tubular epithelial cells (TECs) and glomerular epithelial cells (GECs) in normal kidney tissue and in CGN. Furthermore, a strong glomerular PR-3mRNA expression restricted to the site of cellular crescents was detected in patients with WG. The analysis of 144 glomeruli with cellular or sclerotic crescents revealed a positive correlation of glomerular PR-3mRNA expression with the percentage of cellular crescents per glomerulus. The capability of human TECs and GECs to synthesize PR-3 was confirmed by Northern blot and ISH on cultured cells. CONCLUSION: These data provide evidence that nonhematopoetic renal parenchymal cells express PR-3 and that glomerular expression of PR-3 is associated with crescent formation in WG. Our findings suggest that renal parenchymal cells may directly be involved in the pathogenesis of CGN in WG.

Thrombotic microangiopathy with renal failure in two patients undergoing gemcitabine chemotherapy.

Described here are 2 patients who developed thrombotic microangiopathy of the kidneys after receiving high cumulative doses of the new anticancer drug gemcitabine. The first patient, who received gemcitabine for treatment of a carcinoma of the pancreas, required hemodialysis for 6 months. In the second case, a woman suffering from a cholangiocellular carcinoma, end-stage renal disease was irreversible. Clinical awareness, timely detection and discontinuation of gemcitabine are mandatory to prevent this rare but disastrous complication of gemcitabine therapy.

Acute renal failure complicating non-fulminant hepatitis A.

Hepatitis, A is usually a mild and self-limiting infection of the liver. Whereas the clinical course is usually benign in children, complications such as prolonged cholestasis and fulminant liver failure have been reported in adults. Acute functional renal failure is an uncommon event in the absence of fulminating liver disease. So far, only cases of acute hepatitis A with biopsy-proven interstitial renal disease or tubular necrosis have been reported [Geltner et al. 1992. Kramer et al. 1986]. We present the case of a 35-year-old, previously healthy male with non-fulminant cholestatic viral hepatitis A, who developed progressive oliguric renal failure requiring dialysis therapy. Kidney biopsy ruled out glomerular disease and tubular necrosis. In the absence of bleeding and other causes of fluid depletion this case may be another variant of hepatorenal syndrome whose etiopathogenesis is only poorly understood.

Localized amyloid in the menisci of the knee joint.

This is, to the best of our knowledge, the first report on amyloid deposits in menisci. Fragments of menisci gained by arthroscopy from 316 patients between 20 and 80 years of age were examined. Amyloid was found in 70% of the cases from male, as well as female patients. The amyloid amount found was always very small, but the deposits seemed to increase with age. Patients more than 50 years of age all had menisceal amyloid. Two types of deposits were observed: a)stroma-deposits in the deep central portions of the menisci (tiny dots of intensely stained amyloid and/or ill defined patches of low staining intensity) and b) surface associated deposits: band-like amyloid imbibition of the collagenous stroma immediately beneath the surface of the menisci but not deeper than 0.2 mm. In all cases, amyloid was resistant when pretreated by KMn04 and immunohistologically antisera against amyloid types AA, AB and AF were negative. 3/25 cases showed a reaction with an amyloid-lambda-antibody. We assume, that amyloid in menisci is a further type of localized senile amyloidosis.

Recurrent hematuria: a novel clinical presentation of hereditary complete complement C4 deficiency.

A 10-year-old boy suffered from recurrent attacks of fever, vomiting, and hematuria. During disease flares, circulating immune complexes were detected in the serum. Elevated levels of Bb, Ba, and C3a indicated complement activation through the alternative pathway. Complement C4 was undetectable. C4 phenotyping by agarose gel electrophoresis showed complete C4 deficiency. Restriction fragment length polymorphism (RFLP) studies showed a homozygous deletion of the C4B and 21-hydroxylase A genes. A mild mesangioproliferative glomerulonephritis with mesangial deposits of immunoglobulin (1g) G, IgM, IgA, Clq, C3, properdin, and terminal complement complex was probably caused by immune complex deposition and alternative complement pathway activation. Treatment with low-dose prednisolone substantially reduced the frequency of further episodes.

[Cystic degeneration of the adventitia of the popliteal artery as a possible sequela of entrapment syndrome]. / Die cystische Adventitia-Degeneration der Arteria poplitea als mögliche Folge eines Entrapment-Syndroms.

We report two cases of cystic adventitial degeneration of the popliteal artery in young sportsmen. The pathogenetic role of microtrauma is suggested by the localisation of the adventitial cysts, the clinical symptoms, as well as the immunohistochemical demonstration of foamy macrophages instead of endothelial cells in the wall of the cysts. In one case the cause of the intermittent claudication could be verified by duplex sonography and comparison of the arterial pressure of both legs with a Doppler method only after physical exercise. As expected, an angiodilatation has only a temporary effect. Therefore a surgical resection of the cyst or of the involved arterial segment had be performed.

Malignant rhabdoid tumor in the gastric wall of an aged orangutan (Pongo pygmaeus).

A 34-year-old female orangutan (Pongo pygmaeus) developed renal failure and became uremic. At necropsy, large gastric masses were present around the cardia and in the corpus. Abdominal metastases occurred in the liver, pancreas, and right ovary. Light microscopic examination of the tumor revealed polygonal cells with vesicular nuclei and prominent nucleoli. The growth pattern was predominantly solid. Focal areas contained excentric cytoplasmic intermediate filament inclusions, as identified by immunohistochemistry and electron microscopy. Immunohistochemical procedures demonstrated mainly the vimentin type of intermediate filaments. Except for occasional cytokeratin, other intermediate filament markers and neural, lymphocytic, and histiocytic markers stained negative. The morphologic and ultrastructural characteristics are typical for a malignant rhabdoid tumor, a term used in human pathology to describe a rare and extremely aggressive malignancy of uncertain histogenesis. Although usually located in the infant kidney, a few reports have documented the occurrence of similar lesions in extrarenal sites of adults. In human tumors, vimentin is often combined with the expression of cytokeratins. The sparsity of the cytokeratin filaments in this case might be due to species-specific variations and/or may reflect the hypothesis of a phenotypic concept encompassing a spectrum of histogenetic diversity.

[Adenomatous neoplasia of the middle ear]. / Die adenomatöse Neoplasie des Mittelohres.

Adenomatous neoplasm occurring in the middle ear is rare. The clinical findings are non-specific and include otological symptoms as well as sometimes functional deficits of the lower cranial nerves. The final diagnosis will be made only after elaborate histological examinations because of the inconsistent radiological and clinical findings. DNA analysis yields some information on the prognosis of this tumour. We report on a case where a primary adenomatous neoplasm developed in the middle ear, and discuss our concept of therapy.

Dissection following balloon angioplasty of aortic coarctation: review of the literature.

Balloon angioplasty of the coarctation of the aorta can result in intimal or medial or even adventitial dissections as demonstrated by in vitro studies and animal experiments. As a typical sign of stretching of the aortic wall, patients complain of pain during the angioplasty procedure. In the literature, aortic wall rupture and ventricular fibrillation during the procedure are reported. Additional sudden deaths can occur within 40 hours after the procedure. Mortality ranges from 0.1% to 2.5%. By transesophageal echocardiography, monitoring of balloon angioplasty, control of the positioning of the balloon, and control of the results and detection of complications are possible. Intimal as well as medial dissections can be detected with observed healing for intimal but also medial dissections. In order to avoid the patient's discomfort, intraaortic ultrasound will be used in the future, when major methodological improvements are done. Computed tomography demonstrates medial dissections but is not able to visualize intimal dissections. Using computed tomography and magnetic resonance after angioplasty of the coarctation of the aorta, irregularities are described in up to 17% of the patients. For angiography, a low detection rate of medial dissections has to be expected, when not biplane angiographies of the whole thoracic aorta are performed. Medial dissections can be seen, but intimal dissections are missed. In conclusion, a review of the literature demonstrates a high incidence of intimal and medial dissections after angioplasty of the coarctation of the aorta with spontaneous healing in most patients. As is the way with coronary angioplasty, aortic wall ruptures are rare, but stand-by surgery is necessary.

[Secondary achalasia in non-Hodgkin's lymphoma of low malignancy and leiomyomatosis of the cardia]. / Sekundäre Achalasie bei niedrigmalignem Non-Hodgkin-Lymphom und Leiomyomatose der Kardia.

A 28-year-old man had been dysphagic for 9 months with a weight loss of 4 kg. A preliminary diagnosis of primary achalasia was made on the basis of typical radiological and manometric findings. Despite balloon dilatation of the cardia the symptoms did not improve and further diagnostic tests were performed. Ultrasound demonstrated a 4 cm tumour below the cardia. But its type and possible malignancy remained uncertain even at laparotomy. But as a malignant tumour was suspected a gastrectomy and omentectomy with removal of the local and regional lymph nodes were performed. After this the symptoms regressed and postoperative food intake was without problem. Histological examination of the surgical specimen revealed leiomyomatosis of the cardia and the gastric fundus, combined with a low-malignant B-cell lymphoma of the mucosa-associated lymphatic tissue. Oesophagus manometry 4 months postoperatively gave normal results. The patient has been free of symptoms and without evidence of recurrence for by now 18 months postoperatively.

Alport-type glomerulopathy: evidence for diminished capillary loop size.

Hereditary nephropathy of the Alport type is morphologically characterized by a specific and diagnostic thinning and splitting lesion of the glomerular basement membranes, which can be recognized only by electron microscopy. The light microscopical aspect has not been considered to be characteristic until now. This paper describes a light microscopical constellation of glomerular alterations by which ATGP can be recognized with high probability. Three histological features are of importance: 1. ATGP glomeruli in patients older than 10 years of age mostly have smaller capillary loops than age-matched controls. However, during the first 10 years of life no difference in glomerular capillary loop size was noticed. 2. ATGP loops often stain less intensely with basement membrane stains. 3. Presence of fetal-like glomeruli. Using this triad of light microscopic parameters as a screening tool, ATGP-cases were found without knowledge of any clinical data among other glomerulopathies with a sensitivity of 72% and a specificity of 93%. The definitive diagnosis, however, depends on electron microscopy.

Fibrosing alveolitis responsive to corticosteroids following Legionnaires' disease pneumonia.

Two male patients ages 54 and 58 years had persisting pneumonia with dry cough, dyspnea, weight loss, and fever up to 39 degrees C that did not respond to erythromycin treatment. There was extensive restrictive impairment of ventilation and loss of diffusing capacity for carbon monoxide. Histologic examination of the basal pulmonary infiltrates showed fibrosing alveolitis. Serologic titers indicated that the patients had suffered from Legionella pneumophila infection. We believe that Legionella had caused the fibrosing alveolitis since there was absence of any other causative agents or factors. Both patients responded to corticosteroid treatment with rapid clinical improvement but delayed radiologic regression.

[Obstructive jaundice: its histological diagnosis by percutaneous endoluminal bile duct biopsy]. / Verschlussikterus: histologische Diagnostik durch perkutane endoluminale Gallengangsbiopsie.

32 forceps biopsies were performed in 30 patients with obstructive jaundice during percutaneous transhepatic biliary drainage procedures. In one patient an adequate specimen could not be obtained. In 25 of the remaining 31 cases diagnosis was confirmed histologically (malignant tumours: n = 22, benign stricture: n = 3). In 6 patients false-negative results were obtained. Transluminal biopsy is an easily performed adjunct to percutaneous transhepatic diagnostic interventions with minimal additional discomfort for the patient. In many cases percutaneous needle biopsy can be avoided. Forceps biopsy enables nonoperative histological diagnosis of small carcinomas of the bile ducts.

Bellini duct carcinoma: further evidence for this rare variant of renal cell carcinoma.

Bellini duct carcinomas have recently been identified as a new entity in the spectrum of renal cell carcinomas and 10 cases have now been reported. The present paper adds detailed clinical and morphological data on six new cases. In addition, immunohistological and electronmicroscopical results support the origin of these tumours from the renal collecting ducts, especially the papillary ducts (Bellini ducts). A set of immunohistological reactions, including reactions to cytokeratins 13 and 19, vimentin and UEA-1 was found to facilitate the differential diagnosis of Bellini duct carcinomas from other renal cell carcinomas and infiltrating urothelial carcinomas of renal pelvis.

[An association of arterial occlusive disease with cyclosporine therapy after kidney transplantation]. / Assoziation einer arteriellen Verschlusskrankheit mit Ciclosporin-Therapie nach Nierentransplantation.

Renal transplantation followed by immunosuppression with cyclosporine (whole-blood levels 200-300 micrograms/l) and methylprednisolone (4 mg daily) was performed in a 54-year-old man with chronic glomerulonephritis. Three years later rapidly progressive arterial obstructive disease (peripheral type) developed. Parenteral treatment with prostaglandins, calcium antagonists and nitrates, as well as a lumbar sympathectomy, was unsuccessful so that, in rapid succession, several amputations on upper and lower limbs became necessary. The disease progression was arrested only when azathioprine replaced cyclosporine. Raynaud's phenomenon, present at the time, also disappeared and analgesics were no longer required. Histological examination revealed severe Mönckeberg arteriosclerosis and thromboembolic occlusion of the affected vessels. The cyclosporine treatment, especially the hypercoagulability induced by it, and the Mönckeberg arteriosclerosis were thought to be significant factors in the pathogenesis of the rapidly progressive arterial disease. It is concluded that, in the presence of progressive arterial obstructive disease occurring under cyclosporine treatment, the replacement by other immunosuppressive agents should be considered.