Calcineurin inhibitors exacerbate coronary arteritis via the MyD88 signalling pathway in a murine model of Kawasaki disease.

Calcineurin inhibitors (CNIs) have been used off-label for the treatment of refractory Kawasaki disease (KD). However, it remains unknown whether CNIs show protective effects against the development of coronary artery lesions in KD patients. To investigate the effects of CNIs on coronary arteries and the mechanisms of their actions on coronary arteritis in a mouse model of KD, we performed experiments with FK565, a ligand of nucleotide-binding oligomerization domain-containing protein 1 (NOD1) in wild-type, severe combined immunodeficiency (SCID), caspase-associated recruitment domain 9 (CARD9)-/- and myeloid differentiation primary response gene 88 (MyD88)-/- mice. We also performed in-vitro studies with vascular and monocytic cells and vascular tissues. A histopathological analysis showed that both cyclosporin A and tacrolimus exacerbated the NOD1-mediated coronary arteritis in a dose-dependent manner. Cyclosporin A induced the exacerbation of coronary arteritis in mice only in high doses, while tacrolimus exacerbated it within the therapeutic range in humans. Similar effects were obtained in SCID and CARD9-/- mice but not in MyD88-/- mice. CNIs enhanced the expression of adhesion molecules by endothelial cells and the cytokine secretion by monocytic cells in our KD model. These data indicated that both vascular and monocytic cells were involved in the exacerbation of coronary arteritis. Activation of MyD88-dependent inflammatory signals in both vascular cells and macrophages appears to contribute to their adverse effects. Particular attention should be paid to the development of coronary artery lesions when using CNIs to treat refractory KD.

Prognostic significance of Fuhrman grade and age for cancer-specific and overall survival in patients with papillary renal cell carcinoma: results of an international multi-institutional study on 2189 patients.

PURPOSE: Because the prognostic impact of the clinical and pathological features on cancer-specific survival (CSS) and overall survival (OS) in patients with papillary renal cell carcinoma (papRCC) is still controversial, we want to assess the impact of clinicopathological features, including Fuhrman grade and age, on survival in surgically treated papRCC patients in a large multi-institutional series. METHODS: We established a comprehensive multi-institutional database of surgically treated papRCC patients. Histopathological data collected from 2189 patients with papRCC after radical nephrectomy or nephron-sparing surgery were pooled from 18 centres in Europe and North America. OS and CSS probabilities were estimated using the Kaplan-Meier method. Multivariable competing risks analyses were used to assess the impact of Fuhrman grade (FG1-FG4) and age groups (<50 years, 50-75 years, >75 years) on cancer-specific mortality (CSM). RESULTS: CSS and OS rates for patients were 89 and 81% at 3 years, 86 and 75% at 5 years and 78 and 41% at 10 years after surgery, respectively. CSM differed significantly between FG 3 (hazard ratio [HR] 4.22, 95% confidence interval [CI] 2.17-8.22; p < 0.001) and FG 4 (HR 8.93, 95% CI 4.25-18.79; p < 0.001) in comparison to FG 1. CSM was significantly worse in patients aged >75 (HR 2.85, 95% CI 2.06-3.95; p < 0.001) compared to <50 years. CONCLUSIONS: FG is a strong prognostic factor for CSS in papRCC patients. In addition, patients older than 75 have worse CSM than patients younger than 50 years. These findings should be considered for clinical decision making.

Genome-wide methylation analysis of tubulocystic and papillary renal cell carcinomas.

Tubulocystic renal cell carcinoma (TRCC) represents a rare tumor with incidence lower than 1 % of all renal carcinomas. This study was undertaken to contribute to characterization of molecular signatures associated with TRCC and to compare them with the features of papillary renal cell carcinoma (PRCC) at the level of genome wide methylation analysis.We performed methylated DNA immunoprecipitation (MeDIP) coupled with microarray analysis (Roche NimbleGen). Using the CHARM package, we compared the levels of gene methylation between paired samples of tumors and control renal tissues of each examined individual. We found significant global demethylation in all tumor samples in comparison with adjacent kidney tissues of normal histological appearance but no significant differences in gene methylation between the both compared tumor entities. Therefore we focused on characterization of differentially methylated regions between both tumors and control tissues. We found 42 differentially methylated genes.Hypermethylated genes for protocadherins (PCDHG) and genes coding for products associated with functions of plasma membrane were evaluated as significantly overrepresented among hypermethylated genes detected in both types of renal cell carcinomas.In our pilot study, we provide the first evidence that identical features in the process of carcinogenesis leading to TRCC and/or to PRCC may be found at the gene methylation level.

Review of renal tumors associated with Birt-Hogg-Dubé syndrome with focus on clinical and pathobiological aspects.

Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder characterized by clinical features of skin lesions, pulmonary lesions and renal tumor. The gene responsible for this syndrome is located on chromosome 17p11.2 and designated as FLCN. In this article, we review renal tumors associated with BHDS with a focus on clinical and pathobiological aspects. Renal tumors often occur multifocally or bilaterally in the imaging analyses or gross examination. Histological examination of renal tumors includes a variety of subtypes such as hybrid oncocytic tumor, chromophobe renal cell carcinoma (RCC), oncocytoma, clear cell RCC and papillary RCC. The histologic discordance in multiple tumors seems to be characteristic of this syndrome. Oncocytosis is observed histologically in about half of the cases. Several investigations have elucidated that folliculin may be involved in the mammalian target of rapamycin (mTOR) pathway recently. Renal tumors composed of clear cells may behave in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion.

[Simultaneous procedure in patients with abdominal aortic aneurysm and renal carcinoma]. / Jednodobý výkon u aneuryzmatu brisní aorty a renálního karcinomu.

INTRODUCTION: Renal carcinoma (RC) is present in 0.1-3% of patients with abdominal aortic aneurysm (AAA). There are several options for surgical solution as regards the type and timing of the procedure. The authors discuss the simultaneous AAA resection and radical nephrectomy as a treatment option. MATERIAL AND METHODS: 1168 patients with AAA including 19 (1.6%) with asymptomatic AAA (diameter >50mm) and RC (average diameter 74.3 mm) indicated for radical nephrectomy were operated on in the last 20 years (between 5/1994 and 5/2014). The average age of the patients was 72.8 years (5585). Pre-operatively, sixteen patients were classified as ASA II, three as ASA III. Unilateral nephrectomy was performed in 18 and bilateral nephrectomy in one patient. One-stage AAA resection (1x endovascular aneurysm repair) with nephrectomy was performed in all patients. RESULTS: Two patients with ASA III classification died within 30 days postoperatively due to acute myocardial infarction and multiorgan failure. In the other patients, the simultaneous procedure was without any complications. Four patients died within two years after the operation due to generalized RC, the others have survived in the interval of one and twelve years after the procedure. CONCLUSION: Simultaneous procedure does not represent increased operation load for patients with good health status. Nevertheless, the type of procedure should be optimised for each individual patient with regard to his or her general health status, life expectancy, disease symptomatology, the diameter of the RC and the anatomical conditions of AAA.

Typical signs of oncocytic papillary renal cell carcinoma in everyday clinical praxis.

PURPOSE: Types 1 and 2 by Delahunt of papillary renal cell carcinoma (PRCC) are traditionally differentiated. An oncocytic variant of PRCC (O-PRCC) has recently been described. We compare clinical data of O-PRCC with other subtypes of PRCC such as the main tumour size, necrotic changes, presence of pseudocapsula and real extrarenal growth in retrospective study. METHODS: From 1/1992 to 10/2009, 1,398 patients with 1,436 renal tumours were surgically treated in our institution. PRCCs were described in 109 (7.6%). Among PRCC, O-PRCCs were in 12 (11%), PRCC type 1 in 86 (78.9%), PRCC type 2 in 8 (7.3%) and others in 3 (2.8%) cases. RESULTS: The patient's mean age with O-PRCC (M:F ratio = 2:1) was 67.5 +/- 10.9 versus 63.5 +/- 14.1 in type 1 and 57.9 +/- 5.7 in type 2, the mean tumour size was 35 +/- 12 mm versus 47 +/- 22 and 37 +/- 17, respectively. The follow-up of O-PRCC is 35.3 +/- 12.3 months and all cases are without recurrence. We did not find any pseudocapsula in O-PRCC but it was a major sign of PRCC type 1 (32.6%). Huge microscopic necrotic changes were described in 33.3% of O-PRCC, in 33.7% of PRCC type 1 and 62.5% of PRCC type 2. Extrarenal growth was found only in 16.7% O-PRCC versus 40.7% in PRCC type I. CONCLUSIONS: None of the O-PRCC had pseudocapsula and none had massive necroses in comparison of O-PRCC with PRCC types I and II. Extrarenal growth in O-PRCC is relatively rare. The malignant potential of O-PRCC is low.

[Urogenital trauma associated with pelvic ring fractures]. / Urologické poranení pri zlomeninách pánevního kruhu.

PURPOSE OF THE STUDY: To evaluate, in a retrospective study, injuries to the urogenital tract in patients with pelvic ring fractures. MATERIAL In the years 1998-2007, a total of 308 patients with pelvic ring fractures were treated. The study did not comprise patients with low-energy fractures, such as apophyseolysis in children, osteoporotic bone fractures or pathologic fractures. It also did not include patients with multiple injuries who died within 6 hours of admission to the hospital. The group consisted of 186 men and 122 women with an average age of 34 (range, 6 to 76) years. RESULTS: The fractures sustained were classified as type A in 5 %, type B in 57 % and type C in 38 % of the patients. The average follow-up was 71 (range, 13 to 121) months. A primary injury to the urogenital tract was recorded in 50 (16 %) patients. Injury to the urethra was found in 23 (7.5%) and urinary bladder trauma in 18 (6%) patients, vaginal injury was in four women (1%), and penis injury in three (1%) and lacerated testicles in two men (1%). Injury to the urogenital tract was associated with a pelvic ring fracture type A in 5 %, type B in 34 % and type C in 61 % of the patients. Out of the 23 patients with urethral trauma, only six (26 %) were free from functional and subjective complaints; eight (35 %) continued to receive therapy for urethral stenosis seven (30 %) reported urinary incontinence, and seven men (30 %) had erection problems. In six patients (26%) the lasting sequelae were combined. The 18 patients with injury to the bladder reported no subjective complaints at a one-year follow-up. Two patients with penis root injury had erectile dysfunction. Two patients with the loss of both testicles were in the care of a psychiatrist. The patients' satisfaction was evaluated on a 0-to10-point scale. The average value for the whole group was 4.1 points. In the patients with erectile dysfunction, the value was 0.8, and in those with isolated injury to the urinary bladder it was 9.4 points. DISCUSSION: The increasing number of injuries to the urogenital tract associated with permanent sequelae is caused by a growing number of pelvic ring fractures as well as, and this is more important, by decreasing mortality in patients with severe trauma to the pelvic ring The extent of urogenital injury is related to the degree of dislocation of the pelvic skeleton. Injury to the male urethra is the most frequent urogenital trauma because of the male anatomy. It occurs most often in unstable C type fractures when the pelvic ring is disrupted with bone displacement due to shear force at the site of urethra attachment. The consequences are related to the quality of treatment of urogenital tract injury as well as to how the skeletal injury is managed. The lasting effects of the primary injury to nerve structures are beyond repair by therapy. CONCLUSIONS: Injury to the urethra results in erectile dysfunction in 50 % of the injured patients it is often associated with urinary incontinence that has a strong effect on the patient's life quality. Isolated trauma to the urinary bladder has a good prognosis. The incidence of post-traumatic incontinence is not high, but reduces the patient's personal and social comfort. A prerequisite for a successful therapeutic outcome is a good cooperation of the orthopaedic surgeon and urologist. Key words: pelvic ring fractures, urogenital injury.

Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity.

We present a series of a distinct tumorous entity named renal angiomyoadenomatous tumor (RAT). Five cases were retrieved from the consultation files of the authors. Histologic and immunohistochemical features were evaluated. Sequencing analysis of coding region of the VHL gene was carried out in all cases. The tumors were composed of admixture of an epithelial clear cell component and prominent leiomyomatous stroma. Epithelial cells formed adenomatous tubular formations endowed with blister-like apical snouts. All tubular/glandular structures were lined by a fine capillary network. The epithelial component was positive for epithelial membrane antigen, CK7, CK20, AE1-AE3, CAM5.2, and vimentin in all cases. In all analyzed samples, no mutation of the VHL gene was found. RAT is a distinct morphologic entity, being different morphologically, immunohistochemically, and genetically from all renal tumors including conventional clear cell carcinoma and mixed epithelial and stromal tumor of kidney.

[Neoplastic thrombosis of the inferior vena cava in kidney carcinoma]. / Nádorový trombus dolní duté zíly u karcinomu ledviny.

INTRODUCTION: Neoplastic thromboses of the inferior vena cava occur in about 4-15% of patients with conventional, clear-cell renal carcinoma, and significantly increase intraoperative mortality and morbidity rates. AIM: Assessment of outcomes of radical nephrectomies with inferior vena cava thrombectomies, performed in the PlzeN Faculty Hospital from 01-01-1999 to 31-12-2008. PATIENT GROUPS AND METHODS: The study included 16 patients, 12 males and 4 females of 63.6 years of age on average (44-75 y.o.a), suffering from conventional, clear-cell renal carcinoma and neoplastic thrombosis of the inferior vena cava. The main symptoms included hematuris in 7 subjects (43.8%), lumbar pressure pain in 4 subjects (25%), leg edema in 2 subjects (12.5%). In 2 patients (12.5%), the finding was accidental and in 1 patient (6.3%), the symptoms were caused by distant metastases. The diagnostic methods included duplex sonography, multidetector computed tomography, magnetic resonance imaging and cavography. In three subjects, the thrombus was located in the renal vein only (grade I), in 9 subjects, the thrombus propagated below the entry of the hepatic veins (grade II), in 3 subjects, the thrombus extended above the entry of the hepatic veins (grade III) and, in 1 patient, it reached the right atrium (grade IV). All the patients underwent transabdominal radical nephrectomy (pT3b,c,pN0-2,pM 0-1) and inferior vena cava thrombectomy. RESULTS: The average duration of IVC closure using vascular clips was 6.3 minutes (3-12 minutes), the average blood loss was 1.7 litres. The intraoperativev mortality rate of the whole study group was 0%. In a single patient, an incompletely removed thrombus resulted in massive pulmonary embolization on day 3, which was urgently managed by cardiosurgery with extracorporeal circulation, when the thrombotic mass was removed from the pulmonary artery. Three subjects (18.8%) died due to generalization of the underlying disease 5-17 months after the primary procedure. Two subjects (12.5%) are currently receiving symptomatic treatment for their generalized underlying disease. 11 subjects (68.8%) show no signs of a relaps of the underlying disease at 3 month-5 years after the procedure. CONCLUSION: Radical nephrectomy combined with complete removal of the neoplastic thrombus provides the best prospects for long-term survival rates of the patients.

[Laparoscopic adrenalectomy]. / Laparoskopická adrenalektomie.

OBJECTIVE: Laparoscopy has become the gold standard for the treatment of adrenal tumours in urology. We evaluate our experience with laparoscopic adrenalectomy (LA) in this work. MATERIAL, METHODS: We performed 38 LA between 2003-2008. We use computer tomography (CT) and magnetic resonance imaging (MRI) for the initial evaluation. Indication for procedure is made in cooperation with endocrinologist. We use transperitoneal approach with 3 or 5 ports. RESULTS: Mean age was 57.7 +/- 11.7 year (range 32-74.9 year). Nine LA were made in men (24%), in women 29 (76%). Sixteen tumours (42%) were hormonal active (7 pheochromocytoma, 6 primary hyperaldosteronism, 3 peripheral hypercortisolism). Twenty-two tumours were without hormonal activity. Mean tumour size was 4.1 +/- 2 cm (range 1-10.1 cm), mean operation time was 89 +/- 38 minutes (range 32-220 minutes), mean blood loss was 33 +/- 75 ml (range 0-400 ml), mean hospitalization time was 6.1 days (range 3-12 days). There were histologically 15 cortical adenomas, 5 nodular cortical hyperplasia, 1 calcificated hematoma, 3 cysts, 2 potentional malignant tumours on interface between adenoma and carcinoma, 1 cortical carcinoma and 7 pheochromocytoma. We found 3 metastases of renal carcinoma in adrenal gland and one metastasis mesenchymal chondrosarcoma too. Transperitoneal approach was chosen in 20 patients (53%) after previous abdominal operation (open cholecystectomy, appendectomy, transperitoneal nephrectomy, aortofemoral bypass). Complications were in 3 cases from 38 (8%). It was one perforation of diaphragm, which was resolve with laparoscopic suture, one postoperative delirium with fudge and agitation, one abscess in wound after extraction of specimen. We have got any conversion in our collection. The body mass index was higher than 38 in 3 patients. CONCLUSION: LA is a quick and safe procedure with minimal morbidity and mortality. This procedure requires very experienced surgeon. Patients profit especially from miniinvasivity and short convalescence. Especially benign tumours of smaller size (by 8 cm) are indicated, extensive and especially malignant tumours remain a domain of open approach. Previous operations in abdominal cavity do not have to be a contraindication for LA and operation is possible in patients with monster obesity.

Tumours in end-stage kidney.

OBJECTIVE: Patients with end-stage kidney disease (ESKD) show a greater risk for renal cell carcinoma (RCC), which tends to be multifocal and bilateral. The malignant potential is unclear. The question is whether to remove both kidneys in patients with a tumor on one side only diagnosed by computed tomography (CT). MATERIALS AND METHODS: Kidney tumors were found in 14 patients with ESKD from January 2002 to December 2006. One was unfit for surgery. Thirteen patients underwent nephrectomy and 6 a bilateral procedure of whom only 2 had bilateral tumors on CT, 3 multiple tumors on the contralateral side, and 1 uncontrollable hypertension with tumors as an incidental finding. Tumors were found in all 19 specimens. RESULTS: In 13 kidneys (68.4%), the tumors were multiple; in 6 (31.6%), solitary. The types of tumor were: 13 (68.4%) papillary RCCs (PRCC), 9 (47.4%) clear RCCs (CRCC), a combination of PRCC and CRCC in 4 (21.0%), and myxoid liposarcoma (with solitary PRCC contralaterally). The mean follow-up was short (19 +/- 15 months; maximum, 54 months). Only 1 patient died due to a tumor at 16 months after operation. CONCLUSIONS: There is a high risk for bilateral involvement. Patients who undergo unilateral nephrectomy must be regularly followed and contralateral nephrectomy carefully considered, mainly in transplanted patients on immunosuppression. Further studies are needed to give a definitive answer about the indications for surgery and the indications for contralateral nephrectomy as well. To date, prophylactic contralateral nephrectomy should not be a therapeutic standard.

[Laparoscopic radical nephrectomy--the cohort of 150 patients]. / Laparoskopická radikální nefrektomie pro parenchymový tumor ledviny--soubor 150 pacientu.

BACKGROUND: Minimally invasive surgery has been performing in the treatment of renal cancer at the Department of Urology in Plzen since January 2003. We want to present and evaluate our results of laparoscopic nephrectomy (LRN) in the cohort of 150 patients. METHODS AND RESULTS: In our institution, 150 LRNs were performed in the period between January 2003 and April 2007, 145 of them transperitoneally and 5 retroperitoneally. These 150 LRNs were completed by 4 different surgeons. We use four trocars in a rhombus disposition for left-sided LRN, we add fifth port for liver elevation for right-sided LRN. We place four ports for retroperitoneoscopical LRN. Renal vessels are sectioned either separately with lockable clips or with endoGIA stapler en bloc. After mobilization of the kidney, it is inserted into the endobag and extracted through the extended incision after the 12 mm port in lower abdomen using splitting incision. 73 right and 77 left LRN were performed in our patients cohort. The mean age of patients was 62.5 +/- 10.2 (range 33.9-84.6) years. The mean operation time was 138 +/- 44 (55-270) minutes. The mean size on CT was 54.3 +/- 15.1 (29-101) mm. The mean hospitalization time was 6.1 +/- 2 (3-15) days. Adrenalectomy was performed in 16 patients, cholecystectomy in 4 patients and appendectomy in 3 patients. Per-operative complications appeared in 5 cases (3%). Post-operative complications appeared in 7 patients (5 %). We can not evaluate own oncological results because of short-time follow-up. CONCLUSIONS: LRN has become a new gold standard in the treatment of kidney tumours T1 (to 7 cm) and it represents an important approach in the treatment of kidney tumours in our institution. We highly recommend preoperatively biphasic CT angiography because it shows the exact number and topography of renal vessels. We prefer transperitoneal approach because of the larger working space and better topographical orientation. Patients profit from advantages of miniinvasivity mainly due to the faster return to the normal life. To the main disadvantages of this technique belong the higher technical demands on the operator and a higher prize of the procedure compared to the open operation.

[Intratubular germ cell neoplasia--review article]. / Intratubulární germinální neoplazie--prehled problematiky s prihlédnutím ke klasifikaci WHO 2004.

Intratubular germ cell neoplasia is a precursor lesion for germ cell testicular tumors. It is defined as presence of germ cells with abundant vacuolated cytoplasm and large irregular nuclei with nucleoli within seminiferous tubules. The whole morphologic spectrum of intratubular germinal tumors is discussed. Placental alcaline phosphatase, OCT 3/4 can be demonstrated in majority of the cases. Ultrastructural examination does not play a substantial role in differential diagnosis. Gain of chromosome 12p, which is typical for invasive germ cell tumors is absent in pure intratubular germ cell neoplasia. Spermatogonic arrest and rare reactive changes within seminiferous tubuli have to be distinguished from intratubular germ cell neoplasia.

Stim1 Regulates Enamel Mineralization and Ameloblast Modulation.

Loss-of-function mutations in the Ca2+ release-activated Ca2+ channel genes ORAI1 and STIM1 abolish store-operated Ca2+ entry (SOCE) and result in ectodermal dysplasia with amelogenesis imperfecta. However, because of the limited availability of patient tissue, analyses of enamel mineralization or possible changes in ameloblast function or morphology have not been possible. Here, we generated mice with ectodermal tissue-specific deletion of Stim1 ( Stim1 cKO [conditional knockout]), Stim2 ( Stim2 cKO), and Stim1 and Stim2 ( Stim1/2 cKO) and analyzed their enamel phenotypes as compared with those of control ( Stim1/2fl/fl) animals. Ablation of Stim1 and Stim1/2 but not Stim2 expression resulted in chalky enamel and severe attrition at the incisor tips and molar cusps. Stim1 and Stim1/2 cKO, but not Stim2 cKO, demonstrated inferior enamel mineralization with impaired structural integrity, whereas the shape of the teeth and enamel thickness appeared to be normal in all animals. The gene expression levels of the enamel matrix proteins Amelx and Ambn and the enamel matrix proteases Mmp20 and Klk4 were not altered by the abrogation of SOCE in Stim1/2 cKO mice. The morphology of ameloblasts during the secretory and maturation stages was not significantly altered in either the incisors or molars of the cKO animals. However, in Stim1 and Stim1/2 cKO incisors, the alternating modulation of maturation-stage ameloblasts between the smooth- and ruffle-ended cell types continued beyond the regular cycle and extended to the areas corresponding to the zone of postmodulation ameloblasts in the teeth of control animals. These results indicate that SOCE is essential for proper enamel mineralization, in which Stim1 plays a critical role during the maturation process.

Urological complications of congenital nephrogenic diabetes insipidus--long-term follow-up of one patient.

A male with a severe form of congenital nephrogenic diabetes insipidus (diuresis 10 l per day) had megaureters, megavesica, large residuum and a history of three traumatic ruptures of the megavesica and a recurrent urinary tract infection (RUTI). Hemodialysis was introduced at 41 years of age. At age of 42, he underwent a bilateral retroperitoneoscopic nephrectomy to prevent further RUTI and 8 months later transplantation of a cadaver kidney. Intermittent catheterization is necessary due to residual urine in the urinary bladder.

Chromophobe renal cell carcinoma with microcystic and adenomatous arrangement and pigmentation--a diagnostic pitfall. Morphological, immunohistochemical, ultrastructural and molecular genetic report of 20 cases.

We present clinical, morphological, immunohistochemical, ultrastructural and molecular genetic features of 20 cases of a peculiar form of chromophobe renal cell carcinoma (CRCC) with morphology differing from that of conventional CRCC. Microscopically, the typical features of the tumors were microcystic arrangement and formation of adenomatous structures. Microcystic areas were composed of smaller eosinophilic and bigger pale cells having cytological appearance typical of conventional CRCC. Cytological features of the adenomatous structures were mostly different from those of conventional CRCC. They had a typical columnar arrangement with nuclei positioned at the base of the glandular structures and a small amount of a deeply eosinophilic cytoplasm often endowed with brush border facing the lumen of the glands. In addition, all the tumors showed a brown pigmentation. The pigmentation was located mostly extracellularly, where it formed pools of heavy deposits. Microscopic calcifications present in all cases formed psammoma bodies or else the calcifications were more extensive and amorphous in shape. Ultrastructurally, the cells showed features characteristic of CRCC: typical cytoplasmic vesicles were 100-700 nm in size and mitochondria had tubulovesicular, lamellar or circular cristae. Some tumor cells contained dark, variously sized electron-dense pigment granules. Neither melanosomes nor membrane-bound neurosecretory granules were seen. Using fluorescence in-situ hybridization probes for chromosomes 1, 2, 6, 10, 13, 17 and 21, the tumors revealed massive loss of tested chromosomes typical for conventional CRCC. Monosomy of chromosomes 1, 2, 6, 10, 13 and 21 was found in 100, 36, 91, 82, 82, 82 and 64% of cases, respectively. None of the cases showed mutation of exons 9, 11, 13 and 17 of the c-kit gene. The important feature of pigmented microcystic chromophobe renal cell carcinoma is a relatively benign biological behavior and the absence of distant metastases and sarcomatoid transformation.

[Tumorous diseases in patients with the testicular feminization syndrome ("androgen insensitivity" syndrome)--description of two cases]. / Náidorová onemocnení pacientu se syndromem testikulární feminizace ("androgen insensitivity" syndrom)--popis dvou prípadú.

OBJECTIVE: To describe tumors occurring in two cases of testicular feminization syndrome. SUBJECT: Case report. SETTING: Dpt. of Special Diagnostics SPAU, University Hospital Plzen. SUBJECT AND METHOD: Two cases of testicular feminization syndrome were selected from four cases in our registry. Patients were 45 and 84-year-old Caucasian "females". Resected material was fixed in formaldehyde, routinely processed and stained with hematoxiline-eosin, inhibin, cytokeratines 20, placentar alkaline phosphatase, CD 99, Melan A, hCG. Sertoli cell adenoma was diagnosed in both patients. Older patient had in addition unclassified sex cord tumor of Leydig cell type. The number of sex chromosomes was examined using FISH analysis in both patients. CONCLUSION: Patients with testicular feminization syndrome are frequently affected by benign or malignant tumors in the cryptorchid testes. We documented two benign Sertoli cell adenomas and one sex cord tumor of uncertain biological behavior in our patients. The testes should be removed after puberty with subsequent estrogen therapy in patients with testicular feminization syndrome.

[Renal angiomyolipoma, histology, diagnostics and therapy]. / Renální angiomyolipom, histologie, diagnostika a terapie.

BACKGROUND: Renal angiomyolipoma is a benign mezenchymal tumour with prevalence of 0.3-3% of all surgically resected renal tumours. Tumour is often associated with tuberous sclerosis complex or with another fakomatosis. Tumour has typical pathological image in computer tomography notation that enables the diagnosis. Symptomatology and possible complications depend on the tumour size. Large or symptomatic tumours are indicated for resection, nephrectomy or local ablation. METHODS AND RESULTS: 612 patients with renal tumour were operated at the Department of Urology faculty hospital in Pilsen. Angiomyolipoma occurred in 7 patients. Average age at the time of operation was 64 years, ratio female and male was 5:2. Clinical symptomatology was expressed in 3 patients. The size of tumour was 2 to 8 cm, in 5 patient the tumors were solitary. Translumbal tumour resection was performed in 3 patients. These tumours were at the same time multifocal. Suspicion from tuberous sclerosis was pronounced in some care. The angiomyolipoma was accidentally found in 2 cases of nephrectomy, which was indicated for other diagnosis. CONCLUSIONS: We define diagnosis by means of CT notation in the most of patients. This notation is not typical by any version of angiomyolipoma and we choose therefore surgical revision.

[Miniinvasive laparoscopic or retroperitoneoscopic radical nephrectomy for the parenchymal tumor]. / Miniinvazivní laparoskopická ci retroperitoneoskopická radikální nefrektomie pro parenchymový tumor.

INTRODUCTION: Laparoscopic (LRNE) or retroperitoneoscopic (RRNE) radical nefrectomy originally published in 1991, respectively in 1993, began to be introduced in common practice at the end of the 20th century. In this work we summarize our findings gathered upon introduction of these methods. SUBJECTS AND METHODOLOGY: We present a group of 50 miniinvasive nephrectomies conducted during 2003-2004 (46 LRNE and 4 RRNE). LRNE was combined with ipsilateral adrenalectomy in five cases and with cholecystectomy in three cases (in all cases the LRNE was on the right side). RESULTS: The mean age of the patient subjects was 65 +/- 11 years (34-81). The blood loss was 134 +/- 201 ml (30-1200). The tumor size according to the CT was 50 +/- 13 mm (30-82). Histologically, 45x renal carcinomas, 4x oncocytomas, 1 x urothelial carcinomas were diagnosed. The drain was removed 2.1 +/- 0.9 days following the surgery. The mean hospitalization time was 6.1 +/- 1.6 days (4-9). Only a single major, however lethal, complication arised: In a 74-year old female, the left-sided LRNE due to the carcinoma pT3bG2 and at the same time vaginal hysterectomy, extraction of the renal preparation through the vagina and anterior and posterior vaginoplasty (for a prolaps) were conducted. The patient was repeatedly revised for haemoperitoneum and she exited on the 18th postoperative day due to pneumonia. The mean duration of the LRNE was 168 +/- 40 minutes (80-265). The combined LRNE and cholecystomic procedure lasted 265 minutes. The mean duration of the LRNE with CHE was 213 minutes, and of the LRNE with adrenalectomy was 170 minutes. In 4 cases of the RRNE (indicated three times due to preceeding major surgical procedures in the abdominal cavity and once for the Crohn's disease) the mean duration was 203 minutes. CONCLUSION: Miniinvasive RNE in TI tumors is a modem reproducible methodology suitable for application in clinical practice. The laparoscopic approach appears more appropriate. The retroperitoneoscopic approach is indicated mainly in cases of postoperative adhesions in the abdominal cavity.