A Giant Ureteral Inflammatory Myofibroblastic Tumor in a 4-Year-Old Child: A Case Report and Review of the Literature.

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of children and young adults. It consists of myofibroblastic cells of borderline malignancy admixed with inflammatory cells. According to the WHO classification, of tumors, it is a soft-tissue tumor with an intermediate malignant potential. It may arise within numerous organs. Ureteral location is exceedingly rare. We report the exceptional case of a 4-year-old boy presenting with a giant right ureteral IMT removed during a nephroureterectomy. Histology and immunohistochemistry confirmed the diagnosis of an IMT with Anaplastic Lymphoma Kinase 1 expression. A 7 months follow-up showed no recurrence or distant metastasis. We also reviewed the literature for similar cases of pediatric ureteral IMT.

Duplication of the urethra in an adult male presenting with scrotal fistula: a rare case report.

Urethral duplication is a rare congenital abnormality with varied clinical manifestations; to this day, <300 cases were reported in the literature. It is affecting mainly males and is nearly always diagnosed in childhood or adolescence. It may be complete or incomplete, and the most widely accepted classification of the different types of urethral duplication was developed by Effman et al. Herein, we present a rare case of urethral duplication revealed by scrotal fistula in an adult man. A duplication is an unusual form of Y-type duplication: it is an incomplete urethral duplication urethra opening on the scrotum without communication with the urethra or bladder.

Right-sided varicocele thrombosis complicating an acute epididymo-orchitis: A case report and review of the literature.

Unilateral right-sided varicocele is a rare and warning condition that should hint at a serious retroperitoneal disease such as renal cell neoplasm. Furthermore, its thrombosis is such an unusual entity that only a few cases have been described. We review the literature and report, to our knowledge, the second case of acute scrotal pain caused by unilateral right-sided varicocele thrombosis complicating an ipsilateral epididymo-orchitis, with no underlying coagulopathy or abdominal neoplasm, to illustrate the clinical, morphologic and therapeutic features of this entity.

Synergistic effect and VEGF/HSP70-hom haplotype analysis: relationship to prostate cancer risk and clinical outcome.

Prostate cancer (PCa) is a complex disorder resulting from the combined effects of multiple environmental and genetic factors. Our previous single-locus analysis showed that VEGF and HSP70-hom polymorphisms were significantly associated with PCa susceptibility and prognosis. Both genes encoding these proteins were located on chromosome 6p21, and combining the neighboring single nucleotide polymorphisms (SNPs) into haplotypes may increase the association with the disease. Three tagging polymorphisms, the HSP70-hom 2437 T/C, the VEGF-1154 G/A, and the VEGF-634 G/C SNPs were genotyped in 101 cases and 80 controls. For the combined analysis of VEGF and HSP70-hom, we found a positive gradient in the odds ratios (ORs) related to the number of high-risk genotypes with a 3.53-fold increase of prostate carcinoma risk (OR = 3.53; p = 0.015). Furthermore, the TAG and CAG haplotypes at positions HSP70-hom, VEGF -1154 and VEGF -634 exhibited a two-fold (OR = 0.46; p = 0.014) and a seven-fold (OR = 0.14; p = 0.00005) reduction in PCa risk, respectively. Regarding PCa prognosis, the TAG haplotype had a negative association with the aggressive phenotype as defined by the histopathological grade (OR = 0.28; p = 0.006). Our findings confirm the role of at-risk haplotype across the HSP70-hom/VEGF gene cluster in determining susceptibility to PCa.

Combined effects of the angiogenic genes polymorphisms on prostate cancer susceptibility and aggressiveness.

The single-gene approaches in association studies of polygenic diseases are likely to provide limited value in predicting risk. The combined analysis of genetic variants that interact in the same pathway may amplify the effects of individual polymorphisms and enhance the predictive power. To evaluate higher order gene-gene interaction, we have examined the contribution of four angiogenic gene polymorphisms (VEGF-1154G/A; VEGF-634G/C; MMP9-1562C/T and TSP1-8831A/G) in combination to the risk of prostate cancer. For the combined analysis of VEGF and MMP9 SNPs, we found a significant gene-dosage effect for increasing numbers of potential high-risk genotypes. Compared to referent group (low-risk genotypes), individuals with one (OR = 2.79, P = 0.1), two (OR = 4.57, P = 0.02) and three high-risk genotypes (OR = 7.11, P = 0.01) had increasingly elevated risks of prostate cancer. Similarly, gene-gene interaction of VEGF and TSP1 polymorphisms increased risk of prostate cancer in additive manner (OR = 6.00, P = 0.03), although the TSP1 polymorphism itself was not associated with the risk. In addition, we examined the synergistic effect of these polymorphisms in relation to prostate cancer prognosis according to histopathological grade and clinical stage at diagnosis. Cross-classified analysis revealed potential higher order gene-gene interactions between VEGF and TSP1 polymorphisms in increasing the risk of developing an aggressive phenotype disease. Patients carrying three high-risk genotypes showed a 20-fold increased risk of high-grade tumor (OR = 20.75, P = 0.002). These results suggest that the gene-gene interaction of angiogenic gene polymorphisms' increased risk of prostate cancer onset and aggressiveness.

Association of HSP70-hom genetic variant with prostate cancer risk.

Because of the importance of androgens to prostate cancer (PCa) development, several candidate genes along androgen pathway have been under intensive study. Given the role of the molecular chaperone HSP70 in the regulation of the androgen receptor (AR) transactivation function, we first chose to explore the association between the HSP70-hom functional genetic variant (+2437 T > C) and prostate cancer risk by genotyping DNA samples from 101 unselected PCa patients and 105 healthy men. There was a trend towards lower frequency of TC and CC genotypes among patients when compared with healthy controls, however the difference did not reach the statistical significance (TC genotype: OR = 0.53, P = 0.05; CC genotype: OR = 0.42, P = 0.16). Moreover, individuals carrying at least one C allele have a statistically significant lower susceptibility for PCa (OR = 0.51 (0.26-0.97); P = 0.02). Since some factors may influence tumor progression rather than initiation, we also examined the relationship between the HSP70-hom polymorphism and the clinical characteristics of the malignancy at the time of diagnosis. The stratified analysis of the genotypes with the clinical stage and tumor grade showed that there was no significant difference in the risk estimates according to prognostic indicators of PCa disease in our population study. This is the first report on the studies of HSP70 SNPs in PCa and our data suggest that this genetic variant may be a genetic marker for PCa susceptibility in Tunisians.

[To analyse diagnosis, management and prognosis of florid cystitis glandularis (pseudoneoplastic entity)]. / La cystite glandulaire pseudo tumorale.

PURPOSE: To analyse diagnosis, management and prognosis of florid cystitis glandularis (pseudoneplastic entity). PATIENTS AND METHODS: A retrospective study of 6 consecutive patients with florid cystitis glandularis seen at the department of Urology of Monastir (Tunisia) from January 1996 to July 2006. All patients underwent ultrasonography, and cystoscopy. Computed tomography (CT) was performed in four patients and magnetic resonance imaging (MRI) in one patient. RESULTS: The six patients, all of whom were male, ranged from 22 to 68 (average 42) years of age. The most common complaints was haematuria. Radiological examination and cystoscopy suggested a bladder tumor in all patients. Histopathology revealed features of cystitis glandularis of intestinal type. Features of the pelvic CT and MRI were consistent with the diagnosis of pelvic lipomatosis in one patient. The endoscopic resection was performed in 5 patients. Recurrence occurred in one of these 5 patients within one and 7 years of follow-up. The other patient had developed a low compliance small-capacity bladder and bilateral ureteral obstruction. A total cystoprostatectomy and ileal neobladder construction was considered, but it was declined by the patient. The patient was treated with anti-inflammatory drug (Indometacine 100 mg/day). CONCLUSION: Cystitis glandularis may be mistaken for bladder tumor The diagnosis is histological. Treatment is based on eradication of the irritative factors and endoscopic resection of the tumoral masses. The clinical course is unclear, requiring long-term fellow-up.

TSP1 and MMP9 genetic variants in sporadic prostate cancer.

Angiogenesis plays an important role in the initiation and progression of many malignancies including prostate cancer (PCa). Therefore, genes implicated in angiogenic pathways could be susceptibility candidate genes for this malignancy. In this respect, we investigated the impact of functional genetic variants of TSP1 (N700S) and MMP9 (-1562 C/T) genes on the development and progression of PCa. This case-control study included 101 PCa patients and 106 healthy controls analyzed by polymerase chain reaction -restriction fragment length polymorphism assay. No association was observed between any of the TSP1 genotypes and PCa risk or severity; however, subjects carrying one copy of the MMP9 T allele exhibited threefold higher risk of developing PCa (OR = 2.86; P = 0.004). Regarding prognostic value, a significant association was found between the occurrence of the MMP9 T allele and the high-grade tumor (OR = 3.21; P = 0.004) and the advanced disease (OR = 2.47; P = 0.026). We also analyzed the effect of the combined genotypes on PCa risk. The patients with two high-risk genotypes exhibited 2.8-fold higher risk of developing PCa than those with only low-risk genotypes, but the association was not statistically significant. These findings suggest that MMP9 polymorphism is an independent risk factor of PCa development and aggressiveness.

[Prostate cancer: comparative study of stage T1a and stage T1b]. / Cancer de prostate: étude comparative stade T1a et stade T1b.

OBJECTIVE: To compare the epidemiological profiles and histoprognostic factors of two groups of prostate cancer: T1a versus T1b. MATERIALS AND METHODS: Between January 1996 and December 2005, 1359 patients were operated for benign prostatic hyperplasia (BPH) in our urology department. All patients had a PSA less than 4 ng/ml, an adenomatous prostate on digital rectal examination with a homogeneous appearance on prostatic ultrasonography. The population of patients with prostate cancer was classified into group 1 (stage T1a) and group 2 (stage T1b). The following parameters were studied: age at diagnosis, PSA, volume of prostatic tissue removed (by resection or enucleation), histological findings. RESULTS: In this series of 1359 operated patients (762 by endoscopic resection and 597 by transvesicalprostatectomy), 44 (3.23%) had prostate cancer Group 1 comprised 18 patients (40.9%) and group 2 comprised of 26 patients (59.1%). Group 1 had a mean age of 74.7 years, a mean PSA of 2.36 ng/ml and a mean prostatic volume of 37.16 ml. Corresponding values for group 2 were 74.4 years, 3.11 ng/ml and 26.3 ml, respectively The Gleason score was low (2-4 and 5-6) and no grade 4 was observed for any of the patients of group 1, while 50% of patients of group 2 had a high Gleason score (7-10). CONCLUSION: The incidence of stage T1a and T1b prostate cancer is low due to the increasingly systematic use of PSA. The volume of the prostate and transitional zone does not influence the stage T1a and T1b prostate cancer detection rate. T1b cancer has a poorer prognosis than stage T1a cancer.

Association of VEGF genetic polymorphisms with prostate carcinoma risk and clinical outcome.

OBJECTIVES: Vascular endothelial growth factor (VEGF) is a potent stimulus of angiogenesis that has an important role in many human malignancies including prostate carcinoma (PCa). We evaluated the role of the functional VEGF polymorphisms as genetic markers for PCa susceptibility and prognosis. METHODS: The study included 101 patients with PCa and [corrected] 100 age-matched healthy men. The VEGF genotypes -1154G>A were identified by allele-specific polymerase chain reaction (AS-PCR) and the genotypes -634G>C and 936C>T were identified by restriction fragment length polymorphism-polymerase chain reaction (RFLP-PCR). RESULTS: A negative association was found between VEGF -1154AA genotype and PCa risk (OR=0.27; P=0.009). Furthermore, the presence of the VEGF -1154A allele appeared to be associated with a decreased [corrected] risk of higher tumor grade (OR=0.37; P=0.01). A significant increased risk of prostate cancer was associated with the VEGF -634 (GC+CC) combined genotype (OR=1.95; P=0.02). The VEGF -634C allele was associated with the aggressive phenotype of prostate cancer as defined by the high histological grade (OR=3.48; P=0.007). The VEGF -1154A/-634G haplotype was negatively associated with PCa risk (OR=0.48; P=0.005) and high tumor grade compared to low grade (OR=0.37; P=0.02). CONCLUSIONS: Genetic variations in the VEGF may predict not only PCa risk but also tumor aggressiveness.

[An unusual testicular tumour: splenogonadal fusion]. / Une tumeur testiculaire inhabituelle: fusion splénogonadique.

Splenogonadal fusion is a rare congenital anomaly that is often discovered at operation or autopsy. The diagnosis is difficult, but could be based on preoperative scintigraphy and frozen section histological examination to avoid useless orchidectomy. It is a benign lesion, which must be distinguished from testicular tumour.

[Twin pregnancy after renal transplantation: first case reported in Tunisia]. / La grossesse gemellaire et la transplantation renale a propos d'une premiere observation Tunisienne.

Women with end-stage renal disease or on regular dialysis have low fertility. Renal transplantation restores not only normal renal and endocrine functions but also the reproductive function as well and this conception becomes possible. Pregnancy in transplanted women is at higher risk and necessitates a multidisciplinary follow up. We report the course and out come of two successful pregnancies, the second was the first case of twin pregnancy in Tunisia in a transplanted woman. Our patient is 35 years old had a chronic renal insufficiency, secondary to interstitial nephropathy. After six years of hemodialysis, she had received a renal graft from a living donor (his brother). A double drug immunosuppression was given (Prednisolone - Azathioprine). Two years later, she became pregnant and delivered a normal baby at term, and one year later she had a twin pregnancy that ended successfully and delivered by caesarian section a two babies with different sex. Pregnancy after renal transplantion must be considered as a risk factor for any subsquent pregnancy, and the risk nicreases in case of twin pregnancy.

[Encrusted cystitis]. / La cystite incrustée.

Encrusted cystitis is a chronic inflammation of the bladder mucosa associated with calcified deposits induced by numerous bacteria in an alkaline medium. Corynebacterium urealyticum or group D2 is most frequently incriminated. Encrusted cystitis is a rare disease with non-specific clinical features. The diagnosis may be suspected on imaging, but is often only established later on histological findings. Treatment of this disease is based on appropriate antibiotics with acidification of the urine. The authors report a new case of encrusted cystitis in a 78-year-old woman and review the literature on encrusted lesions of the urinary tract.

[Florid glandular cystitis]. / La cystite glandulaire floride.

INTRODUCTION: Florid glandular cystitis is a rare benign bladder tumour, which can simulate a malignant lesion. MATERIAL AND METHODS: Over a period of fifteen years, we have observed one case of recurrent florid glandular cystitis. RESULTS: This 48-year-old patient presented with terminal haematuria and right low back pain with a history of endoscopic resection of a bladder tumour. Ultrasound showed a solid mass of the bladder base. Intravenous urography showed bilateral uretero-caliceal dilatation, predominant on the right, with a filling defect of the bladder base. CT urography showed invasion of the vesicoprostatic fat and endoscopy revealed a trigonal tumour 5 cm in diameter. Histology concluded on florid glandular cystitis of the intestinal type. Improvement of the symptoms was observed on the fifth postoperative day after resection of the tumour. CONCLUSION: Florid glandular cystitis is a rare benign bladder tumour. The diagnosis is based on clinical examination, radiology and histological examination. Treatment is essentially surgical.

[Leiomyoma of the urinary bladder]. / Le léiomyome de la vessie.

Benign mesenchymal tumors of the bladder are rare. Leiomyoma is the most common mesenchymal tumor of the bladder. We report a new case. A 50-year-old woman presented with total hematuria. Complementary investigations (ultrasonographic, intravenous urography) showed an intravesical solid mass, arising from the bladder dome. Cystoscopic examination suggested a benign tumor, which was confirmed by pathologic assessment of biopsy. Partial cystectomy was performed. Postoperative course was uneventful. Pathologic examination showed a leiomyoma. Leiomyoma of the urinary bladder is a rare benign tumor. Its prognosis is good, the treatment being exclusively surgical.