[LEIOMYOSARCOMA ARISING FROM OVARIAN VEIN WITH RIGHT HYDRONEPHROSIS: A CASE REPORT].

The patient was a 37-year-old woman who had suffered from repeated pyelonephritis. Computed tomography (CT) of the abdomen revealed a 1.9 cm retroperitoneal mass with compression of the right ureter and hydronephrosis. The patient visited our medical center and admitted. The patient underwent a simple total excision of the mass and end-to-end ureteral anastomosis. The tumor involved right ovarian vein and right ureter. Histopathological diagnosis was leiomyosarcoma of the ovarian vein. At 12 months after operation, local recurrence of surroundings tissue of the right ureter and gallbladder, and inferior vena cava invasion is found. Thus, the patient underwent a right nephroureterectomy with partly resection of the inferior vena cava and en block excision of the gallbladder. While CT revealed no recurrence three months after the operation, adjuvant postoperative combination chemotherapy with gemcitabine and docetaxel was administered. Nine cases of this leiomyosarcoma arising from ovarian vein have been reported in the literature. Leiomyosarcoma arising from ovarian vein with hydronephrosis is a second example.

Clinical utility of the prostate cancer gene 3 (PCA3) urine assay in Japanese men undergoing prostate biopsy.

UNLABELLED: WHAT'S KNOWN ON THE SUBJECT? AND WHAT DOES THE STUDY ADD?: It is known that a prostate cancer gene 3 (PCA3) urine assay is superior to serum PSA level or PSA-related indices for predicting a positive biopsy result in European and US men. This is the first report on PCA3 in a large cohort of Japanese men. The diagnostic value of the PCA3 score in Japanese men was similar to those reported in European and US men. The study concludes that a combination of PSA density and PCA3 score may be useful for selecting patients who could avoid an unnecessary biopsy. OBJECTIVE: To examine the diagnostic performance of the prostate cancer gene 3 (PCA3) score for prostate cancer in Japanese men undergoing prostate biopsy. PATIENTS AND METHODS: This Japanese, multicentre study included 647 Asian men who underwent extended prostate biopsy with elevated prostate-specific antigen (PSA) and/or abnormal digital rectal examination (DRE). Urine samples were collected after DRE. The PCA3 score was determined using a PROGENSA PCA3 assay and correlated with biopsy outcome. Its diagnostic accuracy was compared with that of serum PSA level, prostate volume (PV), PSA density (PSAD), and free/total PSA ratio (f/t PSA). RESULTS: A total of 633 urine samples were successfully analysed (the informative rate was 98%). Median PSA was 7.6 ng/mL. Biopsy revealed cancer in 264 men (41.7%). The PCA3 score for men with prostate cancer was significantly higher than that for men with negative biopsies (median PCA3 score: 49 vs. 18; P < 0.001). The rate of positive biopsy was 16.0% in men with a PCA3 score of <20 and 60.6% in those with a PCA3 score of ≥50. Using a PCA3 score threshold of 35, sensitivity and specificity were 66.5 and 71.6%, respectively. The area under the curve of the PCA3 score was significantly higher than that of the f/t PSA in men with PSA 4-10 ng/mL (0.742 vs 0.647; P < 0.05). In men with PSAD < 0.15 and PCA3 < 20, only three (4.2%) out of 72 men had prostate cancer. CONCLUSIONS: The PCA3 score was significantly superior to f/t PSA in predicting a positive biopsy result for prostate cancer in Japanese men with PSA 4-10 ng/mL. The combination of PSAD and PCA3 score may be useful for selecting patients who could avoid an unnecessary biopsy.

Maintenance intravesical bacillus Calmette-Guérin instillation for Ta, T1 cancer and carcinoma in situ of the bladder: randomized controlled trial by the BCG Tokyo Strain Study Group.

OBJECTIVES: We carried out a prospective, randomized, controlled trial to investigate the efficacy and safety of both induction and maintenance therapy with intravesical instillation of bacillus Calmette-Guérin (BCG) for high-risk non-muscle invasive bladder cancer (NMIBC). METHODS: Intravesical instillation of 80 mg Tokyo strain was given to patients with high-risk NMIBC, including carcinoma in situ (CIS), once weekly for eight consecutive weeks as induction therapy. Patients who achieved complete response (CR) were randomly assigned to either the maintenance group or the observation group. RESULTS: A total of 90 patients were enrolled. After induction therapy, 75% of the patients achieved CR and 53 of them were enrolled in the randomized comparative phase. A total of four maintenance instillations were given. Median follow-up was 26.5 and 28.7 months after randomization in the maintenance and the observation group, respectively. Although it was not statistically significant, the 2-year recurrence-free survival rate in the maintenance group (95.8%) was higher than that in the observation group (74.1%, P = 0.078). Univariate analysis identified maintenance therapy as a significant factor influencing recurrence. During induction therapy, 82.2% of patients experienced urination-related adverse drug reactions, but most events were not serious. There were fewer adverse drug reactions with maintenance therapy than with induction therapy. Neither induction therapy nor maintenance therapy reduced patients' quality of life (QOL). CONCLUSIONS: These findings show high levels of efficacy and safety of BCG induction treatment for high-risk NMIBC, and suggest that the number of maintenance instillations could probably be reduced without reducing treatment efficacy or influencing QOL.

Conversion to Silodosin in Men on Conventional α1 -Blockers for Symptomatic Benign Prostatic Hyperplasia.

OBJECTIVES: α1 -blockers have commonly been used as first-line medical therapy for symptomatic benign prostatic hyperplasia (BPH). Recently, a highly selective α1A -adrenoceptor antagonist, silodosin, was developed in Japan. We examined the efficacy and safety of conversion from conventional α1 -blockers to silodosin in men with BPH. METHODS: Conversion to silodosin was proposed to consecutive patients on conventional α1 -blockers for symptomatic BPH for at least 6 months. The effects of conversion were examined by the International Prostate Symptom Score, quality of life index, overactive bladder symptom score, peak flow rate, residual urine volume, and adverse events at 12 weeks. The efficacy of silodosin was also evaluated by patients' impression. RESULTS: Eighty-one men underwent conversion, for the most part because of dissatisfaction with the efficacy of their current treatment in improving nocturia or weak stream. The International Prostate Symptom Score total score significantly improved from 12.7 ± 5.9 at baseline to 10.6 ± 5.4 at 4 weeks (P < 0.001) and 10.9 ± 5.8 at 12 weeks (P < 0.01). The progress was mostly due to improvement in voiding symptoms, although reduction of storage symptoms was also significant. The quality of life index also significantly decreased with conversion to silodosin. Efficacy as judged by patients' impression was 76% (37/49) at 12 weeks of treatment. None of the overactive bladder symptom score, peak flow rate, and residual urine volume exhibited significant change. No serious adverse events were observed during the study period. CONCLUSION: Conversion to silodosin may be beneficial in men who are dissatisfied with conventional α1 -blockers for BPH, and be particularly useful in improving voiding symptoms.

Salvage combination chemotherapy with docetaxel, ifosfamide and cisplatin (DIP): successful treatment of a case with metastatic testicular immature teratoma.

We present a case of metastatic testicular immature teratoma that was successfully treated despite resistance to standard chemotherapy and unsuccessful salvage surgery. At first, BEP (bleomycin, etoposide and cisplatin) treatment was performed but failed. The patient underwent incomplete retroperitoneal lymph node dissection. He was then referred to us. By the time of the referral lung and mediastinal lymph node metastasis had appeared and para-aortic lymph node metastasis had grown larger. We administered the DIP (docetaxel, ifosfamide and cisplatin) regimen as a second line chemotherapy, which was effective with 82% reduction of para-aortic lymph nodes, 88% of mediastinal lymph nodes and 85% of lung metastasis. We performed para-aortic lymph node dissection followed by resection of lung metastasis and mediastinal lymph node dissection. The patient is now followed-up at the outpatient clinic without evidence of recurrent disease 3.5 years after the last surgery.

Identification of Toll-like receptor 3 as a potential therapeutic target in clear cell renal cell carcinoma.

PURPOSE: Renal cell carcinoma (RCC) is one of the most drug-refractory cancers. The aim of this study is to discover a novel therapeutic target molecule for clear cell RCC (CCRCC), which accounts for the majority of RCC. EXPERIMENTAL DESIGN: Gene expression profiles of 27 CCRCCs and 9 normal kidney tissues as well as 15 various adult normal tissues were examined by Affymetrix U133 Plus 2.0 arrays. Among the 34 genes specifically up-regulated in CCRCC, overexpression of Toll-like receptor 3 (TLR3) mRNA and its protein was validated by quantitative reverse transcription-PCR, immunoblot, and immunohistochemistry. The effects of TLR3 signaling on in vitro cell growth were examined. RESULTS: TLR3 gene was highly expressed in CCRCC, with only limited expression in a panel of normal tissues. On immunohistochemical analysis using a monoclonal antibody against TLR3, overexpression of TLR3 was observed in 139 of 189 (73.5%) cases of CCRCC as well as in lung metastatic CCRCC (6 of 8), whereas TLR3 expression was entirely absent in chromophobe RCC (0 of 8). Polyinosinic-polycytidilic acid, a TLR3 ligand, exerted a growth-inhibitory effect against RCC cells in a TLR3-dependent manner. Moreover, a combination of polyinosinic-polycytidilic acid and IFNalpha exerted a synergistic growth-inhibitory effect against Caki-1 RCC cells. CONCLUSIONS: This is the first report that TLR3 is overexpressed in CCRCC. These observations suggest that TLR3 pathway may represent a novel therapeutic target in CCRCC.

The prognostic value of E-cadherin, alpha-, beta- and gamma-catenin in bladder cancer patients who underwent radical cystectomy.

OBJECTIVES: To determine the value of the loss of expression of E-cadherin and cadherin associated molecules as useful markers for both prognosis and chemosensitivity in bladder cancer patients who have undergone radical cystectomy. PATIENTS AND METHODS: In 55 paraffin embedded specimens of radical cystectomy at our hospital from 1982 to 2000, the expression of E-cadherin, alpha-, beta- and gamma-catenin was examined by immunohistochemical staining. To evaluate the prognostic significance of these molecules, Kaplan-Meier survival curves were constructed and a statistical analysis was calculated by a log-rank test. A multivariate test (tumor stage, tumor grade, lymph node metastasis, configuration, the expression of E-cadherin, alpha-, beta- and gamma-catenin) was performed to detect prognostic markers. RESULTS: Normal expression was found in 33 cases (60.0%) for E-cadherin, 29 (52.7%) for alpha-catenin, 31 cases (56.4%) for beta-catenin, and 31 cases (56.4%) for gamma-catenin. The expression patterns for E-cadherin, alpha-, beta- and gamma-catenin were significantly correlated with each other (P < 0.01). Survival analysis showed a significant difference between normal and aberrant expression in each staining. A multivariate analysis revealed that the expression of alpha- catenin was an independent prognostic factor (P = 0.0191). In 23 patients that received adjuvant chemotherapy, there was a significant difference in survival between the normal and aberrant expression of alpha-catenin, but not other molecules. CONCLUSION: Alpha-catenin may not only be a good prognostic marker, but also one of key molecules that determine the chemosensitivities in patients with invasive bladder cancer.

[Giant renal angiomyolipoma in a case with pulmonary lymphangiomyomatosis].

A 27-year-old female with pulmonary lymphangioleiomyomatosis was referred to us because of the right large renal angiomyolipoma (AML), 10 cm in diameter, which was found by abdominal CT during close investigation of her pulmonary disease. About 6 months earlier she had received operation for pneumothorax and the histological examination disclosed pulmonary lymphangioleiomyomatosis (LAM). Clinically she does not have tuberous sclerosis. Partial nephrectomy was performed. The renal tumor arose from the lower part of the kidney with somewhat exophytic growth. She is now followed at outpatient clinic without recurrence of renal AML. Pulmonary LAM often associates with renal AML. Although pulmonary LAM is a progressive disease and has a poor prognosis, because of the risk of the rupture of renal AML, large renal AMLs should be treated. Recently several cases have been reported for whom nephron-sparing surgery was performed. This case is 10th report of LAM, which is associated with AML in Japan.

[Bladder recurrence of renal cell carcinoma: report of two cases].

We present two rare cases with renal cell carcinoma which recurred at urinary bladder. The first case is an 87-year-old female who had been undertaken right nephrectomy 17 years earlier. Through the examination of hypercalcemia, bone metastases and a bladder tumor were found. Transurethral resection of the bladder tumor was performed and histological examination revealed that the tumor was clear cell carcinoma similar to the right renal tumor. The second case is a 67-year-old male who had been undertaken left nephrectomy 4 and half years earlier. In spite of the adjuvant immunotherapy including interferon alpha, gamma and interleukin-2, 16 months later multiple lung metastases appeared. One month before admission he noticed asymptomatic hematuria. Cystoscopy revealed 3 non-papillary pedunculated tumors, which were resected transurethrally. Histologically the tumor was clear cell carcinoma similar to the left renal tumor. In both cases the metastasis was confirmed histologically. As in both cases the recurrent bladder cancers were confined in the mucosa, these metastases were thought to be caused by implantation.

Immunotherapy against metastatic renal cell carcinoma with mature dendritic cells.

OBJECTIVE: We performed a clinical trial of immunotherapy using autologous mature dendritic cells (DC) pulsed with autologous tumor lysate, for patients with metastatic renal cell carcinoma (RCC). METHODS: Patients with refractory metastatic RCC were enrolled in the study. All of them received interferon (IFN)-alpha treatment after nephrectomy and were followed over 3 months prior to this study. Autologous monocyte-derived immature DC were pulsed with lysate from autologous primary tumor as the antigen and keyhole limpet hemocyanin (KLH) as immunomodulator, and cultured in the presence of tumor necrosis factor (TNF)-alpha, interleukin (IL)-1beta, and prostaglandin (PG)E2 to generate mature DC. Mature DC were injected intradermally near bilateral inguinal lymph nodes of the patients. A delayed-type hypersensitivity (DTH) test and enzyme-linked immunospot (ELISPOT) assay were performed to evaluate the immunological response. After 4 months from first injection, the clinical effect was evaluated by diagnostic imaging. RESULTS: The treatments were well tolerated without significant toxicity by the patients who were an average of 65.7 years old and had multiple metastases in the lung and other organs. One of the two patients developed a positive DTH reaction to tumor lysate and the other patient only to KLH. The patient with a positive DTH reaction to tumor lysate had stable disease in the clinical evaluation. CONCLUSIONS: We confirmed the safety of DC therapy in this clinical trial. The DTH test revealed that the DC therapy induced immunological response to RCC. On the other hand, it was necessary to reconsider the patient selection criteria.

Recurrent prostatic stromal sarcoma with massive high-grade prostatic intraepithelial neoplasia.

A unique case of prostatic stromal sarcoma (PSS) that recurred in the pelvic cavity with massive high-grade prostatic intraepithelial neoplasia is described. A 52-year-old man who presented with urinary retention underwent a radical cystoprostatectomy. Tumour tissues of the prostate showed an admixture of hyperplastic glands and markedly cellular stroma of spindle cells arranged in a fascicular pattern, and the tumour was diagnosed as PSS. 66 months after the operation, CT scans revealed three recurrent tumours around the bilateral obturator and left fore iliopsoas. The recurrent tumours were biphasic neoplasms, as before, but the epithelial component had grown prominent and manifested overt atypia in a manner resembling high-grade prostatic intraepithelial neoplasia. Our findings suggest that not only the stromal component but also and the epithelial components of PSS may have malignant potential.

Chronic acid ingestion promotes renal stone formation in rats treated with vitamin D3.

OBJECTIVE: Although hypercalciuria, a well-established adverse effect of vitamin D3, can be a risk factor of renal stone formation, the risk of nephrolithiasis has not been well defined. The consumption of a diet high in acid precursors is often cited as a risk factor for the development of calcium-based kidney stones. In the present study, we investigated the effect of chronic acid ingestion on kidney stone formation in rats treated with calcitriol (1-25[OH]2 D3). METHODS: Control rats (C-C), calcitriol-treated rats (C-V; three treatments of 0.5 microg of calcitriol per week) and acid-ingested (water containing 0.21 mol/L NH4Cl), calcitriol-treated (three treatments of 0.5 microg of calcitriol per week) rats (A-V) were fed in metabolic cages. After 1 month, urine, blood, kidney and bone samples were analyzed. RESULTS: The A-V rats exhibited elevated serum calcium concentrations, urinary calcium and phosphate excretion, urinary type I collagen cross-linked N-peptide (NTx)/creatinine values, mRNA expression of osteopontin in the kidney, and renal calcium contents as well as decreased bone mineral densities, compared with the C-C and C-V rats. Urinary citrate excretion was lower and NaDC-1 mRNA expression in the kidney was higher in the A-V rats than in the C-C and C-V rats. Calcium phosphate kidney stones were found in the A-V rats. CONCLUSIONS: The ingestion of NH4Cl, an acid precursor, promotes calcium phosphate kidney stone formation in calcitriol-treated rats. The chronic intake of a diet rich in acid precursors may be a risk factor for the development of kidney stones in subjects who are being treated with calcitriol.

Predictors of seminal vesicle invasion before radical prostatectomy.

AIM: To predict whether or not seminal vesicle invasion is present before radical prostatectomy, the relationships between clinical parameters and seminal vesicle invasion were analyzed. METHODS: A review was conducted of 187 patients who had been clinically diagnosed with stages A(2), B(0), B(1), B(2) or C prostate cancer and who had undergone radical prostatectomy without neoadjuvant therapy. The parameters analyzed for potential predictors of seminal vesicle invasion before radical prostatectomy included age, clinical stage, serum prostate-specific antigen (PSA) level at biopsy, tumor differentiation of biopsy specimens and percentage of cancer positive cores by biopsy. For percentage of cancer positive cores by biopsy, 143 of 187 patients who underwent transrectal sextant biopsy or more than six transrectal ultrasound guided core biopsies were evaluated. These parameters were subjected to univariate and multivariate logistic regression analyses to identify predictors for seminal vesicle invasion. RESULTS: The median age was 66.8 years (range 51-77 years). Of 187 patients, 27 (14.4%) had seminal vesicle invasion confirmed pathologically. There were significant differences in all parameters except for age between patients with positive and negative seminal vesicle invasion on univariate analysis. Multivariate analysis revealed that serum PSA level, tumor differentiation of biopsy specimens and percentage of cancer positive cores were significant independent predictors of seminal vesicle invasion. CONCLUSIONS: The results showed serum PSA level, tumor differentiation of biopsy specimens and percentage of cancer positive cores by biopsy before radical prostatectomy may be useful predictors for seminal vesicle invasion.

Irinotecan as a new agent for urachal cancer.

The urachal carcinoma, in a 64-year-old male with multiple lung metastases, had shown the resistance to several anti-neoplastic agents including cisplatinum, methotrexate, 5-FU, doxorubicin, epirubicin, and mitomycin C. Because the tumor was adenocarcinoma producing mucin and serum carcinoembryonic antigen (CEA) increased, which resembled colorectal carcinoma, we administrated Irinotecan, which was very effective as the CEA decreased from 98.3 to 38.7 ng/ml and the pulmonary metastatic lesions were reduced by 60%. To our knowledge, this is the first case with urachal carcinoma in which Irinotecan was effective.

Bladder recurrence of upper urinary tract cancer after laparoscopic surgery.

PURPOSE: We reviewed our cases to determine whether laparoscopic nephroureterectomy is a risk factor for the bladder recurrence. MATERIALS AND METHODS: From 1996 to 2003, 65 nephroureterectomies were performed: 47 by open and 18 by laparoscopic surgery. In 43 (28 by open, 15 by laparoscopic surgery), bladder cancer was not observed at the time of the operation. Two laparoscopic operations were converted to open surgery because of technical problems. The other 13 with laparoscopic and 28 with open surgery were enrolled into this study. RESULTS: Significantly higher recurrence rate was observed in laparoscopic cases (69.2%) than that in open cases (35.7%, P = 0.0484) by log rank test. However, the operation time required in laparoscopic surgery (371.5 +/- 90.8 min) was significantly longer than that in the open surgery (229.9 +/- 46.6 min, P < 0.0001). In multivariate analysis (Cox proportional hazards model), only the longer operation time (>250 min) was a significant variable (P = 0.0305), and laparoscopic surgery in itself was not a significant risk factor (P = 0.5011). CONCLUSIONS: Although frequent bladder recurrence was observed in laparoscopic cases, the most important risk factor was the longer operation time. Technical improvements including shortening of operation time and earlier ureteral ligation may decrease the bladder recurrence.

Phyllodes tumor of the prostate with exuberant glandular hyperplasia.

Reported herein is an unusual case of prostatic phyllodes tumor with exuberant glandular hyperplasia that led to misdiagnosis of adenocarcinoma. The tumor was detected in a 52-year-old man who had a 1 year history of dysuria. Adenocarcinoma of the prostate was diagnosed from a needle biopsy specimen. The patient received hormonal therapy for 6 months and underwent radical prostatectomy. Histologically, the tumor had an atypical stromal cell proliferation and elongated slit-like glands characteristic of a phyllodes tumor. The tumor was also accompanied by a florid proliferation of small acini, most of which lacked basal cells, a common manifestation of adenocarcinoma in the overall tumor area. The following features of the resected tumor were helpful for concluding that these acini were benign: lack of cytological anaplasia in spite of structural atypia, presence of scattered basal cells confirmed by immunohistochemistry (high-molecular-weight cytokeratin), and histological transition from these acini to apparently benign slit-like glands. The final diagnosis was then made as 'phyllodes tumor of the prostate with exuberant glandular hyperplasia'. Atypical stromal cells might provide a clue for the recognition of this rare tumor at initial diagnosis by needle biopsy.

The prognostic value of E-cadherin, alpha-, beta-, and gamma-catenin in urothelial cancer of the upper urinary tract.

OBJECTIVES: To determine the value of loss of the expression of E-cadherin and cadherin-associated molecules as useful markers for both prognosis and bladder recurrence in patients with upper urinary tract cancer. MATERIALS AND METHODS: In 61 paraffin-embedded nephroureterectomy specimens, the expression of E-cadherin and alpha-, beta-, and gamma-catenin was examined by immunohistochemical staining. To evaluate the prognostic significance, Kaplan-Meier survival curves were calculated and compared by the log-rank test. A multivariate test was performed to detect prognostic markers. RESULTS: Normal expression was found in 32 cases (52.5%) for E-cadherin, 41 cases (67.2%) for alpha-catenin, 42 cases (68.9%) for beta-catenin, and 31 cases (50.8%) for gamma-catenin. The expression patterns of E-cadherin and alpha-, beta- and gamma-catenin were significantly correlated with each other. Survival analysis showed a significant difference between normal and aberrant expression in each staining. Multivariate analysis revealed that tumor stage and the expression of E-cadherin were independent prognostic factors for cause-specific survival. In contrast, there was no significant correlation between the expression of E-cadherin and alpha-, beta-, and gamma-catenin and bladder recurrence. CONCLUSION: Our data suggest E-cadherin may be a good prognostic marker for patients with upper urinary tract cancer.

Promoter hypermethylation of the potential tumor suppressor DAL-1/4.1B gene in renal clear cell carcinoma.

Renal clear cell carcinoma (RCCC) is a malignant tumor with poor prognosis caused by the high incidence of metastasis to distal organs. Although metastatic RCCC cells frequently show aberrant cytoskeletal organization, the underlying mechanism has not been elucidated. DAL-1/4.1B is an actin-binding protein implicated in the cytoskeleton-associated processes, while its inactivation is frequently observed in lung and breast cancers and meningiomas, suggesting that 4.1B is a potential tumor suppressor. We studied a possible involvement of 4.1B in RCCCs and evaluated it as a clinical indicator. 4.1B protein was detected in the proximal convoluted tubules of human kidney, the presumed cell of origin of RCCC. On the other hand, loss or marked reduction of its expression was observed in 10 of 19 (53%) renal cell carcinoma (RCC) cells and 12 of 19 (63%) surgically resected RCCC by reverse transcription-PCR. Bisulfite sequencing or bisulfite SSCP analyses revealed that the 4.1B promoter was methylated in 9 of 19 (47%) RCC cells and 25 of 55 (45%) surgically resected RCCC, and inversely correlated with 4.1B expression (p < 0.0001). Aberrant methylation appeared to be a relatively early event because more than 40% of the tumors with pT1a showed hypermethylation. Furthermore, 4.1B methylation correlated with a nuclear grade (p = 0.017) and a recurrence-free survival (p = 0.0036) and provided an independent prognostic factor (p = 0.038, relative risk 10.5). These results indicate that the promoter methylation of the 4.1B is one of the most frequent epigenetic alterations in RCCC and could predict the metastatic recurrence of the surgically resected RCCC.

Proteus syndrome with giant myelolipoma in the pelvis.

Proteus syndrome is a very rare and complex disorder with malformations and overgrowth of multiple tissues. This disorder was designated Proteus syndrome by Wiedemann et al. to denote its variable clinical expression. Our patient presented with macrodactyly, cerebriform appearance and a huge abdominal mass. A biopsy under laparotomy was performed, and histopathological examination revealed myelolipoma. Tumor resection was performed. To our knowledge, no case of Proteus syndrome presenting a myelolipoma in the retroperitoneal cavity has been reported before.

Xanthoma of the urinary bladder.

A 55-year-old male presented with asymptomatic macroscopic hematuria. A diagnosis of xanthoma of the urinary bladder was made histologically. Xanthoma of the urinary bladder is a rare condition. Its etiology, diagnosis and treatment are discussed.