[A case of complete disappearance of paraneoplestic syndrome after curative resection of bone metastasis in renal cell carcinoma].

A case of renal cell carcinoma presenting with paraneoplastic syndrome is reported. A 69-year-old man with uncontrolled diabetes was incidentally found to have a left thoracic tumor and a right renal tumor. He had intermittent fever of 39℃ or more and laboratory data showed diabetes and high C-reactive protein level. A radical nephrectomy was performed, but the neoplastic syndrome did not improve. After a second surgery consisting of complete resection of solitary bone metastasis the symptoms resolved immediately. At 6 months postoperatively, he had no reoccurence. Generally prognosis of patients with bone metastasis from renal cell carcinoma has been said to be poor, but surgical control of bone metastasis may be a key factor for the prognosis of patient with metastatic RCC in the era of targeted therapy.

[Case of abdominal wall malignant peripheral nerve sheath tumor which is difficult to distinguish from a urachal disease].

Malignant peripheral nerve sheath tumors (MPNST) are highly malignant soft tissue sarcomas. It is very rare for MPNST to arise in the abdominal wall. We report a case of abdominal wall MPNST that was difficult to distinguish from a urachal disease. A 72-year-old woman found a mass of the umbilicus in October 2011. She visited a digestive surgery department in November because it gradually enlarged. Diagnostic imaging suggested a urachal tumor. She was then referred to our clinic. Contrast enhanced CT showed that the 5-cm cystic tumor extended from the umbilicus to abdominal wall. The tumor showed low uptake value in PET-CT. We diagnosed her with a urachal cyst, but could not deny urachal carcinoma. Therefore, we performed surgical resection in January 2012. The pathological diagnosis was MPNST. She has not experienced recurrence for 9 months. MPNST mostly occur in the retroperitoneum close to the spine, extremities, head, and neck. It is very rare for them to occur in the abdominal wall. This is the sixth case including overseas reports. In addition, this is the first case in which it was difficult to distinguish from a urachal disease.

[A case of metastatic renal cell carcinoma associated with Birt-Hogg-Dubé syndrome treated with molecular-targeting agents].

A 56-year-old man was referred to our clinic because of left lumbar pain and a left solitary renal tumor (9. 8 cm in diameter) and bilateral pulmonary metastases detected by computed tomographic scan. Pathologic diagnosis following open radical nephrectomy was papillary renal cell carcinoma, G2, pT2aN0M1. Subsequently, the patient was sequentially treated with interleukin-2 (3 months (mo), progressive disease (PD)), interferon-alpha (3 mo, PD), and oral S-1 as a clinical trial (28 mo, PD). Because of skin fibrofolliculomas, pulmonary cysts, and spontaneous pneumothorax history, Birt-Hogg-Dubé (BHD) syndrome was suspected during the treatment course, despite his having no family history of the disease. Subsequent genetic testing revealed a FLCN germline mutation (c. 1285dupC). He was started on molecular-targeting therapies sequentially, i.e., sorafenib (1 mo, PD), sunitinib (4 mo, PD), and everolimus (7 mo, PD). The patient died of progressive disease at 78 mo from the initial nephrectomy and 30 mo from the start of targeted agents. Loss of FLCN function has been shown to result in the upregulation of the PI3K/mTORC1 pathway in both in vitro experiments and in vivo FLCN knockout mice models. Despite its use as the sixth-line systematic treatment, the mTOR inhibitor everolimus exhibited a relatively long-term effect as compared with the previously used tyrosine kinase inhibitors and in contrast to the results in the RECORD-1 clinical trial. This finding may provide insight into the molecular mechanism of BHDassociated renal tumors.

Ramelteon combined with an α1-blocker decreases nocturia in men with benign prostatic hyperplasia.

BACKGROUND: Nocturia is defined as waking one or more times during the night due to the urge to void. Recently, the effectiveness of several sedatives and analgesics for nocturia has been reported. We herein investigated the effects of ramelteon, an antioxidant and sleep inducer, on nocturia unresponsive to α1-blocker monotherapy in males with lower urinary tract symptoms (LUTS) as a pilot study. METHODS: Subjects were 19 patients who had LUTS suggestive of benign prostate hyperplasia, received α1-blockers (tamsulosin, silodosin, or naftopidil), and continued to have two or more episodes of nocturia per night before starting ramelteon. Ramelteon at 8 mg once daily for one month was added to the α1-blocker. A self-administered questionnaire including the International Prostate Symptom Score (IPSS), quality of life (QoL) index, Overactive Bladder Symptom Score (OABSS), and Nocturia Quality-of-Life Questionnaire (N-QOL) were assessed before and one month after starting ramelteon. RESULTS: The mean score on IPSS question 7 (nocturia) decreased significantly from 2.88 before starting ramelteon to 2.41 one month after starting the medication (P = 0.03). The mean total OABSS decreased significantly from 6.31 to 5.38 (P = 0.03), and the mean for OABSS question 2 (nighttime frequency of nocturia) also significantly decreased from 2.63 to 2.13 (P = 0.01). The mean total N-QOL score did not change significantly. Two patients had dizziness; the remaining patients had no adverse drug-related events. CONCLUSIONS: Ramelteon in combination with an α1-blocker could be a treatment option for reducing nocturia in men with BPH.

Nonfunctioning juxtaglomerular cell tumor.

The juxtaglomerular cell tumor (JGCT) is a rare renal tumor characterized by excessive renin secretion causing intractable hypertension and hypokalemia. However, asymptomatic nonfunctioning JGCT is extremely rare. Here, we report a case of nonfunctioning JGCT in a 31-year-old woman. The patient presented with a left renal tumor without hypertension or hypokalemia. Under a clinical diagnosis of renal cell carcinoma, radical nephrectomy was performed. The tumor was located in the middle portion adjacent to the renal pelvis, measuring 2 cm in size. Pathologically, the tumor was composed of cuboidal cells forming a solid arrangement, immunohistochemically positive for renin. Based on these findings, the tumor was diagnosed as JGCT. In cases with hyperreninism, preoperative diagnosis of JGCT is straightforward but difficult in nonfunctioning case. Generally, JGCT presents a benign biological behavior. Therefore, we should take nonfunctioning JGCT into the differential diagnoses for renal tumors, especially in younger patients to avoid excessive surgery.

[A case of myocardiac metastasis of clear cell renal carcinoma successfully treated with sunitinib].

A 65-year-old male was referred to our clinic from orthopedics because his right gastrocnemius muscle tumor was diagnosed as a metastatic renal cell carcinoma (RCC) with clear cell/spindle cell morphology. He had right nephrectomy for RCC 18 years previously. At 17 months following the first visit to our clinic, brain, lung and right gluteus medius muscle metastases were identified on positron emission tomography computed tomography (PET-CT). The echocardiogram for the evaluation of his heart function incidentally revealed a 3 cm nodular lesion in the right ventricular wall suggesting myocardiac metastasis. He started to take sunitinib at a standard dose of 50 mg/day. A subsequent echocardiogram during the 2nd cycle of sunitinib showed complete disappearance of the myocardiac metastasis. After the 2nd cycle, PET-CT revealed a partial response (PR) for the other metastases. He received 8 cycles of sunitinib. The best response was PR, and progression-free survival was 6 months. After that, he took everolimus for 7 months. Although the myocardiac metastasis continued to show a complete response during the treatment course, he died of progressive lung metastases at 21 months following the administration of sunitinib. Diagnosis of myocardiac metastasis of RCC during the treatment course as well as its successful treatment with molecular targeting agents has rarely been reported.

Presurgical downstaging of vena caval tumor thrombus in advanced clear cell renal cell carcinoma using temsirolimus.

A 63-year-old man presenting with a 7.2-cm right renal mass, an inferior vena cava tumor thrombus, and pulmonary metastases underwent renal mass biopsy that revealed clear cell renal cell carcinoma. Temsirolimus (25 mg weekly) was given because of the extent of the disease and poor performance status, which resulted in a marked reduction in the tumor thrombus (from level III to level I) after 20 weeks of treatment. Subsequently, radical nephrectomy and tumor thrombectomy were carried out. Final pathological analysis confirmed the diagnosis of high-grade clear cell carcinoma (pT4N0M1). One year after initiation of temsirolimus therapy, the patient remained alive despite the presence of disease.

[Malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma of the penis: a case report].

We report the case of a malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma (MFH/UPS) of the penis in a 78-years-old-man who had undergone previous radical prostatectomy, external beam radiation therapy for prostatic adenocarcinoma. The mass was a 9-cm firm lesion at the base of the penis predominantly composed of malignant spindle cells arranged in sweeping fascicles and storiform pattern. The tumor cells stained for vimentin, beta-smooth muscle actin, S-100, and were negative for keratin, desmin, Melan A, PSA. Despite total penectomy, he developed a local reccurence 4 months after surgery, and died from dissemination 6 months after surgery. This is the 8th case of penile MFH/UPS.

Early ultrasonographic evaluation of tumor thrombus level during sunitinib therapy for renal cell carcinoma.

A 72-year-old man presenting with a 14-cm left renal mass, an inferior vena cava (IVC) tumor thrombus, and pulmonary metastases underwent renal mass biopsy that revealed clear cell renal cell carcinoma. Because of metastases and the extent of the tumor thrombus, sunitinib was administered, which resulted in a marked reduction in the tumor thrombus (from level III to level II after 11 weeks of treatment). Ultrasonography, preceding computed tomography, showed a slight shrinkage of the tumor thrombus level in the first 2 weeks. Therefore, ultrasound may be advantageous to monitor the IVC tumor thrombus level during the early phase of targeted therapy.

Comparison of the loss of renal function after cold ischemia open partial nephrectomy, warm ischemia laparoscopic partial nephrectomy and laparoscopic partial nephrectomy using microwave coagulation.

PURPOSE: Nephron sparing surgery is an effective surgical option in patients with renal cell carcinoma. Laparoscopic partial nephrectomy involves clamping and unclamping techniques of the renal vasculature. This study compared the postoperative renal function of partial nephrectomy using an estimation of the glomerular filtration rate (eGFR) for a Japanese population in 3 procedures; open partial nephrectomy in cold ischemia (OPN), laparoscopic partial nephrectomy in warm ischemia (LPN), and microwave coagulation using laparoscopic partial nephrectomy without ischemia (MLPN). MATERIALS AND METHODS: A total of 57 patients underwent partial nephrectomy in Yokohama City University Hospital from July 2002 to July 2008. 18 of these patients underwent OPN, 17 patients received MLPN, and 22 patients had LPN. The renal function evaluation included eGFR, as recommended by The Japanese Society of Nephrology. RESULTS: There was no significant difference between the 3 groups in the reduction of eGFR. eGFR loss in the OPN group was significantly higher in patients that experienced over 20 minutes of ischemia time. eGFR loss in LPN group was significantly higher in patients that experienced over 30 minutes of ischemia time. CONCLUSION: This study showed that all 3 procedures for small renal tumor resection were safe and effective for preserving postoperative renal function.

Laparoscopic nephroureterectomy in renal pelvic urothelial carcinoma with situs inversus totalis: preoperative training using a patient-specific simulator.

OBJECTIVE: To report the first case of preoperative training using a patient-specific laparoscopic simulator for a patient with situs inversus totalis (SIT). MATERIALS AND METHODS: A 61-year-old man presented with gross hematuria and was diagnosed with right renal pelvic cancer T1N0M0. A whole-body computed tomography (CT) scan also revealed his organs showed a mirror image, with left-to-right reversal, and SIT was diagnosed. A laparoscopic right nephroureterectomy through a retroperitoneal approach was scheduled. Before the operation, we practiced the operation using a patient-specific laparoscopic surgical simulator that we have developed. The patient's CT data were used to reproduce the retroperitoneal space in the simulator, and the surgeon trained and became familiar with his SIT anatomy. RESULTS: The insufflation time was 182 minutes, and the estimated blood loss was 50 mL. The surgeon was able to complete the right retroperitoneoscopic nephroureterectomy safely, without any problems. CONCLUSION: To our knowledge, we report the first case of preoperative training using a patient-specific simulator for a patient with SIT. Preoperative training was useful, and retroperitoneoscopic nephroureterectomy was successfully performed in a patient with renal pelvic urothelial carcinoma and SIT.

Preparation for pyeloplasty for ureteropelvic junction obstruction using a patient-specific laparoscopic simulator: a case report.

INTRODUCTION: Training systems for laparoscopic surgery are useful for basic training but are not suitable for specific training corresponding to the condition of a given patient. We, therefore, have developed an unusual training system: a patient-specific simulator for laparoscopic surgery. When specific data of each individual patient are entered, this system helps surgeons perform a "rehearsal" operation. We applied this technique in laparoscopic surgery by using volume data obtained by multislice computed tomography imaging. CASE PRESENTATION: A 39-year-old Japanese woman consulted a doctor because of back pain and underwent pyeloplasty after an examination revealed a ureteropelvic junction obstruction. Computed tomography data showed that the network of arteries and veins was very complicated. Therefore, we decided to use our simulator before performing surgery. Simulation was helpful because we could obtain information about the complicated vessel network and "rehearse" the procedure. CONCLUSIONS: Our simulator allows surgeons to perform a sham operation with different perspectives and tactile sensations and has received favorable reviews from users.

[Clinical comparison between microporous polysaccharide hemispheres (MPH) and fibrin glue].

PURPOSE: Nephron-sparing surgery for small renal tumors has gained acceptance in an attempt to preserve renal function while achieving a level of cancer control equivalent to that obtained by radical nephrectomy. Moreover, laparoscopic partial nephrectomy (LPN) has been applied to partial nephrectomy because of it is less invasive. However, LPN is a technically complex procedure and has more potential for complications than open partial nephrectomy (OPN). Using hemostatic agents is one of the options to avoid complications during LPN. Microporous polysaccharide hemispheres (MPH) are an absorbable hemostatic powder produced from purified potato starch. We compare the efficacy of this new hemostatic agent, MPH and the standard hemostatic agent, fibrin glue. METHODS: Between January 2007 and March 2011, 55 LPNs for suspected malignancy were completed by a single surgeon in Yokohama City University Hospital. We compare two sequential groups of patients: group A consisted of 12 patients in whom MPH was used (age 41-77, mean age 59.7, male: female = 10 : 2) and group B consisted of 43 patients in whom fibrin glue was used (age 22-79, mean age 60.3, male: female = 31 : 12), retrospectively. These agents (MPH and fibrin glue) were applied to the partial nephrectomy bed before tying a suture in parenchymal suturing and after the renal hilum was unclamped. RESULTS: The MPH group showed significantly less mean estimated blood loss (25.6 vs. 86.3 ml; p = 0.036). There was no significant difference in surgical duration, ischemic time or urine leakage. Postoperative complications occurred in two patients in group B, but there were no postoperative complications in group A. CONCLUSIONS: MPH is available as an adequate hemostatic agent during LPN. There was no significant difference in the incidence of postoperative complications between MPH and fibrin glue.

[Adrenocortical carcinoma detected by retroperitoneal hemorrhage : a case report].

Spontaneous massive retroperitoneal hemorrhage from an adrenal tumor is rare and is usually fatal if unrecognized. We report a case of spontaneous rupture of a primary adrenocortical carcinoma that occurred in a 79-year-old man. He visited our hospital with left abdominal pain. Computed tomography (CT) showed a left retroperitoneal hemorrhage. We could not find the origin of this hemorrhage. Two months later, CT showed the left adrenal tumor, and left adrenalectomy and nephrectomy were performed successfully. The histological diagnosis was adrenocortical carcinoma. He rejected adjuvant therapy. Local recurrence of the tumor was found, and right adrenal gland, brain, and mediastinal lymph node metastases were recognized 6 months after the operation. He died 11 months after the operation.

[A case of retroperitoneal angioleiomyoma resected by laparoscopy].

A retroperitoneal angioleiomyoma was incidentally detected in a 56-year-old woman during an examination of cardiovascular disease, and referred to the department of urology. Computed tomography (CT) showed a solid tumor approximately 3 cm in diameter, enhanced heterogeneously adjacent to the right adrenal and renal vein on magnetic resonance imaging the tumor showed a low intensity in the T1-weighted image and high intensity in T2-weighted image. These radiographic findings suggested a retroperitoneal tumor such as paraganglioma, angioma. Furthermore, because she was a carrier of Human Adult T Cell Leukemia Virus-I (HTLV-I) this tumor was suspected to have relevance to malignant lymphoma. We performed laparoscopic surgical excision of the tumor. Pathlogical diagnosis was an angioleiomyoma. Angioleiomyoma is a rare type of leiomyoma originating from smooth muscle and containing thick-walled vessels. Only a few cases of retroperitoneal angioleiomyoma have been reported.

[Renal metastasis from prostatic adenocarcinoma : a case report].

We report a case of renal metastases from prostate cancer to show that the possibility of tumor metastasis, although rare, should be considered in the differential diagnosis of renal mass. A 67-year-old man was found to have a renal mass on computed tomographic scan incidentally. He had had total androgen blockage (bicalutamide + leuprolerin) and chemotherapy (docetaxel hydrate ) for treatment of prostate cancer discovered 33 months ago. On the basis of the clinical features and radiologic results, the patient was thought to have a second malignant tumor, and we performed left nephrectomy. The pathological finding of this case was renal metastasis from prostatic adenocarcinoma. He died 18 months postnephrectomy.

Gene organization of the liverwort Y chromosome reveals distinct sex chromosome evolution in a haploid system.

Y chromosomes are different from other chromosomes because of a lack of recombination. Until now, complete sequence information of Y chromosomes has been available only for some primates, although considerable information is available for other organisms, e.g., several species of Drosophila. Here, we report the gene organization of the Y chromosome in the dioecious liverwort Marchantia polymorpha and provide a detailed view of a Y chromosome in a haploid organism. On the 10-Mb Y chromosome, 64 genes are identified, 14 of which are detected only in the male genome and are expressed in reproductive organs but not in vegetative thalli, suggesting their participation in male reproductive functions. Another 40 genes on the Y chromosome are expressed in thalli and male sexual organs. At least six of these genes have diverged X-linked counterparts that are in turn expressed in thalli and sexual organs in female plants, suggesting that these X- and Y-linked genes have essential cellular functions. These findings indicate that the Y and X chromosomes share the same ancestral autosome and support the prediction that in a haploid organism essential genes on sex chromosomes are more likely to persist than in a diploid organism.