Reversible Posterior Leukoencephalopathy Syndrome Developing After Restart of Sunitinib Therapy for Metastatic Renal Cell Carcinoma

A 64-year-old Japanese man had started molecular-targeted therapy with sunitinib for lymph node metastasis 5 years after nephrectomy for left renal cell carcinoma (clear cell carcinoma, G2, pT2N0M0). He was transported to our emergency department because of generalized tonic-clonic seizure, vision loss, and impaired consciousness with acute hypertension after 8 cycles of treatment (2 years after the initiation of sunitinib therapy, including a drug withdrawal period for one year). MRI of the brain (FLAIR images) showed multiple high-intensity lesions in the white matter of the occipital and cerebellar lobes, dorsal brain stem, and left thalamus. Reversible posterior leukoencephalopathy syndrome caused by sunitinib was suspected. In addition to the immediate discontinuation of sunitinib therapy, the administration of antihypertensive agents and anticonvulsants improved the clinical symptoms without neurological damage. Physicians should be aware that sunitinib causes reversible posterior leukoencephalopathy syndrome. The early recognition of reversible posterior leukoencephalopathy syndrome is critical to avoid irreversible neurological damage

RETROPERITONEAL FIBROSARCOMA WITH DISTINCTIVE CHARACTERISTICS ON GROWTH P ATTERN AND FINDINGS IN DIAGNOSTIC IMAGING

A 47-year-old man with lumbago had been found to have a perirenal hematoma at the lower pole of the left kidney on CT. MR imaging also demonstrated a hematoma，8 X8X9αn in size. After 1-month observation，the mass had very rapidly increased in size，suggesting it to be a neoplasm. Consequent surgery resulted in noncurative tumor extirpation，because of invasion to the descending colon，left kidney and surrounding tissues. Histlogically，the tumor consisted of uniform spindle-shaped cells with interlacing solid and bundle forms，showing a herring-bone pattern. Immunohistochemical studies finally led to the diagnosis of fibrosarcoma with markedly high malignant potential. Additional therapy was not performed. Rapid local spread of the tumor was demonstrated when bowel obstruction developed 2 months later. The patient died of the disease 3 months after surgery. No distant metastasis was observed throughout the clinical course

Novel missense mutation in the FH gene in familial renal cell cancer patients lacking cutaneous leiomyomas

BACKGROUND: Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare tumor predisposition syndrome characterized by cutaneous and uterine leiomyomas and papillary type 2 renal cell cancer. Germline mutation of the fumarate hydratase (FH) gene is known to be associated with HLRCC. CASE PRESENTATION: We describe a 64-year-old father and his 39-year-old son with HLRCC who developed papillary type 2 RCCs lacking cutaneous leiomyomas at any site. A common missense mutation in the FH gene, (c.1021G > A, p.D341N) in exon 7, was detected in the 2 cases. Functional prediction with the bioinformatics programs, SIFT and Polyphen-2, reported “damaging (SIFT score 0.00)” and “probably damaging (PSIC score 1.621)” values, respectively. In 162 healthy individuals, there were no cases of a G transition to any base. Finally, (c.1021G > A) in exon 7, was identified as a point mutation. CONCLUSION: We report a family with HLRCC in which a novel missense mutation was detected. A familial papillary type 2 renal cancer should be considered HLRCC unless typical cutaneous leiomyomas do not occur

Preoperative MRI Parameters Predict Urinary Continence after Robot-Assisted Laparoscopic Prostatectomy in Prostatic Cancer Patients

We aimed to investigate whether preoperative MRI findings could predict the bladder neck location on postoperative cystography and recovery of urinary incontinence after robot-assisted laparoscopic radical prostatectomy (RALP). We retrospectively reviewed 270 consecutive patients who had complete preoperative data, including MRI, and underwent postoperative observation for more than three months. Preoperative MRI parameters consisted of the membranous urethral length (MUL) and pubic symphysis-prostate apex length (PAL) on sagittal images. The bladder neck location on a postoperative cystography was defined as the lowest extension of the tapering contrast medium in the bladder, and its relation to the pubic symphysis (above (higher group) and below (lower group) the middle of the pubic symphysis height) was evaluated. Those who required no pad or a safety pad were defined as being continent. PAL was significantly shorter in the higher group than that in the lower group (25.5 vs. 29.1 mm; p < 0.0001). The continent group at three months had a significantly longer MUL and shorter PAL than those in the incontinent group (8.1 vs. 6.7 mm; p < 0.05, and 26.0 vs. 28.1 mm; p < 0.05, respectively). Preoperative MRI parameters could predict the bladder neck location on postoperative cystograms and the recovery of urinary incontinence after RALP

External beam radiation plus concurrent intra-arterial chemotherapy with low dose cisplatin for muscle invasive bladder cancer

Introduction: We aimed to investigate the long-term outcome of trimodality therapy consisting of transurethral resection of bladder tumor, external beam radiation therapy, and concurrent intra-arterial low dose cisplatin for patients with muscle invasive bladder cancer. Materials and Methods: We retrospectively reviewed the medical records of 37 consecutive patients (28 men and 9 women) who underwent trimodality therapy for T2-3N0M0 bladder cancer at our hospital between 1996 and 2011. A total of 60Gy of external beam radiation therapy was administered. A daily low dose of cisplatin was administered intra-arterially through a subcutaneously placed reservoir on the days of radiation therapy. Complete response was defined as no residual cancer in transurethral resection specimens and negative cytology. When a complete response could not be achieved, patients underwent additional intra-arterial chemotherapy. Results: Five-year cause specific, disease free, and overall survival rates were 86.4%, 69.7%, and 69.6%, respectively, with a mean follow-up period of 56.5 ± 6.1 months. Five-year cause specific survivals of the complete response group after the trimodality therapy, the complete response group after additional intra-arterial chemotherapy and the non-complete response group were 100% (n = 21), 85.9% (n = 9) and 0% (n = 7), respectively. Five-year overall survivals of the complete response group after the trimodality therapy, the complete response group after additional intra-arterial chemotherapy and the non-complete response group were 82.8%, 85.3% and 0%, respectively. Conclusions: This trimodality therapy for muscle invasive bladder cancer could achieve favorable survival rates with bladder preservation and minimal adverse events. This trimodality therapy can be one of the useful treatment options

The Preoperative Predictive Factors for Pathological T3a Upstaging of Clinical T1 Renal Cell Carcinoma

We aimed to determine the oncological outcomes of patients with clinical T1 renal cell carcinoma (RCC) upstaged to pathological T3a and to identify the preoperative predictive factors for upstaging. We retrospectively reviewed 272 patients with clinical T1 RCC who underwent surgical treatment. Thirty-three patients (12%) were upstaged to pathological T3a. These patients had a significantly larger tumor size on computed tomography (p < 0.0001), a higher aspartate aminotransferase (AST)/alanine aminotransferase (ALT) ratio (p = 0.037), and an elevated c-reactive protein (CRP) level (p = 0.014) preoperatively compared with those with pathological T1 RCC. On multivariate analysis, tumor diameter was the only significant preoperative predictive factor for upstaging [hazard ratio (HR), 3.61; 95% confidence interval (CI), 1.32–9.84; p = 0.01]. The AST/ALT ratio tended to be a preoperative predictive factor for upstaging, although it was not significant (HR, 2.14; 95% CI, 0.97–4.73; p = 0.06). Pathological T3a upstaging occurred in 25% of those with a tumor diameter ≥30 mm and a preoperative AST/ALT ratio ≥1.1. There was a significant correlation between pathological T3a upstaging and the number of preoperative risk factors (p = 0.0002). The preoperative tumor diameter and serum AST/ALT ratio can be predictive factors for pathological T3a upstaging in patients with clinical T1 RCC