Papillary renal cell carcinoma in two young adults with glycogen storage disease type Ia.

Glycogen storage disease type Ia (GSD Ia) is a rare metabolic disease due to glucose-6-phosphatase deficiency. Chronic kidney disease is a frequent complication that may manifest itself by glomerular lesions and tubular dysfunction from the second decade of life. We report two young GSDIa patients with malignant renal tumor. The first patient was a 25-year-old man. He had chronic metabolic imbalance without kidney involvement. The tumor, a type 2 papillary renal carcinoma, was accidentally discovered during follow-up. The second patient was a 27-year-old woman with chronic metabolic imbalance and chronic kidney involvement. The tumor, a grade 2 papillary carcinoma, was accidentally discovered during follow-up. These two observations are, to date, the first to be reported. We suggest that annual monitoring of kidney imaging in GSDI patients should be systematic to detect renal cancer, from the second decade of life.

Conventional versus automated implantation of loose seeds in prostate brachytherapy: analysis of dosimetric and clinical results.

PURPOSE: To review the clinical outcome of I-125 permanent prostate brachytherapy (PPB) for low-risk and intermediate-risk prostate cancer and to compare 2 techniques of loose-seed implantation. METHODS AND MATERIALS: 574 consecutive patients underwent I-125 PPB for low-risk and intermediate-risk prostate cancer between 2000 and 2008. Two successive techniques were used: conventional implantation from 2000 to 2004 and automated implantation (Nucletron, FIRST system) from 2004 to 2008. Dosimetric and biochemical recurrence-free (bNED) survival results were reported and compared for the 2 techniques. Univariate and multivariate analysis researched independent predictors for bNED survival. RESULTS: 419 (73%) and 155 (27%) patients with low-risk and intermediate-risk disease, respectively, were treated (median follow-up time, 69.3 months). The 60-month bNED survival rates were 95.2% and 85.7%, respectively, for patients with low-risk and intermediate-risk disease (P=.04). In univariate analysis, patients treated with automated implantation had worse bNED survival rates than did those treated with conventional implantation (P<.0001). By day 30, patients treated with automated implantation showed lower values of dose delivered to 90% of prostate volume (D90) and volume of prostate receiving 100% of prescribed dose (V100). In multivariate analysis, implantation technique, Gleason score, and V100 on day 30 were independent predictors of recurrence-free status. Grade 3 urethritis and urinary incontinence were observed in 2.6% and 1.6% of the cohort, respectively, with no significant differences between the 2 techniques. No grade 3 proctitis was observed. CONCLUSION: Satisfactory 60-month bNED survival rates (93.1%) and acceptable toxicity (grade 3 urethritis<3%) were achieved by loose-seed implantation. Automated implantation was associated with worse dosimetric and bNED survival outcomes.

Erectile function after permanent 125I prostate brachytherapy for localized prostate cancer.

BACKGROUND AND PURPOSE: To analyze erectile function in men treated by prostate brachytherapy (PB) for localized prostate cancer. MATERIAL AND METHODS: Of a series of 270 sexually active men treated by PB, 241 (89%), mean age 65 yr (range, 43-80 yr), participated in a study on erectile function that was evaluated using the International Index of Erectile Function 5-item (IIEF-5) questionnaire before implantation and by postal survey after a mean follow-up of 36 months (range, 6-70 months). RESULTS: After PB, 27 patients (11%) had no erectile dysfunction (ED), 36 (15%) had mild ED, 58 (24%) had mild to moderate ED, 24 (10%) had moderate ED, 53 (22%) had severe ED and 43 (18%) were not sexually active. In patients with a preimplant IIEF score >12 (cut-off for intercourse with penetration), 73% had a deterioration of erectile function by at least one class after PB. Risk factors for ED after PB were age, preimplant IIEF score and prostate volume. Median time to ED onset was 16 months and was shorter with androgen deprivation (p = 0.007), diabetes (p = 0.03) and age over 55 (p = 0.01). CONCLUSIONS: Following PB, the majority of patients progressively develop or major ED after a free interval that may last several months. SUPPORT: Ligue Nationale contre le Cancer, France.

OBJET: Etude de la fonction érectile chez les hommes traités par curiethérapie pour un cancer localisé de la prostate. MATÉRIELS ET MÉTHODES: A partir de 270 hommes sexuellement actifs, traités par curiethérapie, 241 (89%), moyenne d'âge 65 ans (entre, 43­80 ans), acceptaient de participer à l'étude de la fonction érectile après curiethérapie. Cette étude menée par le questionnaire validé IIEF 5 (International Index of Erectile Function 5-item), évaluait la fonction érectile avant curiethérapie, et en moyenne 36 mois (entre 6-70mois) après la curiethérapie de prostate. L'enquête était faite par envoi postal. RÉSULTATS: Après la curiethérapie, 27 patients (11%) n'avaient pas de dysfonction érectile, 36 (15%) avaient une dysfonction très modérée, 58 (24%) entre très modérée et modérée, 24 (10%) modérée, 53 (22%) avaient une dysfonction érectile sévère et 43 (18%) n'étaient plus sexuellement actifs. Parmi les patients ayant un score IIEF avant curiethérapie >12 (score moyen permettant une pénétration pendant l'acte sexuelle), 73% avait une détérioration de leur fonction érectile d'au moins une classe IIEF. Les facteurs de risque de la dysfonction érectile après curiethérapie étaient: l'âge, le score IIEF avant curiethérapie et le volume de la prostate. La période moyenne pour déclencher une dysfonction érectile après curiethérapie était de 16 mois. Cette période se réduisait lorsque les patients étaient sous hormonothérapie (p = 0.007), avaient du diabète (p = 0.03) et étaient âgés de plus de 55 ans (p = 0.01). CONCLUSIONS: Après curiethérapie, la majorité des patients développaient progressivement une dysfonction érectile plusieurs mois après la curiethérapie. SOUTIEN: Ligue Nationale contre le Cancer, France.

Prospective comparison of T2w-MRI and dynamic-contrast-enhanced MRI, 3D-MR spectroscopic imaging or diffusion-weighted MRI in repeat TRUS-guided biopsies.

OBJECTIVES: To compare T2-weighted MRI and functional MRI techniques in guiding repeat prostate biopsies. METHODS: Sixty-eight patients with a history of negative biopsies, negative digital rectal examination and elevated PSA were imaged before repeat biopsies. Dichotomous criteria were used with visual validation of T2-weighted MRI, dynamic contrast-enhanced MRI and literature-derived cut-offs for 3D-spectroscopy MRI (choline-creatine-to-citrate ratio >0.86) and diffusion-weighted imaging (ADC × 10(3) mm(2)/s < 1.24). For each segment and MRI technique, results were rendered as being suspicious/non-suspicious for malignancy. Sextant biopsies, transition zone biopsies and at least two additional biopsies of suspicious areas were taken. RESULTS: In the peripheral zones, 105/408 segments and in the transition zones 19/136 segments were suspicious according to at least one MRI technique. A total of 28/68 (41.2%) patients were found to have cancer. Diffusion-weighted imaging exhibited the highest positive predictive value (0.52) compared with T2-weighted MRI (0.29), dynamic contrast-enhanced MRI (0.33) and 3D-spectroscopy MRI (0.25). Logistic regression showed the probability of cancer in a segment increasing 12-fold when T2-weighted and diffusion-weighted imaging MRI were both suspicious (63.4%) compared with both being non-suspicious (5.2%). CONCLUSION: The proposed system of analysis and reporting could prove clinically relevant in the decision whether to repeat targeted biopsies.