Multiple chromophobe and clear cell renal cancer in a patient affected by Birt-Hogg-Dubè syndrome: a case report.

OBJECTIVES: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant characterized by the presence of fibrofolliculomas and/or trichodiscomas, pulmonary cysts, spontaneous pneumothorax, and renal tumors. The syndrome is linked to mutations in the FLCN gene, which is preferentially expressed in the skin, kidney, and lung. The aim of our paper is to describe a case of multiple bilateral renal cancer in a patient affected by BHDS. CASE PRESENTATION: Patient subjected to enucleoresection seven kidney tumors discovered right after ultrasound performed for other reasons. Definitive histologic examination were as follows: multifocal type chromophobe renal cell carcinoma and clear cell. After 1 month, the patient was readmitted for spontaneous pneumothorax. After about a year, the patient was again subjected to resection of multiple renal tumors left. Histological examination proved that it was multifocal renal cell carcinoma, clear cell varieties. The genome analysis highlighted positive for mutation c. 1379_1380 of FLCN gene, BHDS gene. Currently, the patient is under close follow-up. After 1 year, the chest computed tomography (CT) confirmed the presence of minute air bubbles scattered on both sides. Instead, the abdominal CT was positive for a small round lesion 6 mm exophytic. CONCLUSIONS: The BHDS is a rare syndrome whose management is extremely complex both in terms of oncological and functional. Kidney tumors associated with BHDS usually have a favorable clinical course. Present evidence suggests a close follow-up of the carriers of the genetic mutation patients whether or not they have expressed the lesions of disease given the high rate of recurrence of renal lesions.

[Nephron sparing surgery in renal cell carcinoma: our experience of a 20-year clinical practice]. / Nephron sparing surgery nei tumori del rene: nostra esperienza relativa a 20 anni di pratica clinica.

OBJECTIVES: Nephron sparing surgery (NSS) is now considered the standard of care in the treatment of renal cell carcinoma (RCC) in stage T1. We retrospectively evaluated our results related to the use of NSS in over twenty years of clinical practice. METHODS: We reviewed our database relating to the use of NSS in the last twenty years of clinical practice, from 1988 to July 2012, in 549 patients. The pre- and post-operative parameters recorded are the evaluation of the site and size of the renal lesion obtained from radiological investigations, the need for clamping the renal pedicle, open or laparoscopic surgical approach, blood loss, histology and intra- and postoperative complications. We also evaluated the parameters related to renal function before and after surgery. RESULTS: The mean follow-up was 95 months (7.6 years). The average diameter of the lesion at CT abdomen was 4.8 cm (1-8 cm). The warm ischemia was required in 317 patients, cold in 18 patients, no need for ischemia in 214 patients. The total duration of surgery was 122.56 ± 52.76 min. 15 procedures were performed laparoscopically. Ischemia time: 3'-25'; bleeding: 50-1000 cc. The lesion was benign in 115 of the 549 patients enrolled; it was a RCC in the remaining cases except for three, which were papillary carcinomas. At 5 years, the cancer free survival rate was 97.5%. CONCLUSIONS: Our data show that the implementation of NSS offers long-term benefits in terms of functional results and a good cancer control.

[A renal cell carcinoma with metastasis to the urinary bladder]. / Carcinoma a cellule renali (RCC) con metastasi vescicali.

We report the case of a renal cell carcinoma with solitary metastasis to the urinary bladder, occurring 3 years after radical nephrectomy in a 68-year-old patient. The cystoscopy revealed a solid and rounded bladder lesion with a fine footstalk. Transurethral resection was performed and the pathological diagnosis was of eosinophil cell and clear cell carcinoma. The patient also presented secondary lesions in under- and upper-diaphragmatic lymph node area, brain and lung; therefore, he received treatment with several systemic therapies (Sorafenib, Sutent, Everolimus, IFN-alpha, Oxaliplatin and Gemcitabine).

[The look into the look: how patients' suffering is perceived by health workers]. / Lo sguardo nello sguardo: la sofferenza del paziente nel vissuto dell'operatore.

The literature about the surgical treatment of the urologic patient shows an increased attention to the outcome measures related to the quality of life and satisfaction of the treated subject. In this context, a crucial position must be attributed to the quality of the relationships among all the subjects involved in the therapeutic process, and especially among caregivers and patients. Cognitive and emotional dimensions play an important role at this regard, to which a spread sensitivity as for the "local culture" of care must correspond. The aim of this study is to identify the health workers' perception and representation of the cognitive and emotional needs of the patients and their relationship with the organizational complexity of the department. The study was implemented in the Urology Department of the Ospedali Riuniti in Bergamo. It involved eleven health workers (two medical doctors and nine nurses). Each of them underwent a semi-structured interview. The anonymous interview transcripts were analyzed using the T-Lab thematic content analysis program. The study highlighted sensitive areas such as: informative dialogue with the patient, reactivity facing the intervention according to gender, the subject's self-adapting process to the clinical environment and the organizational functioning of the department. The health workers' need of new approaches to meet the needs of the subjects under treatment and their family is underlined. Furthermore, the requirement of consistent functional organizational changes to improve the quality of relationships in the process of care is pointed out. The health workers reported both critical situations, efforts, perceived difficulties and important relational resources to face them.

[Treatment of ureteral lesions during gynecologic surgery]. / Il trattamento delle lesioni ureterali in corso di chirurgia ginecologica.

Surgery of gynecologic area and of pelvic cavity in general is a risk situation for ureteral injury. The incidence of these injuries is about 1% and may be considered as "unavoidable", irrespective of the surgical approach which could be either abdominal, vaginal or laparoscopic. In this study, 37 patients who had undergone a previous gynecological surgery were assessed for 38 ureteral units. Ureteral injury was identified at the surgical table in 2 patients who were treated successfully during the same surgical session with an end-to-end anastomosis. In 8 patients, a double-J ureteral stent was placed and the cure was obtained in 7 patients. The single case of failure was converted to surgery. 22 female patients for 23 ureteral units were subjected to open surgery performing 16 ureterocystoneostomies, 6 of them with a combined psoas-bladder hitching. In 1 patient a termino-terminal anastomosis was performed due to an injury in an upper site. Finally, in 3 patients nephrectomy was carried out due to a nonfunctional kidney; 1 patient refused surgery, whereas in another patient a spontaneous canalization of the excretory tract was obtained after reposition of a percutaneous nephrostomy. The minimum follow-up is of at least 1 year. The diagnostic aspects and therapeutic indications are then described with a special emphasis on the so-called prognostic factors which could influence the outcome of the treatment. Finally, the main surgical correction techniques employed in case of leakage of ureteral substance, are reviewed.