Elevated basal serum levels of calcitonin and simultaneous surgery of MEN2A-specific tumors.

Multiple endocrine neoplasia type 2A (MEN2A) is a rare syndrome caused almost by germline RET mutation, and characterized by medullary thyroid carcinoma (MTC), in combination or not with pheochromocytoma (PHEO), hyperparathyroidism (HPTH), cutaneous lichen amyloidosis (CLA), and Hirschsprung's disease (HD). The basal serum calcitonin (Ctn)/carcinoembryonic antigen (CEA) levels are significantly correlated with the MTC stage. Metachronous surgery of MEN2A-specific tumors is a routine procedure. We aimed to explore the clinical significance of pro-gastrin-releasing peptide (proGRP) in MTC with elevated Ctn and simultaneous surgery of MEN2A-specific tumors. We retrospectively investigated 8 RET mutation carriers of 2 Chinese pedigrees with MEN2A. Clinical profiles, imaging examinations, preoperative and postoperative biochemical data, surgical procedures, and follow-up records were evaluated. Three patients showed levels of elevated Ctn but normal proGRP. Among them, one patient (FAIII-6) in Family A (one for RET C634R mutation), diagnosed with bilateral MTC, left PHEO, bilateral HPTH, and CLA, classified as MEN2A-related CLA subtype, underwent successfully simultaneous adrenal-sparing surgery (ASS), total thyroidectomy (TT), and parathyroidectomy, while TT of the other two patients (FBII-3 and FBIII-7) diagnosed with bilateral MTC in Family B (all for RET C618R mutation) were performed. Unexpectedly, the absence of neck lymph node MTC metastasis was indicated by histopathological examination. Postoperatively, all had consistently "undetectable" or normal levels of Ctn/CEA during follow-up. Patients with normal proGRP, despite high levels of Ctn, might have no regional lymph node MTC metastasis, and neck dissection should be avoided. Moreover, simultaneous surgery for coexistent PHEO and either MTC or HPTH is an approach of choice to use as an alternative treatment pattern. Recognition of MEN2A-related CLA and subsequently early screening of RET mutation may be favorable for timely management of MEN2A-specific tumors.

Types of Renal Calculi and Management Regimen for Chinese Minimally Invasive Percutaneous Nephrolithotomy.

Strict selection of patients for minimally invasive percutaneous nephrolithotomy could effectively improve the success rate of surgery. This study aimed to understand the required skills and the efficacy of mini-PCNL in the treatment of five types of upper ureteral calculi. Data collected after X-ray analysis and B mode ultrasound from 633 patients with upper ureteral and renal pelvis calculi who underwent B ultrasound-guided lithotomy was reviewed, including the following: type I, upper ureteral or renal pelvis calculi with moderate hydronephrosis (154 cases); type II, upper ureteral or renal pelvis calculi with severe hydronephrosis (157 cases); type III, upper ureteral or renal pelvis calculi without hydronephrosis (61 cases); type IV, renal pelvis calculi, one or two renal calyx calculi (206 cases); and type V, renal staghorn calculi (55 cases). Operations on 611 cases were successful. The treatment method for five patients was converted to open surgery. Twelve cases were treated by indwelling double-J tube retro-catheterization and extracorporeal shock wave lithotripsy. Five patients gave up the treatment. The rate of calculus clearance was 82.3 %, and the rate of residual calculus was 17.6 %. Selective renal artery embolization was performed in nine cases. Hydropneumothorax occurred in nine cases. No intestinal fistula occurred, and no patient had to undergo nephrectomy. The difficulty and the curative effect of the operation were different because the types of calculi varied. Selection of the procedure based on the different types of calculi could effectively improve the success rate of the procedure, reduce complications, and shorten the learning curve.

Erratum to: Types of Renal Calculi and Management Regimen for Chinese Minimally Invasive Percutaneous Nephrolithotomy.

[This corrects the article DOI: 10.1007/s12262-014-1043-4.].

Clinical effectiveness of the PolyScope™ endoscope system combined with holmium laser lithotripsy in the treatment of upper urinary calculi with a diameter of less than 2 cm.

The aim of this study was to evaluate the clinical value of the PolyScope™ endoscope system in the treatment of upper urinary calculi with a diameter of <2 cm. A total of 86 patients hospitalized with upper urinary tract calculi were included. The patients were placed under general or spinal anesthesia and in a lithotomy position. Following the dilation of the ureter, a guide wire was inserted under the direct vision of an F8/9.8 rigid ureteroscope, and an F12/14 flexible ureteral access sheath was positioned along the guide wire. Holmium laser lithotripsy was subsequently performed, using an F8.0 'PolyScope' modular flexible ureteroscope. Plain film of the kidney-ureter-bladder (KUB) was performed 1 day subsequent to the surgery, in order to determine the result of the lithotripsy and the position of the double-J stent which was inserted after after holmium laser lithotripsy. In addition, in certain patients, KUB radiography was performed 2-4 weeks subsequent to the surgery, and extracorporeal shockwave lithotripsy (ESWL) was performed if the diameter of the residual stones was >6 mm. Lithotripsy was successful in 77 patients and the duration of the surgery ranged between 25 and 80 min (mean duration, 42 min). Little bleeding was observed. Three patients presented with a slight fever following the surgery; however, no ureteral perforation, high fever or septicemia was observed among the patients following anti-inflammatory treatment. The stone-free rate (SFR) of the single-pass lithotripsy was 89.5% (77/86) and the SFR with ESWL was 96.5% (83/86). The study demonstrated that the F8 modular flexible ureteroscope was safe, convenient and effective for the lithotripsy of upper-tract calculi.

[Modified cystectomy with preservation of erectile and ejaculatory functions in men with nonmalignant bladder disease].

OBJECTIVES: To evaluate modified cystectomy with preservation of erectile and ejaculatory functions in men with nonmalignant bladder disease. METHODS: Seven cases with average age of 27 years presented with bladder disease necessitating cystectomy, including 2 cases of tuberculous contractile bladder, 1 case of extensive polypoid cystitis glandularis, 4 cases of late stage of neurogenic bladder. All patients wished to maintain erectile and ejaculatory functions after the operation. We performed a modified simple cystectomy with preservation of the vasa deferens, seminal vesicles, prostate and neurovascular bundles, as well as construction of an Indiana pouch or ileal neobladder. RESULTS: Average operative time was 5 h 45 min without perioperative complications in this group. Follow-up ranged from 9 to 60 months. Erectile and ejaculatory functions were normal in all cases. All patients remained completely continent and no dysuria in neobladder, and there was no difficulty in inserting catheter to empty pouch. Upper urinary tract was in good condition 3 and 24 months after operation. CONCLUSIONS: Modified cystectomy with preservation of the vasa deferens, seminal vesicles, prostate and neurovascular bundles is an effective and reliable option for the patients who wish to maintain their fertility and erectile function after surgery.