Clinical and biochemical features of seven adult adrenal ganglioneuromas.

BACKGROUND: Adrenal ganglioneuroma (GN) is seldom considered in the differential diagnosis of adrenal lesions, and its clinical presentation is not well known. OBJECTIVE: Our aim was to describe the clinical, biochemical, and radiological features of adrenal GNs in adults. METHODS: Seven adults underwent endocrine investigation for adrenal lesions that were confirmed to be adrenal GNs. RESULTS: Mean age of the seven patients was 49 yr (range, 23 to 71 yr). Average tumor diameter was 5.0 cm (range, 1.5 to 10.4 cm). In five patients, the adrenal lesions were found incidentally. A 49-yr-old female carried a germline mutation in MSH2 gene. A 57-yr-old female presented with mild virilization and increased testosterone levels. Bilateral adrenal venous sampling revealed testosterone production from her right adrenal lesion. All tumors showed nonenhanced attenuation between 25 and 40 Hounsfield units on computed tomography scan. Magnetic resonance imaging revealed low- to iso-signal intensity on T1-weighted imaging and high-signal intensity on T2-weighted imaging. [(18)F]-2-Fluoro-deoxy-d-glucose-positron emission tomography scan (n = 5) disclosed a mean standard uptake value of 2.4. Three tumors were composite pheochromocytoma-GN. Microsatellite instability study and immunohistochemical analysis of MSH2 protein in a patient carrying a MSH2 mutation showed normal MSH2 protein expression and low microsatellite instability, indicating that the adrenal GN was not related to the patient's MSH2 germline defect. CONCLUSIONS: We describe one of the largest series of adult adrenal GNs. Adrenal GNs may secrete testosterone or be part of a composite tumor with pheochromocytoma. The association of adrenal GN with MSH2 mutation seems to be a coincidental finding.

The use of partial nephrectomy in European tertiary care centers.

PURPOSE: The objective was to define the trends of PN use over time at six tertiary care European centers. METHODS: Data were retrieved from institutional databases for patients treated with either PN or radical nephrectomy (RN) for stages T(1-2)N(0)M(0) renal cell carcinoma (RCC) between 1987 and 2007. For purpose of temporal trend analyses patients were divided into five equally sized groups according to the date of surgery. Categorical and multivariable logistic regression analyses assessed predictors of PN use. RESULTS: Overall 597 (31.7%) patients were treated with PN. Overall, a 4.5-fold increase of PN was recorded. The absolute increases were 41.7-86.3%, 14.9-69.3% and 8.1-35.3% for lesions < or = 2 cm, 2.1-4 cm and 4.1-7 cm (chi-square trend test p<0.001), respectively. In multivariable logistic regression models, decreasing tumor size, younger age, more contemporary date of surgery, male gender and institutional PN rate represented independent predictors of the individual probability of treatment with PN. Lack of data from community hospitals limits the generalizability of our findings. CONCLUSION: Based on data from six tertiary care centers, the contemporary rate of PN ranges from 86 to 35% for renal masses < or = 2 cm to 4.1-7 cm and is indicative of excellent quality of care.