Adrenal Ganglioneuroma with Multifocal Retroperitoneal Extension: A Challenging Diagnosis

A ganglioneuroma (GN) is the rarest and most benign of the neuroblastic tumors and originates from neural crest cells wherever sympathetic nervous tissue exists, such as in the retroperitoneum and adrenal gland. The diagnosis can be very challenging, given the rarity and asymptomatic presentation of this neoplasia, and can be achieved only by means of histological evaluation. Although benign, a few cases of metastatic GNs have been reported in the literature. The prognosis, however, seems to be excellent after surgical resection. We describe a rare case of multifocal retroperitoneal GN, diagnosed incidentally in a 46-year-old woman, with para-aortic and adrenal localizations. After intraoperative pathological diagnosis was made, complete excision of all the visible masses was performed. The postoperative period was uneventful and she was recurrence free 3 months after surgery. To our knowledge, this is the first case report of a multifocal retroperitoneal GN. Among the broad differential diagnoses of adrenal incidentalomas, an adrenal location of neuroblastic tumors should not be forgotten

The hidden life of NAD+-consuming ectoenzymes in the endocrine system

Ectoenzymes are a family of cell surface molecules whose catalytic domain lies in the extracellular region. A subset of this family, nucleotide-metabolizing ectoenzymes, are key components in the regulation of the extracellular balance between nucleotides (e.g., NAD+ or ATP) and nucleosides (e.g., adenosine). Their substrates and products are signalling molecules that act by binding to specific receptors, triggering signals that regulate a variety of functions, ranging from the migration of immune cells, to synaptic transmission in the brain, to hormone/receptor interactions in the glands. Almost two decades of accumulated data indicate that these regulatory processes significantly affect the endocrine system, a tightly controlled information signal complex with clear evidence of fine regulation. Functional models discussed in this review include insulin secretion, bone modelling and the association between hormones and behaviour. The emerging pattern is one of a system operating as a scale-free network that hinges around hubs of key molecules, such as NAD+ or ATP. The underlying natural link between nucleotides, ectoenzymes and the endocrine system is far from being clearly demonstrated. However, the body of evidence supporting the existence of such connection is growing exponentially. This review will try to read the available evidence in a hypothesis-oriented perspective, starting from the description of NAD+ and of ecto- and endoenzymes involved in its metabolism

New insights on occupational exposure and bladder cancer risk: a pooled analysis of two Italian case-control studies.

Purpose: The main risk factor for bladder cancer (BC) is cigarette smoking, but also occupational exposure to carcinogens is relevant, causing about 4-10% of BC. We aimed at investigating the association between BC risk, occupations held in the past and exposure to occupational carcinogens, also assessing whether these associations were influenced by tumor grade. Methods: We pooled data from two Italian case-control studies on male BC, analyzing 893 cases and 978 controls. Occupations were classified using the International Standard Classification of Occupations and exposure to carcinogens was assigned using a validated Job Exposure Matrix. Logistic regression approach was used as well as a semi- Bayesian model, based on a priori information on exposure. Results: A significantly increased BC risk was found for chemical engineering technicians, postmen, and lathe operators, but only for the latter the association remained significant after Bayesian control for type I error. Among carcinogens, cadmium and trichloroethylene were associated with BC. When analyzing data by grade, exposure to these carcinogens was associated with low-grade BC only. Conclusions: Our results suggest that monitoring workplaces to prevent exposure to carcinogenic agents is still an important task, which should be still given adequate importance in public health

Effect of finasteride on the sensitivity of PSA to detect prostate cancer in rebiopsy series.

To evaluate, in a prospective study, the diagnostic accuracy of PSA in patients with a prior negative prostate biopsy who were given finasteride for 6 months. MATERIALS AND METHODS: 91 men with prior negative biopsy findings, including HGPIN and excluding ASAP, were instructed to take finasteride for 6 months. All patients were evaluated at study onset and after 6 months by clinical examination, digital rectal examination (DRE), International Prostate Symptom Score (IPSS) and National Institutes of Health Chronic Prostatitis Symptom Index (NHI-CPSI). Prostate biopsy was repeated at 6 months. PSA levels were measured at baseline and after 1, 3 and 6 months. We calculated the receiver operating characteristics (ROC) curve of PSA under the effect of finasteride for detecting prostate cancer. RESULTS: The median PSA level decreased similarly both in those with prostate cancer and in those without findings of cancer. There was no statistically significant difference between the two groups. The areas under the ROC curve (AUC) of PSA at study onset and after 6 months of therapy with finasteride were, respectively, 0.48 (95% CI 0.36-0.61) and 0.54 (95% CI 0.42-0.66). There was no statistically significant difference between the two areas. CONCLUSIONS: The results of our study show that PSA itself has a low diagnostic accuracy for detecting prostate cancer in men with prior negative prostate biopsy findings. Finasteride does not seem to improve the accuracy of PSA in this particular population of patient