Genetics and the clinical approach to paragangliomas.

This study analyses new information on gene mutations in paragangliomas and puts them into a clinical context. A suspicion of malignancy is critical to determine the workup and surgical approach in adrenal (A-PGL) and extra-adrenal (E-PGL) paragangliomas (PGLs). Malignancy rates vary with location, family history, and gene tests results. Currently there is no algorithm incorporating the above information for clinical use. A sum of 1,821 articles were retrieved from PubMed using the search terms "paraganglioma genetics". Thirty-seven articles were selected of which 9 were analyzed. It was found that 599/2,487 (24%) patients affected with paragangliomas had a germline mutation. Of these 30.2% were mutations in SDHB, 25% VHL, 19.4% RET, 18.4% SDHD, 5.0% NF1, and 2.0% SDHC genes. A family history was positive in 18.1-64.3% of patients. Adrenal PGLs accounted for 55.1% in mutation (+) and 81.0% in mutation (-) patients (RR 1.2, p < 0.0001). Bilateral A-PGLs accounted for 56.4% in mutation (+) and 3.2% in mutation (-) patients (RR 8.7, p < 0.0001). E-PGL were found in 33.6% of mut+ and 17.3% of mut- (RR 1.7, p < 0.0001). In mutation (+) patients PGLs malignancy varied with location, adrenal (6.4%) thoraco-abdominal E-PGL (38%), H & N E-PGL (10%). Malignancy rates were 8.2% in mutation (-) and lower in mutation (+) PGLs except for SDHB 36.5% and SDHC 8.3%. Exclusion of a mutation lowered the probability of malignancy significantly in E-PGL (RR 0.03 (95% CI 0.1-0.6); p < 0.001). Mutation analysis provides valuable preoperative information to assess the risk of malignancy in A-PG and E-PGLs and should be considered in the work up of all E-PGL lesions.

Oncologic resection achieving r0 margins improves disease-free survival in parathyroid cancer.

BACKGROUND: Parathyroid cancer has a poor mid-term prognosis, often because of local recurrence, observed in half of all patients. Modern diagnostic workup increasingly enables a preoperative diagnosis of parathyroid cancer. There is limited evidence that more comprehensive oncologic surgery can reduce the risk of local recurrence. This study aims to identify the best specific surgical approach in parathyroid cancer. METHODS: This observational cohort study comprises 19 consecutive patients who had undergone oncologic or nononcologic resection for parathyroid cancer. Baseline parameters were compared by using univariate analysis; outcomes were assessed by χ (2) testing and Kaplan-Meier statistics. RESULTS: Fifteen of 19 patients were primarily operated on in our tertiary center between 1996 and 2013, and four were referred for follow-up because of their cancer diagnosis. Patient cohorts defined by histologic R-status were comparable for established risk factors: sex, calcium levels, low-risk/high-risk status, and presence of vascular invasion. Oncologic resections were performed in 13 of 15 patients primarily treated in the center and 0 of 4 treated elsewhere (χ (2) = 5.6; p < 0.01). R0 margins were achieved in 11 of 13 (85 %) undergoing oncologic resection and 1 of 6 (17 %) undergoing local excision (χ (2) = 8.1; p < 0.01). R0 margins and primary oncologic resection were associated with higher disease-free survival rates (χ (2) = 7.9; p = 0.005 and χ (2) = 4.7; p = 0.03, respectively). Revision surgery achieved R0 margins in only 2 of 4 (50 %) of patients. CONCLUSIONS: In parathyroid cancer, a more comprehensive surgery (primary oncologic resection) provides significantly better outcomes than local excision as a result of reduction of R1 margins and locoregional recurrence.

[An update on minimally invasive surgery]. / Attualità in tema di chirurgia mini-invasiva.

Minimally invasive surgery is a highly technological specialty of surgical practice, and requires training and retraining for traditional surgeons. The development of new instruments and new technologies during the last few years has allowed complex procedures that were previously considered unsuitable for minimally invasive surgery, but it has to be recognized that training, accreditation, and case load are fundamental for programs in advanced procedures. This article reviews some of these items including innovative surgical technologies related to minimally invasive surgery.

Parapubic hernia and diabetes insipidus.

According to the current literature parapubic hernia is regarded as a relatively rare pathology. The causative defect is usually related to pelvic surgery, traumatism, or bone malformation. Surgical treatment is difficult, because aponeurotic tissue on which to anchor a mesh on the caudal aspect of the defect is missing. This report describes a case of an incisional pubic hernia related to megabladder in a woman affected by diabetes insipidus and with a past clinical history of multiple pelvic interventions. As far as we are aware this report adds a new pathogenetic issue for parapubic hernias not previously described in the literature.

A camera handler for Miccoli's minimally invasive video-assisted thyroidectomy and paratiroidectomy procedures.

BACKGROUND: Minimally invasive video-assisted thyroidectomy and paratiroidectomy (MIVAT/P) are surgical procedures performed with 5-mm cameras handled by a camera assistant. METHODS: The authors created a new camera handler for video-assisted neck surgery. It consists of a telescopic tripod device designed for mechanical handling of the camera, which is directly oriented by the operator even in solo surgery procedures. The camera is placed inside an O-shaped support, and moved by the operator himself for exploration and work on the surgical field. RESULTS: Thanks to this simple device, the camera holder provides a firm field and prevents blood stains in limited working spaces. CONCLUSIONS: The novel camera handler may be useful in either MIVAT/P or other simple laparoscopic procedures (i.e., cholecystectomy) for a steady handling of the camera, even in solo surgery procedures.

A less invasive, selective, functional neck dissection for papillary thyroid carcinoma.

HYPOTHESIS: To describe an alternative lateral neck access in order to perform neck dissection in papillary thyroid carcinoma (PTC) with lymph node involvement. DESIGN: Prospective (January 2000 to December 2003), 36-month study. SETTING: Catholic University of Rome and University of "Tor Vergata", Rome, Italy. PATIENTS AND METHODS: Twenty-four consecutive patients with PTC and cervical lymph node metastases were included. Functional neck dissection (FND), unilateral or bilateral, was performed acceding via a lateral dissection through a traditional Kocher incision, running along the superficial fascia of the neck and posteriorly to the sternocleidomastoideus muscle. RESULTS: Mean age was 39.04+/-13.69 years. Twenty patients were women and four were men. Mean tumour size was 2.5+/-1 cm, while the greatest metastatic lymph node size was 4.5 cm. Minimally invasive, selective FND was performed in all patients associated to total thyroidectomy and central compartment lymph node clearance. Metastatic lymph nodes were found in 142 out of 340 of the lymph nodes dissected. CONCLUSIONS: Minimally invasive neck dissection seems to carry a lower risk in terms of specific morbidity and allows a quicker recovery and a better aesthetic result. This access has to be considered as a less invasive procedure compared to the other surgical accesses for the radical or modified lateral neck dissection.