RNA Sequencing Provides Novel Insights into the Transcriptome of Aldosterone Producing Adenomas.

Aldosterone producing adenomas (APAs) occur in the adrenal glands of around 30% of patients with primary aldosteronism, the most common form of secondary hypertension. Somatic mutations in KCNJ5, ATP1A1, ATP2B3, CACNA1D and CTNNB1 have been described in ~60% of these tumours. We subjected 15 aldosterone producing adenomas (13 with known mutations and two without) to RNA Sequencing and Whole Genome Sequencing (n = 2).

Global DNA Methylation Analysis Identifies Two Discrete clusters of Pheochromocytoma with Distinct Genomic and Genetic Alterations.

Pheochromocytomas and paragangliomas (PPGLs) are rare and frequently heritable neural-crest derived tumours arising from the adrenal medulla or extra-adrenal chromaffin cells respectively. The majority of PPGL tumours are benign and do not recur with distant metastases. However, a sizeable fraction of these tumours secrete vasoactive catecholamines into the circulation causing a variety of symptoms including hypertension, palpitations and diaphoresis.

Survivin and XIAP: two valuable biomarkers in medullary thyroid carcinoma.

Background: Medullary thyroid carcinoma (MTC) accounts for ∼5% of all thyroid malignancies. To date, surgery is the first-line therapy with curative intention. However, for advanced MTC, conventional chemotherapeutic agents do not provide convincing results.

Activating mutations in CTNNB1 in aldosterone producing adenomas.

Primary aldosteronism (PA) is the most common cause of secondary hypertension with a prevalence of 5-10% in unreferred hypertensive patients. Aldosterone producing adenomas (APAs) constitute a large proportion of PA cases and represent a surgically correctable form of the disease. The WNT signaling pathway is activated in APAs.

Novel somatic mutations and distinct molecular signature in aldosterone-producing adenomas.

Aldosterone-producing adenomas (APAs) are found in 1.5-3.0% of hypertensive patients in primary care and can be cured by surgery.

Is minimal residual lymph node disease in papillary thyroid cancer of prognostic impact? An analysis of the epithelial cell adhesion molecule EpCAM in lymph nodes of 40 pN0 patients.

This study was aimed to assess the extend of nodal microdissemination in patients with pN0 papillary thyroid carcinoma (PTC) using immunohistochemical analysis. In early stage PTC both, systematic lymphadenectomy as well as radio iodine treatment, aimed to eliminate occult nodal tumor involvement, are under controversial debate, since little is known about the extend of lymphatic microdissemination in these patients. Formalin embedded samples of the resected lymph nodes were systematically screened for the presence of disseminated tumor cells using immunohistochemistry (monoclonal antibody Ber-EP4).

Notch 1 tumor expression is lacking in highly proliferative pancreatic neuroendocrine tumors.

To date, very little is known about the development of benign organic hyperinsulinism and its metastatic potential. Typical morphologic, biochemical, or genetic differentiations for benign or malign tumor course of insulinomas do not exist. As signaling pathways may affect pancreatic cancer development and the maintenance of the neoplastic phenotype, the purpose of this study was to examine the role of Notch1 expression in organic hyperinsulinism.

Approach to the management of slipped capital femoral epiphysis and primary hyperparathyroidism.

Context: Worldwide, only nine cases of revealing slipped capital femoral epiphysis (SCFE) associated with primary hyperparathyroidism (PHP) have been reported.

Case Illustration: This study included adolescent subjects with the described association, the clinical course, and exhibiting the leading pathogeneses.

Methods: Here, we reviewed all known cases and developed an effective approach to the management of SCFE and PHP.

Comprehensive re-sequencing of adrenal aldosterone producing lesions reveal three somatic mutations near the KCNJ5 potassium channel selectivity filter.

Background: Aldosterone producing lesions are a common cause of hypertension, but genetic alterations for tumorigenesis have been unclear. Recently, either of two recurrent somatic missense mutations (G151R or L168R) was found in the potassium channel KCNJ5 gene in aldosterone producing adenomas. These mutations alter the channel selectivity filter and result in Na(+) conductance and cell depolarization, stimulating aldosterone production and cell proliferation.

Approach to the management of slipped capital femoral epiphysis and primary hyperparathyroidism.

Context: Worldwide, only nine cases of slipped capital femoral epiphysis (SCFE) associated with primary hyperparathyroidism (PHP) have been reported.

Case Illustration: This is a report on adolescent subjects with SCFE associated with PHP exhibiting the leading pathogenesis and clinical course.

Methods: Here, we reviewed all known cases and developed an effective approach to the management of SCFE and PHP.

Trends in adrenal surgery: institutional review of 528 consecutive adrenalectomies.

Purpose: The increasing detection of adrenal tumors and the availability of a more sophisticated biochemical work-up leading to rising numbers of sub-clinical Conn's and Cushing's syndromes coincide with a rising number of adrenalectomies worldwide. The aim of our study was to report a single institution's experience with adrenal surgery.

Methods: We report data of 528 adrenalectomies, operated at our institution before and after the onset of minimally invasive endoscopic surgery (1986-1994, 1995-2008).

Increased numbers of tumor-lysing monocytes in cancer patients.

Lymphatic infiltration is a well known phenomenon in different tumors including endocrine malignancies. However, little is known about the role of antigen-presenting cells and T cell activation in this context. The aim of our study was to investigate the quantity and function of CD14+/CD56+ monocytes in tumor patients including endocrine malignancies.

Unusual histological findings after partial pancreaticoduodenectomy including benign multicystic mesothelioma, adenomyoma of the ampulla of Vater, and undifferentiated carcinoma, sarcomatoid variant: a case series.

Introduction: The standard operation for carcinoma of the pancreatic head is a partial pancreaticoduodenectomy. Unusual histological findings may occasionally occur in the surgical specimen. We present three unusual histologic diagnoses after pancreaticoduodenectomy.

Systematic comparison of sporadic and syndromic pancreatic islet cell tumors.

Pancreatic islet cell tumors (ICTs) occur as sporadic neoplasias or as a manifestation of multiple endocrine neoplasia type 1 (MEN1) and von Hippel-Lindau disease (VHL). Molecular classification of ICTs is mandatory for timely diagnosis and surveillance. Systematic comparison of VHL-ICTs and sporadic ICTs has been lacking.

Indicators of mineralocorticoid excess in the evaluation of primary aldosteronism.

It is suggested to use the aldosterone-to-renin ratio (ARR) as a first test in the screening for primary aldosteronism (PA). However, many groups rather rely on the determination of urinary tetrahydroaldosterone secretion; others calculate a ratio of urinary aldosterone to plasma renin activity. The aim of the present study was to evaluate the usefulness of different parameters of aldosterone excess in the case finding of PA.

Sporadic solitary aldosterone- and cortisol-co-secreting adenomas: endocrine, histological and genetic findings in a subtype of primary aldosteronism.

Adrenal adenomas producing both aldosterone and cortisol (A/CPAs) have been described in only a few cases. Correct subtype classification is necessary for making therapeutic decisions in primary aldosteronism (PA). Therefore, we studied in detail the clinical, hormonal and histological features of this entity in two patients with A/CPAs.

Increased EpCAM expression in malignant insulinoma: potential clinical implications.

Objective: EpCAM (CD326) is overexpressed in progenitor cells of endocrine pancreatic islands of Langerhans during fetal development and was suggested to act as a morphoregulatory molecule in pancreatic island ontogeny. We tested whether EpCAM overexpression is reactivated in insulinomas, endocrine tumors arising in the pancreas.

Design/method: We used monoclonal anti-EpCAM antibody Ber-Ep4 for immunohistochemistry on formalin-fixed and paraffin-embedded tumor material.

The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders.

Unlabelled: TGR5 (Gpbar-1) is a plasma membrane-bound, G protein-coupled receptor for bile acids. TGR5 messenger RNA (mRNA) has been detected in many tissues, including rat cholangiocytes and mouse gallbladder. A role for TGR5 in gallstone formation has been suggested, because TGR5 knockout mice did not develop gallstones when fed a lithogenic diet.

Multiple giant scalp metastases of a follicular thyroid carcinoma.

Background: The occurrence of skin metastases are rare events in the course of a follicular thyroid carcinoma (FTC) and usually indicate advanced tumor stages. The scalp is the most affected area of these metastases.

Case Presentation: We present a case of a 76 year old Woman with multiple giant scalp metastases of a follicular carcinoma.

Laparotomy enables retrograde dilatation and stent placement for malignant esophago-respiratory fistula.

Background: Malignant esophageal stenosis with complete obstruction and esophagorespiratory fistula (ERF) is difficult to treat with standard endoscopic techniques.

Case Presentation: We report a patient in whom with local recurrence of esophageal carcinoma an esophagotracheal fistula occurred. Initially the patient had undergone esophageal resection with interposition of a gastric tube.

Long-term clinical and biochemical follow-up in medullary thyroid carcinoma: a single institution's experience over 20 years.

Objective: Many patients with medullary thyroid carcinomas (MTC) have reoperative surgery in different hospitals, which makes their follow-up difficult. To comprehend these complex courses and to find relevant prognostic factors we report a 20-year single center experience of 289 patients with MTC or precursor C-cell-hyperplasias.

Patients And Methods: Between April 1986 and May 2006, 289 consecutive patients with MTC or MEN2 gene carriers were treated at the Department of Surgery at the University Hospital Düsseldorf.

Identification of occult metastases of medullary thyroid carcinoma by pentagastrin-stimulated intravenous calcitonin sampling followed by targeted surgery.

Background: High calcitonin (CT) serum levels suggest metastatic spread in medullary thyroid carcinoma (MTC) after thyroidectomy. In limited disease stages, however, morphological investigations including ultrasound, magnetic resonance imaging (MRI) and 18F-FDG positron emission tomography ([18F]FDG-PET) may often fail to identify exact tumour sites.

Objective: The aim of the present study was to establish an improved strategy to identify small cervical tumours by combining pentagastrin stimulation with bilateral cervical intravenous CT sampling followed by high-resolution ultrasound.

Neuropsychiatric and cognitive changes after surgery for primary hyperparathyroidism.

Introduction: Neuropsychiatric symptoms and cognitive impairment are mental manifestations of primary hyperparathyroidism (pHPT). The aim of our study was to determine if surgical treatment results in a long-lasting full recovery from these symptoms.

Methods: In a prospective case-control study with matching pairs, mental changes were examined preoperatively and 6 months postoperatively in 30 patients with primary hyperparathyroidism and 30 patients with nontoxic nodular goiter using the Hamilton depression score and four cognitive tests: DEM Tect, MWT (multiple word test), ZVT, and Benton test.