Endocrine Abstracts

Introduction: Surgery is the established first line treatment in adrenocortical carcinoma (ACC). For benign adrenal tumours, laparoscopic adrenalectomy (LA) has become the treatment of choice. However, the role of LA in ACC remains highly controversial. Data from the German ACC Registry were used to evaluate the technical feasibility of LA in ACC and to compare the long-term outcome after LA with the results of open adrenalectomy (OA).Methods: Out of 521...

Objectives: No immunohistochemical marker has yet been established to reliably differentiate adrenocortical tumors from other adrenal masses (e.g. metastases). Thus, a panel of several markers like melan A and inhibin is currently used for this purpose, but suffers from limited diagnostic accuracy. We hypothesized that expression of steroidogenic factor 1 (SF-1), a nuclear transcription factor involved in adrenal development and steroidogenesis, might hold significant diagnost...

Congenital adrenal hyperplasia (CAH) represents the most common inborn metabolic error and is associated with significant morbidity and mortality. There is a paucity of clinical data in adult patients, a lack addressed by congenital adrenal hyperplasia adult study executive (CaHASE), a multicentre cross-sectional study of the health status of adults with CAH that recruited 203 patients from 17 centres (54% of patients contacted). Psychometric evaluation was assessed by validat...

Adrenocortical carcinoma (ACC) is a malignant tumor with poor prognosis and no established therapy in advanced stage. Cisplatin is the most frequently used cytotoxic drug, but even combined with doxorubicin and etoposid, the response rate is <50%. Recently, it has been demonstrated that the excision repair cross complementing group 1 (ERCC1) plays a relevant role in the DNA repairing process, particularly in the correction of platinum-induced DNA adducts. Accordingly, ERCC...

Standard treatment of adrenocortical carcinoma (ACC) comprises adrenolytic therapy with mitotane. Prolongation of bleeding time has previously been observed based on a series of 7 patients (Haak et al. 1991). As patients with ACC frequently undergo surgery for local recurrence or metastases, we have studied the effect of mitotane on coagulation in 44 patients with ACC before and/or during treatment with mitotane (total sample size n=62).Pla...

Adrenal masses are highly prevalent tumours comprising of a variety of entities. Therefore, therapeutic consequences also vary considerably. The CYP11B-specific PET-tracer [11C]metomidate has been shown to be suitable to characterize adrenal lesions. However, its availability is restricted to PET-centers with an on-site cyclotron. Also imaging is hindered by the short tracer half-life (20 min). Therefore, we have developed [123I]iodometomidate as a tracer...

Objectives: Adrenocortical carcinoma (ACC) is a rare tumor with poor prognosis and limited therapeutic options. Survivin is an anti-apoptotic molecule expressed by neoplastic and tumor-specific endothelial cells of various carcinomas, but rarely or only weakly in normal differentiated tissue. In melanoma and pancreatic cancer, preliminary results of a survivin vaccination trial (www.clinicaltrials.gov) indicated that an immunological response in patients is often paralleled by...

Tissue samples from adrenal tumors provide the basis for standard diagnostic procedures such as pathological examination. In addition, these tumor samples have been an invaluable source for the discovery of novel molecular pathways involved in adrenal tumorigenesis. Information on the molecular phenotype are based on DNA mutation analysis and epigenetic changes, RNA and protein expression pattern and sub-cellular localization as well as post transcriptional protein modificatio...

Background: Heterozygous activating somatic mutations in the catalytic subunit α (Cα) of Protein Kinase A (PKA) underlie 30–40% of cortisol producing adrenocortical adenomas (CPA). The activity of the catalytic subunits α,β,γ is controlled by the regulatory subunits (Iα,Iβ, IIα, IIβ). Previous reports found uncommonly reduced levels of RIIβ in CPA compared to other adrenocortical tumors.Aim: Investig...

Background: We have recently introduced [123/131I]iodometomidate (IMTO) which selectively binds to the adrenocortical enzymes aldosterone synthase and 11ß-hydroxylase as SPECT tracer. IMTO has been proven to be useful for molecular adrenal imaging and radiotherapy in adrenal cancer (ACC). As IMTO is rapidly inactivated by endogenous esterases which may impair target tissue to background activity and therapeutic efficacy, >50 new IMTO derivatives have been d...