Endocrine Abstracts

The neuroendocrine tumor of thymus (TNET) is an extremely rare disease. It can occur sporadically or as a part of the multiple endocrine neoplasia (MEN1) syndrome. TNET may secret hormons (ectopic ACTH production). It is a potentially malignant tumor which often develops distant metastases. Its prognostic factors are the tumor size, histological grade, Ki67 index, paraneoplastic symptoms, surgical resection and Masaoka staging. The options for treatment are radical surgery, ch...

Multiple endocrine neoplasia (MEN-1) is a rare congenital disease with genetic background. The MEN-1 gene encodes the menin protein, which acts as a tumour suppressor. Mutation of one allele and the inactivation of the other allele of this gene lead to clonal proliferation and to the development of tumours. The clinical manifestation of MEN-1 is a combination of endocrine (most commonly parathyreoid adenomas, entero-pancreatic endocrine tumours, pituitary tumours) and non-endo...

The polyglandular autoimmune syndromes (PAS) comprise a wide spectrum of autoimmune disorders and are divided into a very rare juvenile (type I) and a relatively common adult type with (type II) or without adrenal failure (PAS III). For PAS II/III, susceptibility genes are known inreasing the risk for developing autoimmune disorders, but without being causative. Actual diagnosis of PAS involves serological measurement of organ-specific autoantibodies and subsequent functional ...

Introduction: Multiple endocrine neoplasia (MEN) type 1 is a rare congenital disease with genetic background. The MEN-1 gene encodes menin protein, that acts as a tumor suppressor. Mutation of one allele and the inactivation of the other allele of this gene lead to clonal proliferation and to the development of tumors. The clinical manifestation of MEN type 1 is a combination of endocrine (parathyroid adenomas, entero-pancreatic neuroendocrine tumors, pituitary tumors) and non...

Adrenocortical cancer is a rare and heterogenous malignancy with incompletely understood pathogenesis and poor prognosis. Sometimes patients present with hormonal excess symptoms (e.g. virilization, Cushing’s syndrome) or local symptoms consistent with abdominal space-occupation (median tumor size at the time of diagnosis may be >10 cm). Three cases are presented to give an overview of how adrenocortical cancer is currently managed. Tumors typically appear inhomogenou...