Clinical Research Directory

International PPB/DICER1 Registry

PPB has strong genetic implications, with a unique set of conditions in an individual or their family, occurring mostly in childhood and adolescence. The International PPB/DICER1 Registry first reported DICER1 germline loss-of-function variations in individuals with PPB and their family members. Conditions associated with DICER1 include Sertoli-Leydig cell tumor (SLCT), gynandroblastoma, ovarian sarcoma, peritoneal PPB, lung cysts, renal cysts, cystic nephroma (CN), anaplastic sarcoma of the kidney (ASK), Wilms tumor, primary DICER1-associated central nervous system (CNS) sarcoma, pineoblastoma, pituitary blastoma, multinodular goiter, thyroid carcinoma, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma (CBME), juvenile hamartomatous intestinal polyps, embryonal rhabdomyosarcoma (ERMS) and undifferentiated sarcoma. DICER1 variations may also be associated with macrocephaly and overgrowth syndrome. The International PPB/DICER1 Registry estimates that 30-50 cases of Type I and Ir PPB and 40-60 cases of Types II and III PPB are diagnosed annually worldwide. In 2009, the International Pleuropulmonary Blastoma Registry began a prospective study to collect treatment data on individuals for whom therapy decisions were made by their treating institution.

Nivolumab and Ipilimumab in People With Aggressive Pituitary Tumors

The purpose of this study is to determine if nivolumab and ipilimumab are effective treatment for people with pituitary tumors have gotten worse after surgery and radiation.

A Twenty-years' Experience in Pituitary Disease.

Pituitary tumors represent a heterogeneous group of neoplasms by histotype. The pituitary adenomas are the most frequent heteroformation, among those affecting the pituitary gland, followed by meningiomas, craniopharyngiomas, germosomes and tumours secondary, such as metastases and lymphomas. Since these conditions are considered rare, the data epidemiology and prognosis to predict the natural history of these diseases can not be considered conclusive. Pituitary adenomas are a useful model for epidemiology in the study of pathology pituitary. Over the past 20 years, several attempts have been made to identify unique prognostic factors, which predict the outcome of these pathologies, but without To arrive at a definitive classification. The purpose of this study aims to collect clinical, biochemical, morphological and pathological data on the retrospective and prospective cohort of over 1600 patients undergoing neurosurgical removal of pituitary tumors in the last 20 years, to develop a prognostic classification.