Familial isolated pituitary adenomas experience at a single center: clinical importance of AIP mutation screening.

We present four FIPA kindred discussing clinical and molecular data and emphasizing the differences regarding AIP status, as well as the importance of genetic screening. Family 1 consists of five patients harboring somatotropinomas with germline E24X mutation in AIP. In one of the patients, acromegaly was diagnosed through active screening, being cured by surgery.

Low aryl hydrocarbon receptor-interacting protein expression is a better marker of invasiveness in somatotropinomas than Ki-67 and p53.

Background: Some pituitary adenomas exhibit fast growth and invade surrounding structures. To date, there is no robust marker to predict invasiveness.

Aim: To evaluate Ki-67, p53 and aryl hydrocarbon receptor-interacting protein (AIP) expression and compare these between invasive and noninvasive somatotropinomas and nonfunctioning pituitary adenomas (NFPAs).

Germ cell tumor presenting as sellar mass with suprasellar extension and long history of hypopituitarism.

Objective: Primary central nervous system germ cell tumors are rare neoplasms usually located in the pineal and/or suprasellar region. Pure germinomas do not usually secrete beta-human chorionic gonadotropin hormone (beta-HCG) or alpha-fetoprotein (AFP) and diagnosis is made a few weeks or months after beginning of symptoms.

Case: Here we report a case of a pure germinoma in a 20 year-old woman presenting as a sellar mass with suprasellar extension, abnormal serum beta-HCG and a long history of polyuria and polydipsia (4 years), that was initially diagnosed as a pituitary macroadenoma.