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Submitted on November 17, 2004
Accepted on February 4, 2005
Department of Endocrinology (G.A.K., A.B.G.), St Bartholomew's Hospital, London, ECIA 7BE, UK, Department of Neurosurgery (P.N., M.B.), University of Goettingen, Germany, and Department of Pathology (G.K), George Gennimatas Hospital, Athens, Greece
* To whom correspondence should be addressed. E-mail: A.B.Grossman{at}qmul.ac.uk.
Pituitary carcinomas are rare, some 0.2% of all pituitary tumors, but represent a particular challenge to clinical practise. The diagnosis of a pituitary carcinoma requires evidence of metastatic disease, either outside the central nervous system (CNS) or as separate non-contiguous foci within the CNS. They may present as a typical pituitary adenoma, only revealing their malignant character as time progresses, or as peculiarly aggressive tumors ab initio. Recent changes in histopathological classification have clarified many of the features which may be seen in such tumors, including immunohistochemical staining for Ki-67 and p53, but none has so far been found to be pathognomonic. The majority of carcinomas are secretory, usually arising form corticotroph tumors or prolactinomas, but all histological types and secretory patterns are represented. Treatment is by surgery, transsphenoidal wherever possible, and conventional and stereotactic radiotherapy, but ultimately a plethora of therapies may be required including various attempts at medical therapy. Chemotherapy in some instances probably prolongs survival, but in general their progress from the diagnosis of carcinomatous changes is progressive and in exorable. However, we do not believe any real prospect of long-term survival will occur until the development and use of therapies targeted at specific molecular abnormalities.
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