Pituitary carcinomas are rare, making up some 0.2% of all pituitary tumors, but represent a particular challenge to clinical practice. The diagnosis of a pituitary carcinoma requires evidence of metastatic disease, either outside the central nervous system (CNS) or as separate noncontiguous foci within the CNS. They may present as typical pituitary adenomas, which reveal their malignant character only as time progresses, or as peculiarly aggressive tumors ab initio. Recent changes in histopathological classification have clarified many of the features of such tumors, including immunohistochemical staining for Ki-67 and p53, but to date none has been found to be pathognomonic. The majority of carcinomas are secretory, usually arising from 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 inexorable. However, we do not believe there will be any real prospect of long-term survival until the development and use of therapies targeted at specific molecular abnormalities.
Background and aim: The aim of this study was to illustrate the present role of transsphenoidal surgery as primary therapy in GH-secreting adenomas, and to compare the results concerning control of disease with previous series using older criteria of cure. Method: We report on a consecutive series of 688 acromegalic patients treated over a time period of 19 years. Biochemical cure was defined as normalisation of basal GH level, suppression of GH levels to below 1 ng/ml during an oral glucose load and normalisation of IGF-I levels. Of the 506 patients undergoing primary transsphenoidal surgery, a total of 57.3% postoperatively fulfilled the criteria used.Results: The rate of biochemical 'cure' correlated with the magnitude of the initial GH levels, the tumour size and invasion. The overall complication rate was below 2%. Mortality in this series was 0.1% (1 of 688). During a follow-up period of 10.7 years only two recurrences (0.4%) occurred. However, in the patients treated by transcranial surgery and by repeat surgery the cure rate was found to be relatively low (5.2 and 21.3% respectively). Conclusions: These data suggest that surgery remains with very few exceptions the primary treatment of acromegaly for (i) a high cure rate, (ii) low morbidity, (iii) low recurrence rate and (iv) immediate decline of GH. Based on current criteria of cure, recurrences are uncommon. However, cure by surgery alone is improbable in patients harbouring extended, invasive tumours with high secretory activity, in whom further adjuvant treatment is mandatory.
These data indicate that transsphenoidal surgery for non-functioning pituitary adenomas in expert hands is, relatively, far less detrimental to patients compared with transcranial surgery. The latter carries a much greater risk of post-operative deterioration in pituitary function.
Lymphocytic hypophysitis and granulomatous hypophysitis represent related inflammatory disorders. Their conspicuous clinical features frequently allow preoperative diagnosis of hypophysitis. In view of their sometimes insidious clinical course, early surgical exploration is justified.
This work provides characterization of system-related geometric distortions present in MRIs used in Gamma Knife (GK) stereotactic radiosurgery (SRS) treatment planning. A custom-made phantom, compatible with the Leksell stereotactic frame model G and encompassing 947 control points (CPs), was utilized. MR images were obtained with and without the frame, thus allowing discrimination of frame-induced distortions. In the absence of the frame and following compensation for field inhomogeneities, measured average CP disposition owing to gradient nonlinearities was 0.53 mm. In presence of the frame, contrarily, detected distortion was greatly increased (up to about 5 mm) in the vicinity of the frame base due to eddy currents induced in the closed loop of its aluminum material. Frame-related distortion was obliterated at approximately 90 mm from the frame base. Although the region with the maximum observed distortion may not lie within the GK treatable volume, the presence of the frame results in distortion of the order of 1.5 mm at a 7 cm distance from the center of the Leksell space. Additionally, severe distortions observed outside the treatable volume could possibly impinge on the delivery accuracy mainly by adversely affecting the registration process (e.g. the position of the lower part of the N-shaped fiducials used to define the stereotactic space may be miss-registered). Images acquired with a modified version of the frame developed by replacing its front side with an acrylic bar, thus interrupting the closed aluminum loop and reducing the induced eddy currents, were shown to benefit from relatively reduced distortion. System-related distortion was also identified in patient MR images. Using corresponding CT angiography images as a reference, an offset of 1.1 mm was detected for two vessels lying in close proximity to the frame base, while excellent spatial agreement was observed for a vessel far apart from the frame base.
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