Silent corticotroph adenoma: case report and literature review.

OBJECTIVE: To review the initial clinical manifestations and diagnosis of silent corticotroph adenoma. METHODS: We report a case and summarize the relevant literature. RESULTS: A 52-year-old patient with hypopituitarism underwent resection of a silent corticotroph adenoma. A circulating species was detected postoperatively, reactive in a highly sensitive adrenocorticotropic hormone (ACTH) 1-39 immunoradiometric assay (IRMA) and beta-endorphin or beta-lipotropin radioimmunoassay. The basal morning cortisol concentration consistently was <10 microg/dL. Dynamic testing was performed to screen for Addison's disease, congenital adrenal hyperplasia, and Cushing's syndrome. During dexamethasone suppression, the molar concentration of circulating ACTH precursors by a two-site IRMA was 55-fold greater than the concentration of ACTH 1-39 by IRMA. We concluded that the tumor displayed impaired processing of pro-opiomelanocortin (POMC) and secreted a bioinactive POMC-derived peptide that was reactive in the ACTH 1-39 IRMA. CONCLUSION: Patients with silent corticotroph adenoma do not have clinically evident Cushing's syndrome. In some cases, bioinactive ACTH precursors may be detected by a sensitive ACTH 1-39 IRMA.

Magnetic resonance imaging and pathological analysis of a pituicytoma: case report.

A case of a pituicytoma is presented that describes the clinical, pathological, and magnetic resonance imaging features of a rare tumor of the neurohypophysis. A 26-year-old woman presented with a 4-month history of dizziness and visual obscuration. A magnetic resonance image revealed a pituitary mass with suprasellar extension. The specimen obtained from a transsphenoidal decompression identified the mass as an astrocytoma of the posterior pituitary (pituicytoma). Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100, and vimentin. Electron microscopy identified intermediate filaments, numerous broad cell junctions, no secretory granules, and two cellular populations with either an electron-dense or lucent cytoplasmic matrix. This case is unique in that other documented cases originating in the posterior pituitary have all been pilocytic astrocytomas, whereas this neoplasm was not a pilocytic variant. This is also the first case in the literature of a pituicytoma documented by magnetic resonance imaging. This report reviews the cytological elements of the neurohypophysis and the origin of pituicytomas and stresses the proper use of the term "pituicytoma" in relation to tumors of the posterior pituitary.

Recurrent intracranial epithelioid hemangioendothelioma associated with multicentric disease of liver and heart: case report.

Epithelioid hemangioendothelioma is an unusual vascular neoplasm with prominent cytoplasmic vacuolization representing primitive lumen formation. A case is presented of this unique vascular neoplasm in a woman with a seizure disorder who had cardiac, hepatic, and recurrent nervous system lesions. To our knowledge, this is the third known case of intracranial epithelioid hemangioendothelioma. Emphasis is placed on the indolent course of this rare neoplasm, with a recommendation for aggressive surgical treatment and diligent follow-up.

Adenocarcinoma metastatic to a growth-hormone-secreting pituitary adenoma: case report.

Although the pituitary gland is known to harbor metastatic deposits, it is a rare occurrence for a metastatic deposit to appear in a pituitary adenoma. A case is presented of an adenocarcinoma metastatic in an acromegalic patient with a pituitary adenoma. This report adds to the literature of the unusual phenomenon of neoplasm-to-neoplasm metastasis.

Magnetic resonance imaging as a sensitive and specific predictor of neoplasms removed for intractable epilepsy.

Twenty-three patients had magnetic resonance imaging (MRI) and computed tomography (CT) of the head prior to surgery for medically intractable epilepsy. Eleven patients had neoplasms, mostly astrocytomas. Six of the 11 tumors were seen on CT. In five of the six cases, the MRI showed a focal area of increased signal on T2-weighted images. All 11 tumors were detected by MRI. None of the non-neoplastic lesions produced an abnormal T2-weighted signal area on MRI. Only one of the non-neoplastic lesions was seen on both CT and on MRI. MRI allowed clear discrimination between tumors and non-neoplastic lesions in patients coming to surgery for intractable epilepsy.

Severe cerebral damage in ornithine transcarbamylase deficiency.

Two patients are described with hyperammonemia due to ornithine transcarbamylase (OTC) deficiency who suffered severe shrinkage and collapse of the brain. The cerebral cortex was spongy and cavitated, containing only a few residual neurons, and was markedly gliosed. In one patient the basal ganglia were affected and harbored Alzheimer type II astrocytes. These lesions resemble those of acquired hepatocerebral degeneration and occur especially in female children with the milder form of the disease, who have a potential to survive. Strict observance of dietary restrictions is mandatory to avoid catastrophic damage to the brain.

Cortical biopsy results in Alzheimer's disease: correlation with cognitive deficits.

Neuropsychologic and pathologic data are presented for a group of 11 patients with a clinical diagnosis of probable Alzheimer's disease (AD) according to recently proposed criteria. In all cases, the diagnosis was verified by cortical biopsy. In addition, increased cortical plaque counts were associated with greater deficits in language production and comprehension and poorer performance on an index of global mental status. These results suggest that a clinical diagnosis of AD is very accurate when patient selection is restricted to typical cases and that language deficits may provide a useful indicator of severity of disease in AD patients.

Dual energy computerized tomography: a simplified approach.

Attenuation numbers of various organic liquids were measured in a phantom at the low and high X-ray beam settings of the Mark I and CT 1010. The readings were translated into absolute units and ratios computed. There is a nonlinear relationship between this ratio and the effective atomic number of the compounds tested. A ratio of one defines a substance with energy dependence like water. Ratios below one definite anhydrous substances composed of elements with low atomic numbers. Ratios greater than one defined substances containing elements with high atomic numbers.

[The elimination of hydroxyethyl starch 200/0.5, dextran 40 and oxypolygelatine (author's transl)]. / Die Elimination von Hydroxyäthylstärke 200/0,5, Dextran 40 und Oxypolygelatine.

After withdrawal of 400 ml whole blood and subsequent infusion of 500 ml of a colloidal plasma substituent, the intravascular and renal colloid elimination was investigated in 40 test subjects. The individual colloidal solutions could no longer be demonstrated in the intravascular space after the following times: 10% hydroxyethyl starch 200/0.5 (anthrone method) after six weeks, 10% dextran 40 (anthrone method) after two weeks, 6% hydroxyethyl starch 200/0.5 (anthrone method) after four weeks and 5.5% oxypolygelatine (hydroxyproline method) after two days. Colloidal plasma substitutes are polydisperse solutions with various molecular weights and degree of hydroxyethylation and therefore, also have a large number of different elimination constants. With repeated application, the intravascular colloid concentration shifts in favour of the molecules with a longer half life which are difficult to eliminate. The elimination of the clinically employed dextran 40 and oxypolygelatine solution could be best described with an open two-compartment model. As a result of its greater heterogeneity, the elimination of the moderately high molecular weight hydroxyethyl starch 200/0.5 could only be characterized approximately even assuming three elimination constants. In the first four days, the hydroxyethyl starch 200/0.5 was more rapidly eliminated compared to dextran 40. However, subsequently a very much lower elimination from the intravascular space was found for about 3% of the administered hydroxyethyl starch 200/0.5. Oxypolygelatine was eliminated especially rapidly. Accordingly, the greatest renal clearance was found for oxypolygelatine, which showed a close relation to the molecular weight. On the other hand, a rapid elimination simultaneously is followed by a correspondingly lower volume effect.

Protein and electrolyte changes in experimental cerebral edema.

Analysis of protein and electrolyte data in cryogenic cerebral edema in the rhesus monkey has led to the conclusion that, in the first 24 hours (h) after injury, the edematous process is not homogenous, but compartmentalized. This involves, first of all, a division into intra- and extracellular compartments. The intracellular compartment is further divided into a compartment containing water, electrolytes, and serum proteins, and a compartment containing only excess sodium. The extracellular compartment is also subdivided into a compartment containing albumin, globulin, and electrolytes, and a compartment containing only albumin and electrolytes. Anatomically, the latter is most likely the pre-existing normal extracellular space.

[The effects of 500 ml 10% hydroxyethyl starch 200/0.5 and 10% dextran 40 on blood volume, colloid osmotic pressure and renal function in human volunteers (author's transl)]. / Blutvolumen, killoidosmotischer Druck und Nierenfunktion von Probanden nach Infusion mittelmolekularer 10% Hydroxyäthylstärke 200/0,5 und 10% Dextran 40.

The effects of administration of a new, middle-molecular 10% hydroxyethyl starch 200/0.5 (HAES-steril) were compared to 10% dextran 40 (Rheomacrodex) in 20 hypovolaemic volunteers after withdrawal of 400 ml blood. The total increase of blood volume after 500 ml of 10% hydroxyethyl starch 200/0.5 was 10.19 +/- 1.1 ml/kg (754 ml), after 500 ml of 10% dextran 40 14.10 +/- 1.1 ml/kg (1032 ml), which was significantly higher (p less than 0.01) and in correspondence with the known volume expanding properties of 10% dextran 40. The volume effect after administration of both substances was stable, as demonstrated by the ability to compensate the loss of 400 ml blood for more than 8 hours. The maximal increase of the colloid osmotoc pressure after 10% hydroxyethyl starch 200/0.5 was 5.3 +/- 0.2 mm Hg, after 10% dextran 40 7.2 +/- 0.4 mm Hg and paralleled the increase of the blood volume. After 10% hydroxyethyl starch 200/0.5 an increase of serum amylase was observed. This hyperamylasemia is caused by the formation of a high molecular hydroxyethyl starch-amylase complex, which cannot be eliminated easily. Urinary volume and endogenous creatinine clearance were increased by administration of both colloids. After dextran 40 a pronounced increase of the urinary viscosity occurred.

A correlative study of computed tomography and histology in human and experimental vasogenic cerebral edema.

The computed tomography (CT) scans of nine patients with vasogenic cerebral edema and five monkeys with cryogenic lesions were correlated with histologic findings. The areas of diminished density on the CT scan corresponded to foci of histologically recognizable edema. The attenuation number of the edematous tissue in the human varied from 18.0 to 28.8 Hounsfield units (HU: 1,000 scale) and in the monkey from 27.0 to 34.7 HU. There was a rough correlation between the extent of decrease in the attenuation number and the degree of staining of edematous tissue by the periodic acid-Schiff method. The decreased attenuation number of edematous tissue signifies dilution by edema fluid only when it is above 20 HU or represents a change of less than 12 HU. Lower numbers imply an additional process, possibly transformation of complex into neutral lipids. It is our belief that cerebral edema in the human can be quantitatively studied with the presently available CT scanners.