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Biomedical subjects

B W Scheithauer

Publications and source records attributed to B W Scheithauer.

At least 361 records · Page 20Linked to original sources

Lymphocytic hypophysitis with isolated corticotropin deficiency.

In a 32-year-old woman, acquired, isolated corticotropin deficiency resulted from postpartum lymphocytic hypophysitis. The literature suggests that lymphocytic hypophysitis may cause acquired deficiencies of anterior, and possibly posterior, pituitary hormones. Immunologic evaluation of our patient failed to uncover anticorticotroph antibodies. Prompt recognition of this potentially fatal condition is important because of the availability of effective treatment.

Adrenocorticotropic Hormone↗

Reassessment of malignant "angioendotheliomatosis". Evidence in favor of its reclassification as "intravascular lymphomatosis".

Malignant angioendotheliomatosis (MAE) is a lethal intravascular proliferation which has been thought to be of endothelial origin. In order to characterize its cellular nature, we studied 15 cases of MAE immunocytochemically, using antisera for factor VIII-related antigen, cytokeratin, epithelial membrane antigen, vimentin, blood group isoantigens, thoracic duct lining cell antigens (TDLCA), common leukocyte antigen, and Ulex europaeus I lectin. In 14 of 15 cases, common leukocyte antigen was observed in malignant intravascular cells. Similar reactivity for factor VIII-related antigen was present in 14 cases, but was largely restricted to cells enmeshed in fibrin-platelet thrombi, and probably represents adsorption of platelet-derived factor VIII by tumor cells. All cases failed to bind Ulex europaeus lectin and lacked immunoreactivity for TDLCA, cytokeratin, epithelial membrane antigen, and blood group isoantigens; two manifested positivity for vimentin. Immunofluorescent microscopy of frozen tissue in one case showed monoclonal IgM-kappa immunoglobulin on the surfaces of tumor cells. Electron-microscopic study of three cases disclosed a predominant cell type lacking features of epithelial or endothelial differentiation; a minor cell population displayed endothelial characteristics and was thought to be reactive. Four patients with typical MAE also had extravascular large-cell lymphoma in lymph nodes, spleen, adrenal glands, stomach, or soft tissues. Six patients showed clinical evidence of autoimmune disease. These results suggest that MAE displays lymphoid rather than endothelial differentiation.

Aged↗

Plurihormonal pituitary adenomas.

Plurihormonal adenomas of the pituitary, ie, tumors that engage in the production of unusual combinations of hormones, represent approximately 10% to 15% of all adenomas. Such tumors comprise in excess of 50% of adenomas in the setting of acromegaly and occur with somewhat greater frequency in childhood and adolescence than in adulthood. Eight percent are associated with multiple endocrine neoplasia, type I. The most common variant of plurihormonal adenoma produces growth hormone, prolactin, and one or more glycoprotein hormones, the most common being TSH. Clinical effects most often reflect the presence of growth hormone, and to a lesser extent, prolactin cells; expression of glycoprotein hormone production is rare. The tumors are more often macroadenomas (80%) than microadenomas (20%) and demonstrate gross invasion in 50% of cases. Plurihormonal adenomas may be ultrastructurally monomorphous, bimorphous, or trimorphous; thus, one morphologic cell type may elaborate several hormones.

Adenoma↗

Meningio-angiomatosis: a report of six cases with special reference to the occurrence of neurofibrillary tangles.

We report six cases of meningio-angiomatosis, a disorder of the cerebral cortex of probable malformative origin frequently associated with neurofibromatosis and either asymptomatic or associated with a seizure disorder. The patients, three males and three females, ranged from 10 to 70 years of age at diagnosis. The lesion was an incidental autopsy finding in two patients; four subjects had a seizure disorder which improved following surgical resection of the cortical lesion. In five instances the process was unifocal; one patient with neurofibromatosis had multifocal involvement. Grossly, the lesions were firm, sharply demarcated, transcortical plaques with leptomeningeal calcifications in five cases. The cortical plaques showed characteristic features of meningio-angiomatosis, i.e. a circumscribed, proliferating microvasculature with perivascular meningothelial cell proliferation and fibrosis. In five cases Alzheimer's neurofibrillary change in neurons was present both within the plaques and in the surrounding cortex. Neither senile plaques nor granulovacuolar degeneration were noted. Electron microscopy in one case demonstrated typical intraneuronal accumulations of neurofilaments with regular constrictions. Intracortical vessels lined by endothelial cells with tight junctions were surrounded by pericytes and meningothelial cells. The significance of neurofibrillary tangles described in a variety of disorders, and the factors stimulating their production in the present cases are unknown.

Adolescent↗

Chronic syndrome of inappropriate antidiuretic hormone secretion and hypertension in a patient with olfactory neuroblastoma. Evidence of ectopic production of arginine vasopressin by the tumor.

A 28-year-old man with the chronic syndrome of Inappropriate antidiuretic hormone secretion and hypertension was found to have an olfactory neuroblastoma. We demonstrated evidence of elevated circulating arginine vasopressin levels, significantly elevated arginine vasopressin and vasopressin neurophysin levels in the tumor extract, and immunohistochemical staining for arginine vasopressin and vasopressin neurophysin in the tumor cells. The patient's clinical syndrome, including hypertension, resolved following subtotal removal of the tumor and radiation therapy. This study identified olfactory neuroblastoma as a definite cause of ectopic arginine vasopressin secretion causing the syndrome of inappropriate antidiuretic hormone secretion.

Adult↗

Gliomatosis peritonei.

Gliomatosis peritonei, the occurrence of mature glial peritoneal implants, is a rare complication that is observed almost exclusively in the setting of ovarian teratoma. The clinicopathologic features of four such cases associated with ovarian teratoma of all grades are described herein. Gliomatosis peritonei is a benign condition. Although its presence is believed to suggest a more favorable prognosis in cases of high-grade ovarian teratoma, this was not found in one of our cases. Extensive histopathologic sampling of peritoneal implants is essential to exclude the presence of other teratomatous or germ cell elements, a negative factor in prognosis.

Adolescent↗

Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases.

The study involved 77 myxopapillary ependymomas of the spinal cord encountered during a 60-year period (1924-1983). This variant of ependymoma was, with few exceptions, limited to the lumbosacral region, particularly the filum terminale. The male:female ratio was 1.7:1, and the mean age at diagnosis was 36.4 years (range, 6-82); at presentation, 15 (19%) of the patients were in the first two decades of life. The duration of symptoms ranged from 1 month to 30 years; the most frequent complaint was low-back pain, and eight patients had undergone prior "disc surgery." Generally, myelographic block was disclosed. Preoperative cerebrospinal fluid protein levels averaged 2462 mg/dl. Myxopapillary ependymomas are slow-growing tumors that show no significant tendency to histologic dedifferentiation. Despite some variation in cytologic features and the presence of atypia and modest mitotic activity in most cases, the gross characteristics of the tumors appear to be of greater prognostic significance than the histologic features. Tumors that were encapsulated (25%) and amenable to intact, total surgical removal had a recurrence rate of 10%, whereas those that were removed either piecemeal (34%) or subtotally (41%) had recurrence rates of 19%. Overall survival, however, was more closely related to residual disease; total removal of tumor, whether intact (encapsulated) or piecemeal, resulted in longer survival (19 years) than did subtotal resection (14 years). Patients who died (6.5%) did so after a prolonged course marked by multiple recurrences. Radiotherapy may be of particular benefit to patients whose tumors are not amenable to intact total removal.

Adolescent↗

Prolactin cell carcinoma of the pituitary. Clinicopathologic, immunohistochemical, and ultrastructural study of a case with cranial and extracranial metastases.

A patient with a primary adenohypophyseal neoplasm who had a long course marked by multiple surgical resections, radiation therapy, and high-dose dopamine agonist therapy developed local invasion as well as cranial and extracranial osseous metastatic lesions. The serum prolactin levels were greatly elevated, and immunohistochemical studies demonstrated prolactin in the cytoplasm of primary and metastatic tumor cells. Ultrastructural features of lactotrophic differentiation, including misplaced granule exocytosis, were observed. This is the third reported case of prolactin cell carcinoma that metastasized despite high-dose dopamine agonist therapy. Analysis of the patient's serum prolactin showed no abnormality in the chromatographic profile of biologic activity.

Biological Assay↗

The hypothalamus and pituitary in cerebral gigantism. A clinicopathologic and immunocytochemical study.

Cerebral gigantism, or Sotos' disease, is a rare disorder of unknown cause characterized by the early onset of excessive growth, acromegalic features, and some degree of mental retardation. Although several endocrinologic abnormalities have been described in such patients, none has been specific or sufficient to explain the clinical features. Our report of the endocrinologic and pathologic aspects in a young woman with Sotos' disease includes the first microanatomic study of the hypothalamus and immunocytochemical examination of the pituitary gland in this disorder.

Autopsy↗

Pituitary adenomas associated with hyperprolactinemia: a clinical and immunohistochemical study of 97 patients operated on transsphenoidally.

Immunohistochemical studies were performed on the tumors of 97 of 100 patients who had undergone an operation for a presumed prolactin-secreting pituitary adenoma; no tissue was available for study in the other 3 patients. Appropriate immunohistochemical studies were done to identify the presence of growth hormone, prolactin, adrenocorticotropic hormone, luteinizing hormone, follicle-stimulating hormone, and thyroid-stimulating hormone within the adenoma cells. The presence of a prolactin-producing pituitary adenoma was confirmed in 91 patients, 3 of whom had an adenoma that consisted of cells that contained prolactin and growth hormone. One other patient, not counted among the 91 with prolactinoma, had lactotrope and thyrotrope hyperplasia. Among the five patients whose adenoma did not contain prolactin cells, four had a null cell adenoma and one had a tumor consisting of follicle-stimulating hormone and luteinizing hormone immunoreactive cells. On the basis of preoperative serum prolactin values in these patients, we concluded that moderately increased values (30 to 200 ng/ml) of serum prolactin are not a reliable guide for determining whether a prolactin-producing pituitary adenoma is present, whereas levels exceeding 200 ng/ml are usually associated with a prolactin-secreting tumor.

Adenoma↗

Glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultrastructural study.

Necrolytic migratory erythema has been considered to be a paraneoplastic dermatosis. Although some individuals have had no associated neoplasm, a pancreatic glucagonoma is typically found in the vast majority. We describe a 71-year-old woman with findings strongly suggestive of the glucagonoma syndrome. The plasma glucagon level was modestly elevated. Special studies of the pancreas, including computerized axial tomography and selective angiography, did not demonstrate a tumor. Surgery, however, revealed a 5-cm tumor in the head of the pancreas. Although immunocytochemical stains showed only rare, weakly reactive cells, the electron microscopic study confirmed the diagnosis of an islet cell tumor and revealed characteristic morphologic features of glucagonoma.

Adenoma, Islet Cell↗

Nimodipine improves outcome when given after complete cerebral ischemia in primates.

Twenty-seven pigtailed monkeys (Macaca nemestrina) were subjected to 17 min of complete cerebral ischemia followed by 96 h of intensive care treatment. Fourteen of the monkeys were assigned randomly to the treatment group and received nimodipine 10 micrograms . kg-1 5 min postischemia followed by 1 microgram . kg-1 . min-1 for 10 h. Six monkeys (three treated) failed to meet preestablished protocol criteria and were excluded. The remaining treated and untreated monkeys were well matched for age, sex, and other physiologic variables. Neurologic outcome at 96 h postischemia was significantly better in the nimodipine-treated monkeys than in the controls. Eight of the 11 treated animals had an apparent normal level of consciousness; four of these had no detectable neurologic deficits and a fifth had only a slight motor apraxia. Only two of the 10 untreated animals had an apparent normal level of consciousness, and all had major neurologic deficits. Histopathologic examination showed variable ischemic neuronal change and infarction to involve gray matter in distal arterial perfusion zones. Significant white matter changes were not observed. A histopathologic scoring system yielded a significantly better mean score for the treated group than for the untreated group, and there was significant correlation between neurologic function and histopathologic findings. The authors conclude that nimodipine improves the neurologic outcome when given after an episode of complete cerebral ischemia in primates, and they recommend controlled clinical trials in patients resuscitated after cardiac arrest.

Animals↗

CT of the sella turcica after transsphenoidal resection of pituitary adenomas.

A retrospective review of 120 patients undergoing transsphenoidal surgery for pituitary adenomas revealed that computed tomography (CT) was less sensitive and less specific than hormonal methods in identifying residual functioning adenomas. However, CT was the only useful method of evaluating nonfunctioning tumors, including pseudo-prolactinomas. Enlargement of the pituitary stalk, when seen on preoperative CT, was 100% predictive of "cure" if the enlargement returned to normal size on a remote follow-up scan (n = 8) and was 100% predictive of residual tumor if the enlargement persisted or evolved (n = 8). Resolution of stalk displacement was the next most reliable predictor of cure (91%, n = 11), but persistent displacement was less reliable than abnormal intrasellar enhancement in predicting the presence of residual tumor (71%, n = 28, vs. 81%, n = 26). Enhancement in the postoperative sella by other than normal pituitary gland was presumed to be due to inflammation in 19% of patients. Inflammatory enhancement was observed in the presence of autograft and homograft muscle plugs and, unlike enhancement due to untreated tumors, was observed to decrease in size and intensity with time. Intrasellar enhancement was an unreliable criterion of success or failure in cases of microadenoma. All 14 patients with functioning adenomas and preoperative parasellar tumor extension had persistent tumor at postoperative evaluation. Of the 47 patients with resected functioning adenomas who had CT scans showing empty or partly empty sellas after operation, 22 (47%) had hormonally detectable residual tumor.

Adenoma↗

The pathogenesis of acromegaly. Clinical and immunocytochemical analysis in 75 patients.

A series of 75 patients with acromegaly and immunocytochemically characterized pituitary adenomas has been analyzed. Tumors secreting growth hormone (GH) only were found in 21% of cases. The remainder had tumors immunoreactive for more than one pituitary hormone: GH and prolactin in 31%; GH, prolactin, and glycoprotein in 40%; and GH and glycoprotein in 8%. Microadenomas were surgically treated in 17 patients with a success rate of 82%. Overall, normalization of basal GH secretion (to less than or equal to 5 ng/ml) was achieved in 54% of cases. The implications of these findings for the pathogenesis and neurosurgical management of acromegaly are discussed.

Acromegaly↗

Metastasis to pituitary adenoma.

Neoplasm to neoplasm metastasis is a medical curiosity. We report two cases of adenocarcinoma metastatic to pituitary adenoma. In both, abrupt progression of symptoms referable to the sellar region was noted and followed by the death of the patient.

Adenoma↗

Pituitary gland in hypothyroidism. Histologic and immunocytologic study.

Primary hypothyroidism is associated with hypertrophy and hyperplasia of thyrotropic cells. In addition, pituitary adenomas that produce thyroid-stimulating hormone occur in both hypothyroidism and hyperthyroidism. The relationship between thyrotropic hyperplasia and adenoma formation is, however, unsettled. We summarize the results of a histologic and immunocytologic study of the pituitary glands of 64 patients with long-standing primary hypothyroidism in an effort to characterize the changes in thyrotropic cells as related to the duration and severity of disease, to therapy, and to the development of thyrotropic adenomas. Diffuse and nodular thyrotropic cell hyperplasia was noted in 69% and 25% of glands, respectively. A crude correlation was observed between the degree of thyrotropic cell hyperplasia and the relative lack of thyroid hormone replacement therapy. In 12% of glands, tumorlet formation was observed, perhaps representing an intermediate stage between nodular hyperplasia and the development of microadenoma. Twelve adenomas were noted, five of which contained thyroid-stimulating hormone immuno-reactive cells. Although thyroid hormone deficiency seemed to selectively affect thyrotropic cells, lactotropic hyperplasia was observed in 20% of patients; the mechanism accounting for prolactin cell hyperplasia remains obscure.

Adenoma↗