Intracranial vascular sarcomas. A series of 12 cases and a review of the literature.
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Biomedical subjects
Publications and source records attributed to F Grisoli.
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A series of 28 cases of meningioma of the tuberculum sellae is reviewed. This tumor was most frequent in the fifth decade, with a clear predominance in women. Neurological and endocrine deficits were minimal, whereas ophthalmologic signs were always present. Visual deficits were bilateral in cases with more than a 1-year history. Osteoma of the tuberculum sellae was rare and had no correlation with tumor size. Angiography of the internal carotid artery was found useful in demonstrating vascular displacements and tumor blush. Computed tomography was the most reliable diagnostic tool. All patients underwent a unilateral pterional craniotomy using the operating Outside of patients with total blindness, improvement of visual acuity was the rule, even in cases of long-term duration. Best results were obtained in patients operated upon within 1 year of the onset of visual symptoms.
Six cases of hyperthyroidism secondary to thyroid-stimulating hormone (TSH)-hypersecreting pituitary adenomas are presented (five females and one male). Hyperthyroidism presenting with diffuse goiter was associated with hyperprolactinemia in three cases. Elevation of triiodothyronine (T3) and thyroxine (T4) plasma levels was associated with TSH levels ranging between 2 and 2,000 microU/mL. Polytomography and computed tomography scanning with coronal views revealed four invasive and two enclosed tumors. Two patients underwent transfrontal operations, four underwent transsphenoidal operations, and one underwent both. Two cases of enclosed adenoma were cured primarily by a transsphenoidal approach without additional pituitary deficit. In four cases of invasive adenomas, operations and radiation therapy resulted in three failures and one cure. In cases presenting with hyperthyroidism and detectable TSH, early neuroradiological studies are indicated, as biological cure can be achieved by the transsphenoidal approach. Early treatment is also advocated because of the tendency for these tumors to become invasive (four out of six tumors).
Cylindromas are rarely encountered in the neurosurgical field. Four cases of this rare tumor are presented. Conventional and computerized tomography scanning were most useful in establishing the diagnosis; angiography did not provide any further information. Because these tumors are well demarcated from surrounding structures, even in cases of recurrence, surgery is the treatment of choice. Radiation therapy is useful in the postoperative management. Long survival times with multiple recurrences are the rule.
A series of 100 patients with acromegaly who were operated on using the transsphenoidal microsurgical approach is presented along with a review of the literature. Emphasis is placed on long-term follow-up to assess the value of the technique. The authors caution against early normalization of growth hormone as a criterion for biologic cure in that cases of late recurrence are presented. It is proposed that 5 ng/mL baseline growth hormone values with normal dynamic testing is the most reliable way to ascertain biologic cure. Using these criteria, a cure rate of 78% for enclosed and 33% for invasive adenomata was obtained. No long-term recurrence was seen in patients considered cured along these lines.
Thyrotropin-releasing hormone (TRH) stimulates the prolactin (PRL) release from normal lactotrophs or tumoral cell line GH3. This effect is not observed in many patients with PRL-secreting tumors. We examined in vitro the PRL response to TRH on cultured human PRL-secreting tumor cells (n = 10) maintained on an extracellular matrix in a minimum medium (DME + insulin, transferrin, selenium). Addition of 10(-8) M TRH to 4 X 10(4) cells produced either no stimulation of PRL release (n = 6) or a mild PRL rise of 32 +/- (SE) 11% (n = 4) when measured 1, 2 and 24 h after TRH addition. When tumor cells were preincubated for 24 h with 5 X 10(-11) M bromocriptine, a 47 +/- 4% inhibition of PRL release was obtained. When TRH (10(-8) M) was added, 24 h after bromocriptine, it produced a 85 +/- 25% increase of PRL release (n = 8). This stimulation of PRL release was evident when measured 1 h after TRH addition and persisted for 48 h. The half maximal stimulatory effect of TRH was 2 X 10(-10) M and the maximal effect was achieved at 10(-9) M TRH. When tumor cells were pretreated with various concentrations of triiodothyronine (T3), the PRL release was inhibited by 50% with 5 X 10(-11) M T3 and by 80% with 10(-9) M T3. Successive addition of TRH (10(-8) M) was unable to stimulate PRL release at any concentration of T3. The addition of 10(-8) M estradiol for up to 16 days either stimulated or had no effect upon the PRL basal release according to the cases. In all cases tested (n = 4), preincubation of the tumor cells with estradiol (10(-8) M) modified the inhibition of PRL release induced by bromocriptine with a half-inhibitory concentration displaced from 3 X 10(-11) M (control) to 3 X 10(-10) M (estradiol). These data demonstrate that the absence of TRH effect observed in some human prolactinomas is not linked to the absence of TRH receptor in such tumor cells. TRH responsiveness is always restored in the presence of dopamine (DA) at appropriate concentration. This TRH/DA interaction seems specific while not observed under T3 inhibition of PRL. Furthermore, estrogens, while presenting a variable stimulatory effect upon basal PRL, antagonize the dopaminergic inhibition of PRL release.
In an effort to better characterize the ultrastructural, morphometric, and immunocytochemical changes induced by 10(-8) M bromocriptine (BR), tumor cells from three surgically removed PRL-producing pituitary adenomas were cultured on an extracellular matrix in serum-free medium. In each instance, the treated cultures were compared to control cells at the end of 24 h and 16 days. PRL RIAs were performed on culture medium. A decrease in cell and nucleus surface area was found on day 16 in two cultures. This supports the well known shrinkage of BR-treated PRL-producing adenomas. BR induced no change in these parameters in the tumor from a third patient who was partly resistant to the drug. Changes in the secretory process were discernible as of day 1 in all three tumors, with a dramatic reduction of exocytosis and intracellular accumulation of PRL-immunoreactive granules. This induced delayed inhibition of protein synthesis, demonstrated by preembedding immunocytochemistry on day 16. These results, obtained for the first time in human PRL-producing adenomas, are informative as to the subcellular events subsequent to short term BR treatment and illustrate that secretory inhibition and tumor shrinkage are not necessarily linked.
Prolactin dosage in delayed growth and puberty leads to the diagnosis of pituitary prolactinoma in a 14 years old boy. Adenoma's size, visual disturbance leads us to elect surgical treatment but persistent hyperprolactinemia after surgery requires medical treatment with bromocriptine.
Eleven cases are reported of lateral lumbar herniated disc, an anatomical form presenting fairly atypical features and of relatively recent discovery. The patients presented with a highly painful single root lesion usually affecting L4 and L5 roots. Saccoradiculography was negative in almost all cases whereas computed tomography imaging allowed easy diagnosis of a lateral herniated disc, and demonstrated its foraminal or extraforaminal site. An appropriate surgical approach was thus possible, extremely lateral forms requiring an extraisthmian approach.
A case of suprasellar hemangioblastoma attached to the pituitary stalk is reviewed. The patient presented with amenorrhea-galactorrhea and a highly vascularized suprasellar lesion. Though the exact diagnosis was not made before the operation, the authors emphasize the importance of angiography, which had significant features regarding the diagnosis.
A 43-yr-old woman, who had previously had a subtotal thyroidectomy, presented with hyperthyroidism and amenorrhea-galactorrhea due to a pituitary adenoma secreting TSH, TSH-alpha, and PRL. Her serum T4 concentration was 14 micrograms/dl; T3, 5.7 ng/ml, and TSH, 19-33 microU/ml. Serum TSH was not altered by TRH stimulation or T3 suppression. Basal plasma PRL levels were 19-27 ng/ml and plasma PRL doubled after TRH stimulation. A 900-mg pituitary tumor, removed by transphenoidal surgery, was studied in cell culture. After dispersion, tumor cells were maintained on an extracellular matrix produced by bovine corneal endothelial cells in a defined serum-free medium. The hormones released in the culture medium were analyzed by high pressure gel chromatography. Three fractions of tumor TSH were found, with respective apparent mol wts of 45,000 (11%), 28,000 (70%), and 20,000 (19%). Tumoral PRL eluted as a single peak of apparent mol wt of 24,000. Pharmacological studies of TSH, TSH-alpha, and PRL release using thyroid hormones (T3), dopamine agonist (bromocriptine), TRH, and cholera toxin yielded the following results: 1) T3 after 3 days of incubation produced a dose-dependent inhibition of TSH, TSH-alpha, and PRL release. Maximal inhibition (81%) was obtained at 10(-9) M and half-maximal inhibition at 4-6 X 10(-11) M. 2) Bromocriptine produced rapid and partial inhibition of hormone release. Maximal inhibition (51%) was obtained at 10(-8) M and half-maximal inhibition at 5 X 10(-10) M. 3) TRH at 10(-8) M concentration significantly stimulated PRL release but it had no effect on TSH release. 4) Adenylate cyclase activation by 10(-11) M cholera toxin increased TSH (152%), TSH-alpha (150%), and PRL (220%). Immunohistochemical analysis of serial 2 micron sections of the tumor showed that: 1) TSH-alpha immunoreactive cells were the most numerous, 2) TSH-beta positive cells were always positive for TSH-alpha, 3) PRL immunoreactivity was found either uniquely in some cells and colocalized with TSH-alpha immunoreactivity in other cells. However, by electron microscopy, the tumor cells were thyrotrophs. These data indicate that in this patient's tumor: 1) cells secreting TSH were responsive in vitro to near physiological concentrations of thyroid hormones. 2) The colocalization of PRL and TSH-alpha immunoreactivities in some cells raises the possibility either of fusion of differentiated pituitary cells synthesizing distinct hormones or of transformation of less differentiated multipotential pituitary cells.
Four patients with tentorial arteriovenous malformations (AVM's) were treated surgically. The operative findings in the first case suggested that clipping of the draining vein close to the AVM may result in complete cure. The three subsequent cases were treated with this technique. The clinical and radiological implications are discussed.
The authors report a case of neuro-endocrine sarcoidosis, associating panhypopituitarism , diabetes insipidus, and involvement of the optic chiasma with behavioural changes, chronic meningitis and a diffuse pyramidal syndrome. The diagnosis was suggested by the presence of mediastinal lymphadenopathy and confirmed during a surgical procedure to conserve the optic nerves. The clinical course on corticosteroids was unfavourable because of iatrogenic cushing' syndrome and steroid dependence . It was not possible to withdraw steroids, even with the use of immunosuppressors (Methotrexate).
Two purely subdiaphragmatic intrasellar meningiomias are described. The rarity of this unusual intrasellar tumor is stressed, and the differential diagnosis of intrasellar meningioma versus pituitary adenoma is discussed. The good results of the rhinoseptal route for surgery are emphasized.
A technique for the removal of sphenoid blocks from cadavers and for selective injection of the hypophyseal arteries of these specimens is described. Results of such injections are presented, with emphasis on the role of the inferior hypophyseal artery (IHA). The IHA was found to be the most important artery supplying the pituitary gland, and in particular, the structures involved in production, transportation, and storage of the antidiuretic hormone. The literature pertinent to the arterial blood supply of the normal pituitary gland is reviewed.
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