Giant-cell tumour involving the cranial vault: imaging and treatment.

A giant-cell tumour involving the cranial vault was diagnosed in a 37-year-old man who presented with a large swelling at the vertex. The role of imaging in the diagnosis and treatment of this tumour is described. On CT and MRI the appearances were nonspecific and the diagnosis was established by histological examination after removal of the tumour. A preoperative angiogram showed a tumour blush and before surgery, embolisation was performed via the percutaneous and transarterial routes.

Postoperative aneurysm remnants: endovascular treatment as an alternative to further surgery.

Because further surgery on postoperative aneurysm remnants can be difficult and lead to significant morbidity and mortality, endovascular treatment, using controlled detachable coils, was performed in three patients with such remnants. The endovascular approach was technically more difficult in these cases than in previously untreated patients. In one case, the "remodelling" technique was necessary. Given the successful outcome in these patients, endovascular treatment can be proposed as an alternative to another operation, when further surgery appears too risky or is refused by the patient.

Prevalence of lymphocytic infiltrate in 1400 pituitary adenomas.

To evaluate the prevalence of lymphocytic infiltrate in a large series of pituitary adenomas, we retrospectively studied tumor tissues from 1400 patients. Based on immunocytochemical data, tumors were classified as PRL (n=411), multihormonal (n=310), immunonegative (n=275), ACTH (n=166), GH (n=137), alpha subunit (n=44), FSH and/or LH, (n=42), and TSH (n=15) adenomas. The lymphocytic infiltrate was diagnosed on histological examination and investigated by immunostaining with anti LCA (human leucocyte common antigen), anti CD45RO (human T cell) and anti CD20 (human B cell) antibodies. Lymphocytic infiltrate was present in 40 adenomas (2.9%), 26 females and 14 males, aged 18 to 77 years (mean+/-SD, 37+/-14 years). The tumors were 19 PRL, 8 multihormonal, 4 GH, 4 alpha subunit, 3 ACTH, and 2 immunonegative adenomas. In PRL adenomas, the sex ratio (female/male) and the age were similar in patients with and without lymphocytic infiltrate (2.8 vs. 4.6 and 29+/-6 years vs. 32+/-11 years, respectively). The frequency of lymphocytic infiltrate was similar in PRL, GH, ACTH and multihormonal adenomas, but lymphocytic infiltrate was significantly more frequent in PRL adenoma than in immunonegative adenoma, and in alpha subunit adenoma than in immunonegative, ACTH and multihormonal adenomas. The lymphocytic cells were almost exclusively T cells. We conclude that lymphocytic infiltrates are rare in pituitary adenomas. Their frequency is not statistically different in major categories of secreting adenomas (PRL, GH, ACTH, multihormonal). Their pathophysiological significance remains to be established.

[A peculiar mechanism of hydrocephalus: the "water-hammering" effect]. / Un mécanisme particulier d'hydrocéphalie: l'effet "coup de marteau".

BACKGROUND AND PURPOSE: Aneurysms of the basilar artery can cause hydrocephalus due to compression of the third ventricle or the sylvian aqueduct. The observation of a particular case led to discuss another possible mechanism of hydrocephalus. CLINICAL PRESENTATION AND RADIOLOGICAL STUDIES: An aneurysm of the basilar artery was revealed by an ischemic stroke in a 65-year-old man. Hydrocephalus developed during the following months. The MRI studies showed that it could not be explained merely by a permanent compression. However, the patient improved clearly after a ventriculo-peritoneal derivation. CONCLUSION: The hydrocephalus could be explained by a "water-hammering" effect due to the pulsating blood in the ectatic vessel, which created a cerebrospinal fluid outflow impairment through the third ventricle.

Unusual scotomas after transsphenoidal surgery of a pituitary macroadenoma.

The case of a woman presenting unusual bilateral campimetric defects following transphenoidal surgery is presented. The defects, consisting of a necklace of small round scotomas, disappeared after a corticosteroid treatment. A postoperative inflammatory mechanism may explain these unusual visual field abnormalities.

[Cushing disease in children and in adolescents. Therapeutic results]. / Maladie de Cushing de l'enfant et de l'adolescent. Résultats thérapeutiques.

BACKGROUND: Trans-sphenoidal surgery is currently the treatment of choice for Cushing's disease in children. PATIENTS AND METHODS: The results obtained in 20 consecutive patients referred to the Pediatric Endocrinology Department of hôpital Saint-Vincent-de-Paul are reported. RESULTS: A remission of Cushing's disease was observed in 12/16 (75%) patients in whom surgery was the first treatment. Among these 12 patients, three relapsed (25%) 21 to 80 months after surgery. Four patients were initially treated with steroid synthesis inhibitors: three of those patients were subsequently operated on and their disease remitted. Among the seven patients in whom surgery failed (primary failure or relapse), two were reoperated and also remitted. Taken together, 21 operations were performed and resulted in four immediate failures (19%), three relapses (14%) and 14 long-term remissions (67%, follow-up 40 +/- 35 months). None of the biological, radiological or operative criteria were predictive of the therapeutic results. CONCLUSION: Our results illustrate the efficacy and limits of trans-sphenoidal surgery for Cushing's disease of children and emphasize the need for a very long follow-up of these patients. Treatment of patients in whom surgery has failed (initially or secondarily) is particularly difficult and requires a multidisciplinary approach.

[Late cerebral radionecrosis of cystic aspect]. / Radionécroses cérébrales tardives d'aspect kystique.

Two cases of delayed cerebral radionecrosis with cystic presentation are reported. The patients had received radiation therapy for a cutaneous tumor 16 and 12 years before. Surgical extirpation of the cerebral lesion permitted to confirm the diagnosis and to treat the patients with success. A cystic aspect has been reported rarely in the neuroradiologic descriptions of the cerebral radionecrosis in the literature. Its mechanism is discussed. The treatment of the delayed cerebral radionecrosis is not clearly established, but a cystic presentation is an argument for surgery.

[Magnetic resonance imaging in Cushing disease. Prediction of surgical results]. / Imagerie par résonance magnétique dans la maladie de Cushing. Prédiction des résultats opératoires.

OBJECTIVES: The purpose of the study is to determine if pituitary Magnetic Resonance Imaging (MRI) can predict the outcome of transsphenoidal surgery in patients with Cushing's disease. METHODS: Fifty four patients were divided in three groups according to MRI findings: those with a well circumscribed focal lesion clearly separated from the cavernous sinus (group 1, n = 24), those with adenomas in close contact with the cavernous sinus (group 2, n = 18), and those with no identified lesion (group 3, n = 12). RESULTS: The adenoma is found on the predicted side in 97,6% of the cases with positive MRI. A histologically proven adenoma is found in 96, 100 and 33% of the cases in groups 1, 2 and 3, with successful outcome in 91, 94 and 58% of the patients respectively. In group 2, surgery failed in a patient in whom tumoral tissue surrounded the internal carotid artery, but succeeded in two other cases in whom a MRI involvement of the cavernous sinus was suspected. CONCLUSION: We confirm the excellent localization value of MRI. Failure due to cavernous sinus involvement can be predicted only when MRI shows that the adenomatous tissue surrounds the carotid artery. In cases of negative MRI, the outcome is less favorable.

Treatment of Cushing's disease by transsphenoidal, pituitary microsurgery: prognosis factors and long-term follow-up.

Transsphenoidal pituitary microsurgery is considered as the best treatment of Cushing's disease. However, some recent studies reported disappointing results, leading their authors to suggest the possibility of returning to a first line adrenalectomy treatment. The aim of this study was to evaluate long-term results of transsphenoidal surgery in Cushing's disease, with special interest in factors that could affect the surgical outcome on the one hand and particular attention to surgical endocrine effects on the other. Fifty consecutive patients (34 females, 16 males, mean age 29.64 +/- 1.52 yr) were studied. The median post-operative follow-up was 71.5 months (range 25-219). Clinical, biological, surgical and pathological data between the success and failure groups were compared. Criteria of cure were: normal urinary free cortisol excretion, circadian cortisol rhythm and low dose dexamethasone test. Recovery of corticotroph and somatotroph functions were followed using the insulin test. Particular attention is given to clinical evolution in evaluating other pituitary functions. T4 or FT4, prolactin, E2 in women, testosterone in men, were measured. TRH and LHRH tests were not systematically performed. Only two parameters differed significantly between the cured and failure groups: the size of the adenomas was smaller and the pathological confirmation of the adenoma more frequent in the cured group. One patient had permanent corticotropic failure while two other had impaired response to hypoglycemia with normal cortisol basal levels. No acquired hypothyroidism nor hypogonadism were observed except in a patient who underwent two operations and radiotherapy. Recovery of GH function was slow. Definitive short stature was observed in all the patients whose disease began before the age of 16. Two patients had permanent diabetes insipidus. In conclusion, the most favorable prognosis in transsphenoidal surgery for Cushing's disease is observed in case of microadenoma confirmed by pathological examination. With this treatment, we obtained satisfactory results in Cushing's disease with minimal complications and no necessity of life-long endocrine substitutive therapy.

Adenomatous transformation of the human anterior pituitary is associated with alterations in integrin expression.

Integrins are heterodimeric transmembrane molecules that mediate cell-cell and cell-substratum adhesion. Because alterations in the adhesive properties of tumor cells influence tumor growth and progression, the distribution of different alpha and beta integrin subunits was studied in both the parenchyma and the connective tissue in 6 normal and 25 adenomatous human anterior pituitaries. All normal parenchymal cells expressed the alpha3beta1 and alpha6beta4 integrins. By contrast, in adenomatous parenchymal cells the expression of alpha3beta1 was down-regulated and that of alpha6beta4 abrogated. Neoexpression of alphavbeta3 Occurred in the parenchyma of a subset of adenomas. All normal connective tissue cells expressed the alpha1 and beta1 subunits, a third subunit (alpha5) being present in the normal endothelium. By comparison, all adenomatous stromal cells expressed many more integrin subunits (alpha1, alpha3, alpha5, alphav, beta1 and beta3), adenomatous endothelial cells bearing additional subunits (alpha6, beta4 and beta5). Vitronectin, absent from the normal connective tissue, was constantly observed in the adenomatous stroma. To conclude, compared with cells of the normal gland, adenomatous anterior pituitary cells display a decreased expression of integrins whereas the adenomatous stroma expresses a rich repertoire of integrins. These changes are not related to the secretory type, grade or invasiveness of the adenoma. The resulting alterations in the adhesive properties of adenomatous cells could facilitate their dissemination. Enrichment of the integrin repertoire expressed by the adenomatous vasculature is indicative of its dual nature, systemic and tumoral.

[Surgery of pituitary adenoma]. / La chirurgie des adénomes hypophysaires.

The transsphenoidal approach is used in 99% of pituitary adenomas cases. In this kind of surgery, mechanical and endocrinological complications are rare. The visual disturbances of the huge macroadenomas are easily cured if the patient is operated on before optic atrophy. In secreting adenomas the cure of the hypersecretion depends mainly on the tumor size and is obtained in 90% or 95% of microadenomas. Recurrencies may occur in 10% or 15% of patients; if they are easily diagnosed in hypersecreting tumors they must be systematically searched for in ron secreting adenomas which require a regular follow-up.

Retrobulbar optic nerve cysticercosis. Case report.

The authors report a first case of intraoptic neurocysticercosis in a 12-year-old boy living on Reunion Island. Cysticercosis of the retrobulbar portion of the optic nerve is rare. Because of the patient's age and disturbances in both visual acuity and visual field, it was initially believed to be an optic nerve tumor. Computerized tomography scans and surgical aspects were confirmed by pathological findings. A conservative removal using en bloc orbitotomy showed good functional and aesthetic results.

[Frontal mucoceles of orbital or cerebral extension: therapeutic strategy]. / Mucocèles frontales à extension orbitaire ou cérébrale: stratégie thérapeutique.

The aim of this study was to investigate ten patients who underwent surgery for mucoceles of the frontal sinus. Bicoronal direct access was required by the localization of the mucocele limited to the frontal sinuses, its extension to the orbit and/or the brain and because of the anatomy of the frontal sinuses (large size, lateral horn...) as evidenced at imaging. Direct access to the frontal sinuses was achieved in 9 patients allowing marsupialization associated with repermeabilization of the naso-frontal duct (7 cases) or exeresis of the mucocele by cranialization (2 cases). Mean follow-up is 27 months. Repermeabilization of the naso-frontal duct was effective in 7 out of 8 cases. There were no complications after cranialization and no recurrence has been observed. Drainage of frontal mucoceles is a first intention strategy. In case of complication or recurrence, cranialization of the frontal sinuses would appear to be better than an exclusion-filling procedure.

Fluconazole penetration in cerebral parenchyma in humans at steady state.

We studied fluconazole penetration in the brain in five patients who had a deep cerebral tumor whose removal required the excision of healthy brain tissue. Plasma and brain samples were simultaneously obtained after oral ingestion of 400 mg of fluconazole daily for 4 days (90% of steady state). Fluconazole penetration in healthy cerebral parenchyma was determined. Plasma and brain samples were assayed by high-pressure liquid chromatography. Concentrations in plasma and brain tissue were 13.5 +/- 5.5 micrograms/ml and 17.6 +/- 6.6 micrograms/g, respectively. The average ratio of concentrations in the brain and plasma (four patients) was 1.33 (range, 0.70 to 2.39). Despite the lack of data concerning the penetration of fluconazole in brain abscesses, these results should permit the use of a daily dose of 400 mg of fluconazole in prospective clinical studies that evaluate the effectiveness of this drug in the treatment of brain abscesses due to susceptible species of fungi.

Fibronectin isoforms are differentially expressed in normal and adenomatous human anterior pituitaries.

The expression of fibronectin (FN) isoforms containing the extradomains A and B (ED-A+ and ED-B+ FNs) as well as a differentially O-glycosylated oncofetal form of the protein (onf-FN) was investigated in 6 normal human anterior pituitaries and 25 human pituitary adenomas. In normal tissue, immunohistochemical experiments showed the presence of FN molecules lacking the extradomains A and B (ED-A- and ED-B- FNs) without onf-FN immunoreactivity. These proteins were localized in the connective tissue compartment and especially in the vessel walls. Analysis of FN mRNA demonstrated an in situ synthesis of ED-A- and ED-B- FNs in the normal anterior pituitary. By contrast, in the adenomas, immunoreactivity for ED-A+ FN was observed in all cases. ED-B+ and onf-FN immunoreactivities were observed in 14 and 8 adenomas, respectively, regardless of the type, grade or invasiveness of the adenomas. ED-A+ FN mRNA was expressed in all adenomas studied, and ED-B+ FN mRNA was present in ED-B+ immunoreactive cases only. In pituitary adenomas, these 3 forms of FN were specifically associated with the endothelium and vascular smooth-muscle cells. Our results demonstrate that the processes of remodelling of the connective tissue compartment that occur in adenoma angiogenesis are associated with pre- and post-translational alterations of FN synthesis leading to the expression of ED-A+, ED-B+ and oncofetal FNs.

[Surgical anatomy of the parathyroid glands. Apropos of 200 cases. Practical implications]. / Anatomie chirurgicale des glandes parathyroïdes. A propos de 200 cas. Implications pratiques.

An operative procedure aimed at decreasing the risk of post-surgery hypothyroidism was proposed on the basis of parathyroid gland surgical anatomy described from a retrospective study of 200 thyroidectomies performed by the same operator It is almost always possible to visualize 1 or parathyroids during thyroid procedures. This fact makes it possible to reduce the rate of definitive hypoparathyroidism to less than 5% in surgery for invasive cancer of the thyroid and to 1% for non-invasive cancers requiring total thyroidectomy and uni or bilateral mediastinorecurrent curettage.