National Cancer Institute®
Last Modified: August 1, 2002
UI - 7509101
AU - Kontogeorgos G; Asa SL; Kovacs K; Smyth HS; Singer W
TI - Production of alpha-subunit of glycoprotein hormones by pituitary somatotroph adenomas in vitro.
SO - Acta Endocrinol (Copenh) 1993 Dec;129(6):565-72
AD - Department of Pathology, St. Michael's Hospital, Toronto, Ontario, Canada.
Somatotroph adenomas of the pituitary secrete growth hormone in excess and are associated with acromegaly. Morphologically, they can be separated into two entities, densely and sparsely granulated variants. It has been shown that a number of somatotroph adenomas produce alpha-subunit of glycoprotein hormones; however, it is not clear whether alpha-subunit production correlates with tumor cell morphology. We studied 32 surgically removed pituitary somatotroph adenomas in tissue culture to determine structure-function correlations of growth hormone and alpha-subunit production. All tumors were classified on the basis of detailed histological, immunocytochemical and electron-microscopic studies. Fifteen tumors were densely granulated and 17 were sparsely granulated. In addition to growth hormone, all 15 densely granulated tumors released alpha-subunit in vitro, whereas of the 17 sparsely granulated tumors only 4 released alpha-subunit; moreover, the mean baseline levels of alpha-subunit were significantly higher in densely granulated adenomas than in sparsely granulated adenomas. Parallel response of release of both hormones was found during stimulation with growth hormone-releasing hormone or thyrotropin-releasing hormone and during suppression with somatostatin or bromocriptine in densely granulated tumors. alpha-subunit response to stimulation or suppression could not be determined with significance in sparsely granulated tumors because of low basal levels. The results indicate that alpha-subunit production and release is characteristic of densely granulated somatotroph adenomas and that alpha-subunit is coregulated with growth hormone by adenohypophysiotropic substances; in contrast, alpha-subunit production, by sparsely granulated somatotroph adenomas is rare and, when present, much lower in quantity.(ABSTRACT TRUNCATED AT 250 WORDS)
UI - 11922704
AU - Chen HJ
TI - The neurological abnormalities and operative findings in the transcallosal approach for large juxtasellar-ventricular craniopharyngiomas.
SO - J Clin Neurosci 2002 Mar;9(2):159-63
AD - Department of Neurosurgery, Chang Gung University and Medical Center at Kaohsiung, Taiwan. email@example.com microsurgical resection for craniopharyngiomas. Of6 adult patients, large tumours (>4 cm) with extension from intrasellar or parasellar space to the foramen of Monro were found. There were 4 male and 2 female patients with ages from 18 to 44 years (mean 32.4 years). All of them underwent one-stage interhemispheric transcallosal total exc ision of tumours. This approach affords good overhead view of tumours and surrounding structures.Preoperatively, decreased visual acuity and restriction in the visual field was found in 5 patients. Studies of anterior pituitary function revealed far below the normal range in all patients. Polyuria was noted in 4 patients. All of 6 patients showed abnormally high levels of blood cholesterol and triglyceride and 4 patients were obese. Tumours with extension into the pituitary fossa were found in 2 cases. A relatively caudal location in the third Ventricle was found in case 6, who showed better preoperative visual function and no polyuria. There were no major surgical complications encountered. One patient with high cholesterol (731 mg%) died of acute pancreatitis 6 months after surgery.For large craniopharyngiomas without pre-chiasmatic extension, proper treatment could be achieved under substitution therapy and one stage transcallosal total excision of tumours with preservation of important neural structures. Copyright 2002, Elsevier Science Ltd. All rights reserved.
UI - 11922715
AU - Hori E; Akai T; Kurimoto M; Hirashima Y; Endo S
TI - Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella.
SO - J Clin Neurosci 2002 Mar;9(2):196-9
AD - Department of Neurosurgery, Toyama Medical and Pharmaceutical University, Japan.
We present a case of growth hormone (GH)-secreting ectopic pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. It has been well known that acromegaly is sometimes associated with an empty sella. However, such a case usually has a macroadenoma and an empty sella that is large. The authors considered the possible mechanisms of the association between a normal-sized empty sella and an ectopic pituitary adenoma in the sphenoid sinus as the following. Primary empty sella existed originally, and the pituitary adenoma developed later. The adenoma extended into the sphenoid sinus because of the pulsatile intracranial cerebrospinal fluid pressure. Copyright 2002, Elsevier Science Ltd. All rights reserved.
UI - 11925822
AU - Tanis AA
TI - [Diagnostic image (72). An adult woman with coarsening of the face. Acromegaly]
SO - Ned Tijdschr Geneeskd 2002 Mar 16;146(11):542; discussion 542
UI - 12124824
AU - Ma W; Ikeda H; Yoshimoto T
TI - Clinicopathologic study of 123 cases of prolactin-secreting pituitary adenomas with special reference to multihormone production and clonality of the adenomas.
SO - Cancer 2002 Jul 15;95(2):258-66
AD - Division of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
BACKGROUND: Prolactinoma is the most invasive type of pituitary adenoma and is generally believed to be well-differentiated adenoma and to produce only prolactin (PRL). The factors related to the various biologic behaviors occurring in patients of different ages and sexes await clarification. Since different immunophenotypes of adenoma may show different biologic behaviors and responses to medical agents, the authors examined hormone production and tried to clarify the clonality of plurihormonal prolactinoma. METHODS: Clinicopathologic factors were studied in 123 patients with prolactinomas (40 males and 83 females). The specimens were fixed in either 10% neutral buffered formalin or 70% alcohol and used for light microscopy. Alcohol-fixed tissue was used to extract DNA from 26 samples obtained from female patients for human androgen receptor gene (HUMARA) assay. RESULTS: Sixty one cases (50%) were pure prolactinoma and 62 cases (50%) were plurihormonal prolactinoma. Spearman rank correlation analysis revealed a significant relationship between age and serum PRL level (P = 0.0002), age and tumor volume (P < 0.0001), and tumor volume and serum PRL level (P < 0.0001). Multiple regression analysis showed a significant correlation only between tumor volume and serum PRL level. The Mann-Whitney U test revealed that prolactinomas associated with higher PRL levels, larger adenomas, and higher ages were significantly more invasive to the cavernous sinus and that male patients had significantly higher PRL levels and larger adenomas. The HUMARA assay disclosed that 11 of 13 plurihormonal prolactinomas (85%) were compatible with monoclonal origin. CONCLUSIONS: The current results suggest that not only can various hormones other than PRL be secreted by prolactinoma, but also that most multihormone-producing prolactinomas are monoclonal in origin. Copyright 2002 American Cancer Society.DOI 10.1002/cncr.10676
UI - 12124840
AU - Juven Y; Sadetzki S
TI - A possible association between ionizing radiation and pituitary adenoma: a descriptive study.
SO - Cancer 2002 Jul 15;95(2):397-403
AD - Cancer Epidemiology Unit, Gertner Institute, Sheba Medical Center, Tel Hashomer, Israel.
BACKGROUND: Despite the recognition of ionizing radiation as a causal risk factor for a variety of solid tumors (including brain tumors), to date, such an association with pituitary adenoma (PA) has not been demonstrated. METHODS: To evaluate a possible association between past exposure to radiation and the occurrence of PA, the authors reviewed about 4900 medical records of patients who had been irradiated in childhood for tinea capitis. An additional search for patients was performed using the Israel Cancer Registry. The average radiation dose to the pituitary gland was estimated as 0.56 grays, and, for all patients, a meticulous validation of the irradiation was performed. RESULTS: A group of 16 patients who developed symptomatic PA after childhood exposure to radiotherapy were identified. Overall, the clinical and demographic characteristics of these patients were similar to other series reported in the literature. There was an apparently high rate of second primary tumors (25%), all of them in the irradiated area, diagnosed among this group. The methodologic issues that limit the demonstration of a possible association between radiation and PA and the epidemiologic and experimental findings in the literature are discussed. CONCLUSIONS: In view of the ample amount of evidence identifying low-dose ionizing radiation as a risk factor for a number of intracranial tumors as well as for tumors arising in endocrine organs, a radiation immunity of the pituitary gland is difficult to accept. Hence, the authors suggest that this series should be considered as preliminary observation that supports the role of ionizing radiation in the development of this tumor. Copyright 2002 American Cancer Society.DOI 10.1002/cncr.10667
UI - 11939762
AU - Sleilati GG; Kovacs KT; Honasoge M
TI - Acromegaly and pheochromocytoma: report of a rare coexistence.
SO - Endocr Pract 2002 Jan-Feb;8(1):54-60
AD - Department of Endocrinology and Metabolism, Henry Ford Health System, Detroit, Michigan 48202, USA.
OBJECTIVE: To describe a patient with the rare coexistence of acromegaly and pheochromocytoma. METHODS: We report a case of a 57-year-old woman, who was initially examined because of polyarthritis, she was also diagnosed with type 2 diabetes mellitus and hypertension at age 56 years. Her history, clinical findings, laboratory results, and management are summarized, and etiologic hypotheses are discussed. RESULTS: The patient had recurrent headaches and reported an increasing size of her shoes and gloves during the previous 4 years. Enlargement of her hands and feet and a bilateral temporal field defect were noted on examination. Laboratory studies revealed high levels of insulin-like growth factor I (IGF-I) and growth hormone (GH). Magnetic resonance imaging (MRI) showed a 3-cm sellar mass with impingement on the optic chiasm. The plasma level of growth hormone-releasing hormone (GHRH) was normal. She underwent transsphenoidal adenomectomy. Histologic examination confirmed a pituitary adenoma, immunoreactive for GH. Postoperatively, her headaches and arthritic pain diminished, and her levels of IGF-I and GH normalized; however, labile hypertension persisted. The urinary metanephrines and plasma catecholamines were increased. A 3-cm left adrenal mass, seen on abdominal MRI, was removed laparoscopically, after which urinary metanephrines normalized and both the diabetes and the hypertension resolved. Histopathologic analysis confirmed the diagnosis of pheochromocytoma. Immunohistochemical staining was negative for GHRH. CONCLUSION: The finding of a pheochromocytoma and acromegaly could be a fortuitous coexistence of two separate endocrine tumors; however, the probability of such an event is extremely low. A cause-and-effect relationship has been suggested because of previous reports of GHRH production by pheochromocytomas. Some investigators have also suggested that this coexistence might be a multiple endocrine neoplasia variant. Our patient had no evidence of GHRH production, nor did we document any familial autosomal dominant transmission pattern.
UI - 11942779
AU - Guven S; Bozkus H; Zirh A; Sarioglu AC; Matfin G
TI - What's in the "sellar"?
SO - Endocr Pract 2002 Mar-Apr;8(2):133-4
AD - Deptartment of Endocrinology, American Hospital, Istanbul, Turkey.
UI - 12134929
AU - Van Effenterre R; Boch AL
TI - Craniopharyngioma in adults and children: a study of 122 surgical cases.
SO - J Neurosurg 2002 Jul;97(1):3-11
AD - Department of Neurosurgery, Groupe Hospitalier Pitie-Salpetriere, Paris, France. firstname.lastname@example.org
OBJECT: This work is devoted to a 25-year retrospective study of 122 cases of craniopharyngiomas in adults and children treated and followed by the same neurosurgeon (R.V.E.). In this homogeneous series, the aim was total microsurgical removal of the tumor, without postoperative radiotherapy. METHODS: The operation was performed via a frontopterional approach in 112 cases and a transsphenoidal approach in 10 cases. The tumor removal was considered total in 59%, subtotal in 29%, and partial in 12%. The surgical mortality rate was 2.5%. Even when tumor removal was not complete, radiotherapy was not systematically administered; it was reserved for cases of recurrence. The authors have studied clinical signs, operative characteristics, and ophthalmological, endocrinological, and functional outcomes, as well as recurrence risk and long-term patient survival. The mean follow-up period was 7 years. The functional results in these patients were excellent in 85%, good in 9%, fair in 5% (usually because of ophthalmological sequelae), and poor in 1%. Tumors recurred in 29 patients, but the salvage treatment, by operation or radiotherapy, was successful in 83%. The actuarial patient survival rate was 92% after 5 years and 85% after 10 years. CONCLUSIONS: These results compared favorably with the data reported in the literature, suggesting that radical surgery of craniopharyngiomas allows good outcome in terms of survival, full recovery, and quality of life for both adults and children.
UI - 12134671
AU - Kurozumi K; Tabuchi A; Ono Y; Tamiya T; Ohmoto T; Furuta T; Hamasaki S
TI - [Pituitary adenoma associated with neurofibromatosis type 1: case report]
SO - No Shinkei Geka 2002 Jul;30(7):741-5
AD - Department of Neurological Surgery, Field of Neuroscience, Branch of Biophysical Science, Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikada-cho, Okayama-city, 700-8558, Japan.
A case of pituitary adenoma associated is neurofibromatosis type 1 is reported. On June 6, 2000, a 49-year-old man was admitted to the Department of Neurological Surgery, Okayama University Hospital, for bitemporal hemianopsia. Twenty-nine years previously, he had been operated on for a left inguinal tumor that proved to be a neurofibroma. Based on the presence of other manifestations, such as cafe-au-lait spots and subcutaneous nodules, he had been diagnosed with neurofibromatosis type 1, the same as his father, sister, and daughter. Computed tomography and magnetic resonance imaging demonstrated an intrasellar mass lesion with a cystic portion in the suprasellar region. Endocrinologically, almost all of his basic hormone levels were normal. A right front-temporal craniotomy was performed for a preoperative diagnosis of craniopharyngioma, and total intracapsular tumor extirpation was achieved. The histological diagnosis was clinically silent corticotroph pituitary adenoma. Neurofibromatosis is sometimes associated with neoplasms of the central nervous system, usually optic gliomas. Associations between pituitary adenomas and NF 1 are very rare and have been reported in only four cases, including the present case.
UI - 11141695
AU - Tan EU; Ho MS; Rajasoorya CR
TI - Metamorphosis of a non-functioning pituitary adenoma to Cushing's disease.
SO - Pituitary 2000 Oct;3(2):117-22
AD - Fatima College of Medicine, Fatima Medical Science Foundation, Inc., Valenzuela City, Philippines, Singapore.
Silent pituitary adenomas occur in 25-40% of all clinically apparent pituitary tumours. However, the subsequent development of florid Cushing's disease in a patient with a previous non-functioning tumour is extremely rare. We describe a 47 year-old woman presenting initially with a large, invasive and recurrent, non-functioning pituitary tumour. Histopathologic study of the initial tissue did not stain for any hormones. Six years after the initial presentation, she manifested florid ACTH dependent Cushing's syndrome. A recurrent invasive pituitary macroadenoma necessitated a third transphenoidal surgery. The resected specimen, in this instance, revealed positive staining for ACTH, FSH, prolactin, and growth hormone on immunocytochemistry. An incomplete response to surgical, radiation and medical therapy necessitated a bilateral adrenalectomy to control the hypercortisolism, which resulted in remarkable clinical improvement. We also review five previous case reports from the published literature of similar transformations from non-secreting pituitary adenomas to Cushing's disease. This subset of patients may represent yet another entity in the widening spectrum of Cushing's syndrome.
UI - 12058124
AU - Bonneville JF; Cattin F; Bonneville F; Schillo F; Jacquet G
TI - [Pituitary gland imaging in Cushing's disease]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):173-85
AD - Service de Neuroradiologie, Centre hospitalier Universitaire, 25030 Besancon Cedex, France.
Specific MR techniques are required for optimal detection of adenocorticotropic hormone secreting adenomas responsible for Cushing's disease. Adequate MR sequences, high resolution coronal T1 and T2 - weighted images, dynamic MR imaging, post-gadolinium delayed images, dose of gadolinium adjusted for each sequence can routinely demonstrate pituitary adenomas less than 3 mm in Cushing's disease.
UI - 12058125
AU - Perrin G; Stevenaert A; Jouanneau E
TI - [Technical aspects and surgical strategy for removal of corticotroph pituitary adenoma]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):186-214
AD - Service de Neurochirurgie A, Hopital Neurologique et Neurochirurgical Pierre-Wertheimer, BP Lyon-Montchat, 69394 Lyon Cedex 3, France. email@example.com
The development of transsphenoidal microsurgery and the refinement of endocrinological and radiological diagnostic procedures have afforded therapeutic options appropriate for each individual case in patients with pituitary-dependent hypercortisolism. Compared with other secreting pituitary tumors, the corticotroph adenoma seems to be the most biologically active tumor. Clinical evidence of hypercortisolism mainly occurs at an early stage of tumor growth when the tumor is very small, below the detection threshold of modern imaging techniques. While the treatment of large tumors remains difficult due to the non-discrete boundary lines of the tumor and extension or invasion, surgical removal of very tiny tumors requires reliable preoperative or peroperative identification in order to achieve total tumor resection for clinical remission and pituitary preservation to prevent hypopituitarism. We reviewed all the current surgical techniques or clever surgical procedures used to achieve both goals with the lowest complication rate. We report here the state-of-the-art of surgical management of corticotroph pituitary adenoma focusing on preoperative radiological and biological data required for performing guided intrasellar surgical exploration and reliable tumor identification. Different technical aspects of the nasosphenoidal approaches are reported as well as the modified transdiaphragmatic or transtubercular transcisternal approaches to tumors in a suprasellar localization or lying along the pituitary stalk. The advantages of minimally invasive surgical techniques such as intrasellar endoscopic surgery are discussed. Adapted surgical techniques for second transnasal surgery indicated for recurrent tumors are described. Guidelines are given for peroperative tumor identification with macroscopic assessment or histological control with frozen section biopsies. Different techniques for tumor removal are discussed from selective microadenomectomy to enlarged pituitary resection and total hypophysectomy. Methods for preoperative guidance of total tumor removal are proposed including histological or biological assessment of normal adjacent pituitary tissue. the strategy of surgical intrasellar exploration and tumor resection is outlined using a set of algorithms. The first is devoted to positive preoperative documentation of the tumor. The second is proposed for the surgical scenario where there is no preoperative MRI evidence of the tumor. Special strategies are discussed for ectopic adenoma or multiple tumors. Revision surgical management after surgical failure or tumor recurrence is described. Special guidelines for surgical treatment of large clinically silent corticotroph macroadenomas are given with emphasis on the high risk of recurrence in comparison with other silent pituitary tumors such as gonadotroph or immunonegative adenomas.
UI - 12058126
AU - Jouanneau E; Perrin G; Trouillas J
TI - [Corticotroph microadenomas of the pituitary stalk. Diagnostic and therapeutic difficulties]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):215-22
AD - Service de Neurochirurgie A, Hopital Neurologique et Neurochirurgical Pierre-Wertheimer, 59, boulevard Pinel, 69003 Lyon. firstname.lastname@example.org
Thirteen percent of the pituitary adenomas in our series have been corticotroph adenomas. Most have been microadenomas located in the adenohypophysis, more often laterally than posteromedially. In a few rare cases, the corticotroph cells of the pars tuberalis gave rise to an adenoma. Only 16 cases of pituitary stalk adenomas have been published to date. We report here three new cases among a series of 150 patients who underwent surgery for corticotroph adenomas (2% incidence as in the literature). There was no difference in the clinical or biological presentation of intrasellar and pituitary stalk adenomas. This location must be systematically searched on the MRI, but a negative exploration cannot excluded the diagnosis of pituitary stalk adenoma like in intrasellar ones. Two anatomical entities seems to be separated. Some stalk adenomas developed preferentially in the supradiaphragmatic space (one in our series) and are accessible for selective resection. Others develop within the pituitary stalk itself and sometimes in the pars distalis (two patients in our series) which requires section of the pituitary stalk with hypophysectomy if the lower limit is not clearly seen during surgery. The subfrontal or pterional access is the approach of choice for suprasellar tumors. These approaches remain controversial for pituitary stalk adenomas because of the poor visibility of the pituitary stalk and the floor of the third ventricle. The transnasosphenoidal approach with extension to the sphenoidal planum and opening of the optochiasmatic cisterna appears to be the best alternative for surgical removal of these adenomas.
UI - 12058127
AU - Passagia JG; Gay E; Chabre O; Martinie M; Labat-Moleur F; Bachelot I
TI - [Role of perioperative biological tests during the performance and follow-up of corticotroph adenoma exeresis]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):223-5
AD - Service de Neurochirurgie, CHU de Grenoble, BP 217, 38043 Grenoble Cedex, France. JGPassagia@chu-grenoble.fr
ACTH assay in cavernous sinus samples during resection of pituitary adrenocorticotroph adenomas is a simple and safe technique providing an intraoperative assessment of adrenocorticotroph hormone gradients. Bilateral puncture of the cavernous sinus can be achieved vial the standard transsphenoidal approach to the sella turcica. ACTH is determined with IRMA at 37;C with an incubation time of less than one hour. Among 71 cases in our experience, the ACTH gradient accurately predicted the position of the adenoma in 93% of the cases. This rate is higher than the 61% accuracy reported for inferior petrosal sinus sampling. The technique reported is more precise than MRI which correctly identifies adenomas in only 50% of the cases. The remaining cases are either false positives or false negatives. We report an 82% cure rate either via direct resection of the microadenoma or via partial hypophysectomy guided by the ACTH gradient. In our series, 20 cases of Cushing's disease had a normal MRI and no surgically identifiable adenoma. In 10 of these cases however, cure was achieved by performing ACTH gradient guided partial hypophysectomy. This method produces no morbidity and is most helpful for the neurosurgeon allowing confirmation of the position of an MRI-visible adenoma or an adenoma identified intraoperatively. It does not however replace neurosurgical experience which remains the most important predictive factor for outcome in surgical treatment of Cushing's disease.
UI - 12058128
AU - Martin D; Valdes-Socin H; Beckers A; Stevenaert A
TI - [Cushing's disease and corticotroph adenoma: anterior pituitary function before and after trans-sphenoidal microsurgery]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):226-33
AD - Service de Neurochirurgie, Centre Hospitalier Universitaire, Domaine Universitaire, Belgique, France. email@example.com
BACKGROUND AND PURPOSE: Cushing's disease is characterized by an overproduction of ACTH inducing an hypersecretion of corticosteroids. We assessed the non-corticotrophic pituitary functions before and after microsurgical resection of the corticotrophic adenoma.METHODS: We reviewed retrospectively a series of 98 patients (19 males, 79 females) undergoing transsphenoidal surgery between 1974 and 2001. Prolactin levels, somatrotrophic, gonadotrophic and thyroidotrophic functions were assessed and compared before and after resection of the adenoma.RESULTS: Before surgery, we observed an elevation of prolactin and a reduction of the GH, TSH, LH and FSH levels. These modifications were not linked to the volume of the adenoma but depended on the systemic effect of the hypercorticism or on local paracrine mechanisms. Selective adenomectomy usually restored altered corticotrophic and also, unlike (partial or total) hypophysectomy, the non-corticotrophic functions.CONCLUSIONS: Non-corticotrophic functions are often altered in Cushing's disease but can be restored after selective adenomectomy.
UI - 12058129
AU - Stevenaert A; Perrin G; Martin D; Beckers A
TI - [Cushing's disease and corticotrophic adenoma: results of pituitary microsurgery]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):234-65
AD - Service de Neurochirurgie, Centre Hospitalier Universitaire, Liege, France. A.Stevenaert@ulg.ac.be underwent transsphenoidal surgery: 167 patients had adenomectomy, 14 had ante hypophysectomy, 5 had subtotal hypophysectomy, 4 had hemihypophysectomy 4 had central hypophysectomy. Complications occurred in 18 patients (9.3%), including 4 deaths (three were apparently not related to surgery). Remission of disease was achieved in 162 of 190 (85.3%) patients analyzed. Surgical failures were associated with lack of pituitary adenoma, size of the tumor and invasiveness. Among patients with confirmed adenomas, the rate of remission was significantly higher (p<0.01) in patients with microadenomas (92.6%) than in patients with macroadenomas (66.7%). Reoperation in 6 failures was followed by remission in 4 cases. The overall remission rate was 87.4%. In the 162 patients with immediate success, duration of follow-up was 10.0 +/- 5.9 years (m +/- DS; median=10.0). Recurrence of the disease occurred in 24 (14.8%) of 162 patients at a mean 4.8 years (range: 0.8-12.0 years). Our longest sustained remission is 25.6 years. Actuarial analysis indicates that the probability of a patient remaining well 12 years after surgery is 80.0%. It is 86.2% in microadenomas versus 52.5% in macroadenomas and 94.5% in the patients with postoperative hypocortisolism versus 59.2% in the others.
UI - 12058130
AU - Jan M; Francois P; Trouillas J; Hage P
TI - [Indications for total hypophysectomy in Cushing's disease]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):266-70
AD - Service de Neurochirurgie, CHU Bretonneau, 37044 Tours Cedex 1, France. firstname.lastname@example.org
OBJECTIVE: Transsphenoidal selective adenomectomy is the treatment of choice for Cushing's disease. In some patients, magnetic resonance imaging (MRI) fails to detect small pituitary ACTH-secreting adenomas. Total hypophysectomy can be performed when MRI appears normal. The aim of this paper is to study results and complications after total 1999, 49 patients underwent transsphenoidal surgery for Cushing's disease at our institution. The criteria for inclusion in this study were clinical and biochemical studies strongly suggestive of Cushing's disease with normal MRI. Total hypophysectomy was performed in 7 patients who fulfilled these criteria. Their results were analyzed retrospectively. RESULTS: The average age of the patients was 43 years; there were 7 women. Five adrenocorticotrophic hormone-secreting adenomas were proven histologically. Complications occurred in 6 patients (cerebrospinal fluid fistulas in 6 patients, loss of vision in 1 patient, meningitis in 5 patients, anterior pituitary insufficiency in 5 patients, diabetes insipidus in 5 patients). Six patients had sustained remission 36 months after surgery. CONCLUSION: Total hypophysectomy can be perform ed for Cushing's disease with normal MRI. Complications occurs frequently, especially cerebrospinal fluid fistulas. Inferior petrosal sinus sampling can be helpful in localizing the adenoma allowing hemihypophysectomy and thus reduced morbidity.
UI - 12058131
AU - Linglart A; Visot A
TI - [Cushing's disease in children and adolescents]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):271-80
AD - Service d'Endocrinologie Pediatrique, Hopital Saint-Vincent-de-Paul, Paris, France.
Fifty-seven unselected children and adolescents with Cushing's disease underwent transsphenoidal exploration and microadenomectomy before their 16th birthday. Hypercortisolism was corrected in 42 of the 57 patients (74%). The recurrence rate was 17% and the long-term remission rate was 57%. Diagnostic peculiarities specific surgical problems and outcome were reviewed. Direct transnasal submucosal surgery for Cushing's disease is successful, and pituitary function can be preserved in most of these young patients.
UI - 12058132
AU - Estour B; Duthel R; Mounier Ch
TI - [Clinical recurrence of Cushing syndrome without evidence of tumor recurrence: radical hypophysectomy?]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):281-4
AD - Service d'Endocrinologie, Hopital Bllevue, CHU de Saint-Etienne, 42055 Saint-Etienne Cedex, France. email@example.com
Outcome following radiation therapy for hormonally-active Cushing adenoma is reported. Conventional radiation using 20 to 30 grays improved mass and hormone level in 30 to 80% of the patient. Gamma-knife radiosurgery was at least as effective. These results are discussed in this review.
UI - 12058133
AU - Colin P; Delemer B; Nakib I; Caron J; Bazin A; Bernard MH; Peruzzi P;
TI - Scavarda D; Scherpereel B; Longuebray A; Redon C; Petel F; Rousseaux P [Unsuccessful surgery of Cushing's disease. Role and efficacy of fractionated stereotactic radiotherapy]
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):285-93
AD - Service de Neurochirurgie, CHU, Reims, France. firstname.lastname@example.org
BACKGROUND AND PURPOSE: To optimize and reduce the toxicity of pituitary adenoma irradiation, we evaluated the feasibility and effectiveness of fractionated stereotactic radiotherapy (FSR) in 51 patients with functional adenoma; 12 of them had Cushing's disease.PATIENTS AND METHODS: Eleven females and one male, median age: 49 years, 8 of them relapsed and 4 had persistent hormonal secretion after surgery. The delivered dose was 50 grays (Gy) (5 x 1.8 Gy/week) using 5 convergent micro-beams scanning in arc therapy mode. The median planned target volume (PTV) receiving 90 to 100% of the total dose was 2,1 cm(3). We used relocatable non-invasive stereotactic device Brain Lab, and adapted Linac for stereotactic radiotherapy.RESULTS: Nine out of twelve patients (75%) presented with complete remission after a median time of 29 months, 3/12 patients with partial remission. Actuarial probability to have hormonal persistent secretion was 88% at on year, 44% from 3 to 5 years, 22% at 7 years, and 11% to 0% after 7 years. The 12-corticotroph adenomas were significantly (p=0.007) more radiosensitive than 39 non-corticotroph adenomas. Toxicity was significantly lower for corticotrope adenoma, with no radio-induced pituitary deficiency and no neurological or optic injury.CONCLUSIONS: Combined surgery and FSR appears to be safe and effective for treatment of pituitary adenomas. However, long-term follow-up is required to determine the impact in terms of tumor response and hormone deficiency. The focused treated volume obtained by FSR seems more suitable for these benign tumours than standard radiotherapy. In addition to this conformal approach, the use of standard fractionation reduced the risk of severe damage to the optic chiasma, which is sometimes observed after radiosurgery and is inherent in single fraction radiotherapy.
UI - 12058134
AU - Laws ER; Reitmeyer M; Thapar K; Vance ML
TI - Cushing's disease resulting from pituitary corticotrophic microadenoma. Treatment results from transsphenoidal microsurgery and gamma knife radiosurgery.
SO - Neurochirurgie 2002 May;48(2-3 Pt 2):294-9
AD - Department of Neurosurgery, University of Virginia, Charlottesville, VA 22908-0212, USA. EL5G@virginia.edu
Cushing's disease and its associated clinical syndrome reflect the effects of excess cortisol on the individual. The cause of Cushing's disease is ordinarily an ACTH-secreting benign pituitary adenoma. The diagnosis of Cushing's disease is established by sophisticated endocrine testing and comprehensive imaging studies. Because of the devastating effects of excess cortisol, therapy that provides prompt and effective normalization of serum cortisol is essential. Currently this goal is best achieved by transsphenoidal microsurgery. This paper reviews the clinical presentation, laboratory analysis, surgical management and outcome in patients with Cushing's disease.
UI - 12116534
AU - Nomura H; Kurimoto M; Nagai S; Hayashi N; Hirashima Y; Tsukamoto E; Endo
TI - S Multiple intracranial seeding of craniopharyngioma after repeated surgery--case report.
SO - Neurol Med Chir (Tokyo) 2002 Jun;42(6):268-71
AD - Department of Neurosurgery, Toyama Medical and Pharmaceutical University, Toyama, Japan. email@example.com
A 17-year-old woman presented with a rare case of intracranial seeding of craniopharyngioma after repeated surgery. She initially presented with secondary amenorrhea and visual impairment. Magnetic resonance imaging revealed a suprasellar mass. Subtotal removal of the tumor was performed. The diagnosis was adamantinomatous craniopharyngioma. Seven months later, the patient underwent a second operation for recurrence of the craniopharyngioma. Subsequently, ventriculoperitoneal (VP) shunting and gamma knife surgery were performed. Twenty-seven months after the first operation, multiple cystic lesions were found in the right frontal and temporal lobes. Positive tumor cytology was observed in the cerebrospinal fluid obtained from the VP shunt chamber. These tumors were subtotally resected. However, the patient died from consecutive tumor recurrence 4 years after the initial diagnosis.
UI - 12138986
AU - Gourgiotis L; Skarulis MC; Brucker-Davis F; Oldfield EH; Sarlis NJ
TI - Effectiveness of long-acting octreotide in suppressing hormonogenesis and tumor growth in thyrotropin-secreting pituitary adenomas: report of two cases.
SO - Pituitary 2001 Aug;4(3):135-43
AD - Clinical Endocrinology Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
BACKGROUND: The subcutaneous (s.c.) administration of somatostatin analogs, such as octreotide acetate (SMS) and lanreotide, in patients with thyrotropin (TSH)-secreting pituitary adenomas (TSPA's)--thyrotropinomas with residual tumor after initial surgical therapy is effective in controlling hyperthyroidism, as well as curtailing tumor growth in the majority of patients. Long-acting preparations of the above agents, i.e. SMS-LAR and lanreotide-SR, have been synthesized and can be administered as depot injections intramuscularly (i.m.) at intervals of several weeks. Recent studies have reported on preliminary data regarding the use of such preparations in patients with TSPA's. MATERIALS AND METHODS: We present two cases of TSPA's with residual tumor following transsphenoidal adenomectomy. Neither of the two patients underwent external beam pituitary irradiation. The presence and extent of tumoral TSH hypersecretion was assessed by standard biochemical and dynamic endocrine testing, while tumor size was evaluated by conventional radiographic techniques. RESULTS: In both patients, TSH secretion was effectively suppressed by SMS-LAR. Moreover, administration of this compound halted further tumor growth, as well as resulted in improved patient comfort, for 12 and 10 months respectively. CONCLUSION: Our date corroborate earlier reports on the usefulness of SMS-LAR in the medical management of patients with TSPA's who have residual disease after initial pituitary surgery and/or irradiation.
UI - 12138990
AU - Lohmann T; Trantakis C; Biesold M; Prothmann S; Guenzel S; Schober R;
TI - Paschke R Minor tumour shrinkage in nonfunctioning pituitary adenomas by long-term treatment with the dopamine agonist cabergoline.
SO - Pituitary 2001 Aug;4(3):173-8
AD - Department of Internal Medicine III, University of Leipzig, Germany. Tobias.Lohmann@med1.imed.uni-erlangen.de
OBJECTIVE: The purpose of this study was to define safety and efficacy of medical therapy in the treatment of nonfunctioning pituitary tumours. DESIGN: We studied thirteen patients with a clinically nonfunctioning pituitary macroadenoma for response to cabergoline treatment for 1 year. Twelve/13 patients were already operated and had residual or recurrent tumours. METHODS: We determined the outcome of treatment by visual perimetry, computed tumour size measurement in MRI and hormonal response (changes in pituitary function, reduction of alpha-subunit). RESULTS: Seven/13 patients on cabergoline had a tumour shrinkage above 10% of the initial tumour volume. In 4 patients, this tumour shrinkage was correlated to an increasing distance of the tumour to the optic chiasm. Only 2/9 patients with visual field defects before therapy showed improvements in visual acuity under cabergoline. No significant side effects of the therapeutical regimens were observed. Neither LH and/or FSH expression in the tumour cells nor the reduction of the alpha-subunit serum levels by medical therapy was correlated to tumour shrinkage. CONCLUSION: Given that these patients had advanced disease which makes it difficult to find significant therapeutic effects, medical therapy with potent dopamine agonists such as cabergoline may evolve as a novel therapeutic option in a subgroup of patients with clinically nonfunctioning tumours declining operation and radiotherapy.
UI - 12138991
AU - Liu C; Tyrrell JB
TI - Successful treatment of a large macroprolactinoma with cabergoline during pregnancy.
SO - Pituitary 2001 Aug;4(3):179-85
AD - Department of Medicine, University of California, San Francisco, 94143-0326, USA. firstname.lastname@example.org
We report a pregnant woman with a large macroprolactinoma successfully treated with cabergoline after a suboptimal response to bromocriptine. A 7 week pregnant woman with a history of a prolactinoma presented to the endocrine clinic with the complaints of headaches and nausea. She had a prolactin level of 65 microg/L 1 1/2 weeks following her last menstrual period. Bromocriptine was discontinued at 6 weeks gestation when pregnancy was confirmed. A PRL concentration was 1899 microg/L (non-pregnant normal range 1.39-24.20 microg/L, the mean peak levels during pregnancy reported from the literature are 200-210 microg/L) at 7 weeks gestation, and a repeat was 2197 microg/L. An MRI showed a 3 x 2.2 x 2.5 cm seller mass abutting the optic chiasm and displacing the optic nerves superiorly; the visual field testing was normal. Bromocriptine was reinitiated and the patient responded initially with decreasing headaches and declining PRL concentrations to 1488 microg/L at 15 weeks gestation. However, PRL increased to 1836 microg/L at 16 weeks and remained elevated despite bromocriptine 2.5 mg three times a day; in addition, she complained of severe nausea, vomiting, and persistent headaches. Cabergoline was added at 18 weeks gestation. PRL decreased dramatically from 1710 to 859 microg/L in 1 week, and to 488 microg/L within 4 weeks. A repeat MRI showed more than 30% reduction in tumor size. Bromocriptine was discontinued at 24 weeks gestation; she was maintained on cabergoline 0.5 mg twice a week without complaints. PRL levels ranged from 190 to 278 microg/L during the last 10 weeks of pregnancy. She had a C-section electively at 37 weeks gestation and delivered a healthy baby. Management options in this patient and during pregnancy are discussed.
UI - 12138993
AU - Rubin MR; Bruce JN; Khandji AG; Freda PU
TI - Sarcoidosis within a pituitary adenoma.
SO - Pituitary 2001 Aug;4(3):195-202
AD - Department of Medicine, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
A 54 year old man presented with frontal headaches for one year. A CT scan of the head revealed a pituitary mass. He denied a change in vision or galactorrhea, but did have decreased frequency of erections and a recent episode of renal stones. On physical exam, the cranial nerves were normal. Visual field exam revealed mild bilateral temporal defects. The genitalia were normal and the testes were soft. Laboratory evaluation revealed: Na, 134 mM/l; K, 6.7 mM/l; Cl, 104 mM/l; HCO3, 22 mM/l; BUN, 47 mg/dl; Cr, 8.3 mg/dl; Ca, 12.5 mg/dl; Phos, 5.5 mg/dl; prolactin, 32.0 ng/ml; T4, 4.46 microg/dl; TSH, 2.07 microU/ml; LH, 18.1 mIU/ml; FSH 3.2 mIU/ml; alpha subunit 1.6 ng/ml; testosterone 255 ng/dl; cortisol, 20.3 microg/dl; cortisol after 250 microg cortrosyn, 38.5 microg/dl (time 60 minutes); growth hormone, 1.4 ng/ml; IGF-1, 47 ng/ml; PTH, <1 pg/ml; 25-hydroxyvitamin D, 14 ng/ml; 1,25-dihydroxyvitamin D, 69 pg/ml. These results were felt to be consistent with a non-PTH-mediated hypercalcemia, such as humoral hypercalcemia of malignancy, or a vitamin D-mediated hypercalcemia, such as lymphoma, sarcoidosis or tuberculosis. Head MRI demonstrated a 3.5 x 3.5 x 2.5 cm heterogeneous mass enlarging the sella, deforming the clivus and compressing the cavernous sinus, basilar artery and left side of the optic chiasm. There was a small focus of high signal in the superior part of the mass on the T1-weighted image from either a proteinaceous cyst with early calcium deposition or sub-acute blood. These radiographic findings were felt to be consistent with a pituitary adenoma. The patient was treated with intravenous hydration and thyroxine 50 microg daily and underwent a transsphenoidal resection of the pituitary lesion. Pathologic examination revealed a pituitary adenoma with multiple granulomas and crystalline material; this was consistent with sarcoid within the adenoma. Post-operatively, the serum LH fell to 5.5 mIU/ml. A subsequent transbronchial biopsy revealed multiple non-caseating granulomas. A serum ACE level was elevated at 132.6 U/l. He received oral prednisone 60 mg daily with resolution of the hypercalcemia. Neurosarcoidosis occurs in 5 to 15% of patients with sarcoidosis and can involve the hypothalamus and pituitary gland. This is the first reported case of sarcoidosis occurring within a pituitary adenoma.
UI - 11798773
AU - Zhang Y; Wang Z; Ma Z
TI - [Pathological changes and protection of hypothalamus in pediatric craniopharyngioma]
SO - Zhonghua Yi Xue Za Zhi 2000 Apr;80(4):280-2
AD - Beijing Tiantan Hospital, Beijing 100050, China.
OBJECTIVE: To understand the pathological changes of pediatric craniopharyngiomas so as to analyze its diagnosis, operative strategy and to prevent the damage of hypothalamus. METHODS: 189 cases of pediatric craniopharyngiomas treated from 1990 to 1998 were reviewed and analyzed via their CT, MRI, and surgery. RESULTS: 187 cases (98.9%) were cystic tumors, including 176 with calcification (93.1%); two cases were solid tumors (1.1%), and calcification occurred in only one. A gliosis layer between the wall of tumor and the hypothalamus was seen. CONCLUSION: Cystic change and calcification are the pathological features of pediatric craniopharyngiomas. There are some special relations between the tumors and stalk. These are the bases for total removal of pediatric craniopharyngiomas.