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PubMed1978

[Corticotrophic adenomas with melanoderma (Nelson's syndrome). Two cases clinical and laboratory studies before and after spontaneous intercurrent necrosis (author's transl)].

Two cases of Nelson's syndrome (macroscopic cortico-melanotropic pituitary adenoma developing after bilateral total adrenalectomy for Cushing's disease) are reported. The unusual feature was spontaneous necrosis of the adenoma, as indicated by the rapid disappearance of melanoderma, the normalisation or decrease in plasma ACTH level, retraction in the diaphragm of the sella at air encephalography and autopsy findings in one case.

Adult↗
PubMed1981

[Pituitary adenomata. Status of diagnosis and therapy (author's transl)].

The advances in pituitary surgery are based on the diagnosis of neuroendocrinological function and the microsurgical operative technique with the help of which pituitary adenomata are approached by the transsphenoidal route in approximately 90% of cases and transcranially in about 10%. On the one hand, hormone-active microadenomata can presently be detected in good time endocrinologically and 80-90% can be removed selectively while retaining the pituitary. On the other hand, it can be shown that the greater the tumor the more frequently hormone excesses persist after the operation. Tumors can be directly visualized in the cranial computer tomogram if they ar not smaller than 5-7 mm. Also, larger extrasellar remains of tumors left after operation can be demonstrated by computer tomography. Larger suprasellar adenomata produce a chiasma syndrome which improves in 80% of patients after transphenoidal operation. The slight operative risk is shown in a total of 737 operations for pituitary adenomata in the last 11 years.

Adenoma↗
PubMed1978

Plasma steroid dynamics in Cushing's syndrome.

The dynamics of five plasma steroids were studied by CPB and RIA in twenty patients with Cushing's syndrome of differing etiologies: pituitary induced adrenal hyperplasia (10), recurrence after adrenal surgery or pituitary irradiation (4), adrenal carcinoma (4) and adrenal adenoma (2). Basal mean values of plasma steroids, showed increase of cortisol in hyperplasia and cancer groups, increase of 17-hydroxyprogesterone (17-OH-P) in recurrence and cancer groups and decrease of testosterone in male patients. In hyperplasia, the changes in 11-desoxycortisol and 17-OH-P (respectively by CPB assay and RIA after LH-20-Sephadex separation) were the reverse of those for cortisol 2 h and 24 h after metyrapone administration. Adrenalectomy determined in all but one patients, significant decrease of cortisol and in male patients, parallel gradual increase of testosterone.

Adrenal Gland Neoplasms↗
PubMedDate not supplied

[Hypophyseal tumors].

Explore the source record for details and available documents.

Adenoma, Chromophobe↗
PubMed1981

[Incidence and clinical manifestation of ACTH-producing pituitary adenoma after bilateral adrenalectomy for Cushing's syndrome (Nelson syndrome)].

29 out of 39 patients bilaterally adrenalectomized for pituitary-dependent Cushing's syndrome between 1958 and 1979 have been followed up at regular intervals. ACTH-producing pituitary adenomas were detected and removed in 9 of them up to 1981. In 4 additional patients an enlarged sella turcica was already found at the time they had Cushing's syndrome, and therefore they were first treated by pituitary surgery or radiotherapy. In the adrenalectomized patients hyperpigmentation was the major clinical symptoms of the adenoma. Only in three instances did extrasellar growth of the adenoma cause ophthalmoplegia or narrowing of the visual fields. The existence of intrasellar ACTH-producing microadenomas in 5 additional patients seems highly probable in view of hyperpigmentation, excessively elevated plasma ACTH concentrations and radiological evidence of sella deformations. These observations indicate that in patients adrenalectomized for pituitary-dependent Cushing's syndrome ACTH-producing adenomas are more common than previously assumed. Moreover, according to recent reports, ACTH-producing microadenomas are frequently found in pituitary-dependent Cushing's syndrome and are assumed by many clinical endocrinologists to be the primary cause of this disease. Therefore, bilateral adrenalectomy is no longer the undisputed therapy of first choice and it is necessary in each individual case to consider carefully whether an attempt to remove selectively an ACTH-producing pituitary adenoma by microsurgery might not be preferable.

ACTH Syndrome, Ectopic↗
PubMed1981

[Hyperprolactinemia in the male: clinical aspects and therapy].

Fifty-seven male patients with hyperprolactinemia have been investigated. Macroadenomas were found in 49 patients of whom 24 had received pituitary surgery before elevated PRL levels were discovered. In 8 patients radiology of the pituitary fossa revealed either evidence of microadenoma or a completely normal sella turcica. Prolactin levels ranged from 582 to 498 000 microU/ml. All patients with macroprolactinomas, including those who had undergone previous surgery, had PRL levels above 5000 microU/ml. Disturbances of libido and sexual potency were prevalent in the majority of patients, less frequently accompanied by gynacecomastia and galactorrhea. However, visual disturbances were the reason for the first visit to the doctor in the majority of patients. Pituitary surgery led to improvement of visual field defects, though prolactin levels were never normalized. Postoperative radiotherapy had no significant influence on PRL levels. In contrast, bromocriptine therapy led, in the majority of patients, not only to improvement of hypogonadal symptoms but also to normalization of PRL levels.

Adolescent↗
PubMed1981

[Follow-up of patients with pituitary tumors before and after operation, medical treatment and radiotherapy (author's transl)].

From 1976 till 1981 56 patients with pituitary tumors were observed. From a total of 51 adenomas 17 prolactinomas (33.3%), 15 acromegalies (29.4%), 4 Cushing-syndromes (7.8%), 1 TSH-producing adenoma (1.9%) and 14 hormone-inactive adenomas (27.4%) were found. In addition there were 4 craniopharyngeomas and 1 intrasellar meningeoma. For the individual types of adenomas prae-and postoperative hormonal data after transcranial resp. transsphenoidal adenomectomy are listed. Prolactinomas responded well to bromocriptine therapy in some cases, whereas acromegalies showed no remission after bromocriptine. After radiotherapy of 4 recidive-adenomas remission in one case was seen. Problems and objects of long-term-care of patients with pituitary tumors are discussed.

ACTH Syndrome, Ectopic↗
PubMed1980

Parasellar tumors in children. II. Surgical management, radiation therapy, and follow-up.

Despite the predominance of histologically benign tumors in the parasellar region of children, their proximity to the hypothalamus, optic nerves and tracts, and the internal carotid artery and its branches often renders surgical removal exceptionally difficult. The possible presence of a variety of tumor types makes biopsy verification of tumor diagnosis essential. The surgical management, radiotherapeutic treatment, and clinical follow-up of a group of 74 children whose preoperative findings were reviewed in the preceding paper are presented to illustrate the management of four major tumor types encountered: craniopharyngioma, pituitary adenoma, germinoma, and diencephalic glioma. Both subfrontal and transsphenoidal surgical approaches were used. Although total resection of benign tumors is desirable, prudent use of a variety of techniques can achieve effective neurosurgical management and optimal long-term results.

Adenoma↗
PubMed1995

[Endocrinological aspects of hypophyseal tumors].

Local expansion of pituitary tumors causes nonspecific symptoms, including hormone insufficiency, that may exist for years before diagnosis. Although they are more specific, symptoms of pituitary hormone oversecretion may also remain unrecognized and give rise to difficult diagnostic problems. Gonadotropic tumors do not elicit specific endocrine symptoms, whether they secrete complete gonadotropins or their biologically inactive free subunits. Hyperprolactinemia is not due to tumor secretion in the majority of cases, including some with a tumor in the pituitary (compressive hyperprolactinemia). When acromegaly is suspected, the unequivocal proof of excess growth hormone secretion is not easy to obtain, due to intermittent growth hormone secretion, both in normals and in acromegalics. The large variety of tests available for the etiological diagnosis of hypercorticism indicates the diagnostic difficulties that can be encountered. Corticotrope pituitary adenomas may be minute, and ectopic corticotropin secretion may remain occult. But on the other hand, the quality of the diagnostic tools available improves constantly. This includes new hormone measurements, such as insulin-like growth factor 1 and its binding proteins, dynamic tests, selective pituitary blood drawing by inferior petrosal sinus catheterism, pituitary nuclear magnetic resonance imaging and somatostatin-analogue scintiscan. In addition to the improving techniques of transphenoidal neurosurgery and pituitary radiotherapy, medical management of pituitary tumors remains a distinct possibility. Tumor hormone secretion can be controlled in some cases by drugs such as dopaminergic agents, somatostatin analogues and gonadoliberin analogues, which may also exert an antitumoral mass effect. Within the next few years we will be confronted with an increasing number of incidentally discovered pituitary tumors, due to the frequent use of high-resolution intracranial imaging.

Acromegaly↗
PubMedDate not supplied

Necrotizing brainstem leukoencephalopathy six weeks following radiotherapy.

A 71-year-old male with a null cell pituitary macroadenoma was given peripituitary region radiotherapy (4,346 Gy units) over a 1-month-period and 6 weeks later developed the rapid onset of blurred vision, diplopia, ataxia, incoordination, cranial nerve palsy, somnolence, and respiratory distress. His fixed neurologic deficits required ventilatory support, feeding tube placement, and chronic hospitalization until his death 1 year later. At autopsy, necrotic foci with dystrophic axonal calcification were found throughout the brainstem, associated with mild focal vascular hyalinization but without fibrinoid necrosis, telangiectasias or large vessel abnormalities. This unusual case of fatal "early delayed" radiation injury is compared to previous literature cases.

Adenoma↗
PubMed1994

[Pituitary tumors: clinical presentation and evaluation of transphenoidal surgical treatment].

The clinical manifestations and the surgical treatment results of 280 patients (179 female), undergoing a total of 319 operations at the Asenjo Institute of Neurosurgery were retrospectively analyzed. The surgical approach for the first operation was transphenoidal in 89.3% of patients and transcraneal in the rest. Tumors were non-secretory in 169 (60.4%) patients, prolactinomas in 75 (27.8%) and produced acromegalia in 29 (10.4%) and Cushing syndrome in 7 (2.5%) patients. There was extraselar extension in 42% of women and 71% of men. The surgical treatment of prolactinomas corrected endocrine alterations in 25 of 29 and visual alterations in 18 of 27 patients assessed. Surgical treatment of acromegalia improved endocrine alterations in 11 of 13 and visual alterations in 4 of 10 patients assessed. Treatment of non secretory tumors corrected visual alteratons in 38 of 64 patients assessed. The principal complication of transphenoidal surgery was transient diabetes insipidus in 6.8% of patients. Overall mortality was 2.3% for transphenoidal surgery and 5.6% for transcraneal surgery. It is concluded that transphenoidal surgery is effective for the management extraselar complications of pituitary tumors and endocrine management of selected cases.

Adolescent↗