Acromegaly and diabetes mellitus associated with hyperthyroidism.

A 66-year-old woman with acromegaly and diabetes mellitus as well as primary hyperthyroidism is described. Serum GH Levels were inappprpriately high.MRI revealed an enlarged sella turcica with intrasellar mass. Her HbAlc was 12.2% and fasting blood glucose 8.89 mmol/l. Thyroid hormone levels in serum and thyroidal radioiodine uptake values were elevated, while TSH measurements in serum were low. Anti TPO antibodies were negative, TSH receptor antibodies were normal. Thyrotoxicosis as the first presenting illness in acromegaly was particulary uncommon. An ultrasound thyroid scan showed a multinodular goiter. Histology of the pituitary lesion showed a typical eosinophilic adenoma which only secreted GH when tested with specific immunostain. Post-operatively, the patient's clinical conditions improved, however, secondary hypoadrenalism appeared.

Apoplexy accompanying pituitary adenoma as a complication of preoperative anterior pituitary function tests.

Pituitary apoplexy occurs as a very rare complication of the pituitary function test. We have experienced two cases of pituitary apoplexy following anterior pituitary function tests for preoperative assessment: a triple bolus test and a TRH test. To elucidate such a rare complication, we outline our two cases and review 28 cases from the literature. The clinical characteristics, etiology, pathophysiology, and diagnostic and therapeutic implications are also discussed. The combined data suggest that pituitary function tests have the potential to precipitate pituitary apoplexy, and its manifestations range from a clinically benign event to a catastrophic presentation with permanent neurological deficits or even death, although most patients may fortunately have a good outcome. We suggest that the pituitary function test should not be done as a routine test, and when such a test is planned, the patient should be observed with caution for any symptomatic changes for at least 2 hours following the test for appropriate treatment. Further, MRI, especially enhanced studies, may provide an earlier diagnosis of the pituitary apoplexy since CT scan images often fail to demonstrate either density changes or obvious enlargement of the pituitary adenoma at the acute stage.

Ectopic growth hormone-releasing adenoma in the cavernous sinus--case report.

A 55-year-old woman presented a rare ectopic pituitary adenoma in the right cavernous sinus manifesting as acromegaly. The tumor was removed via transsphenoidal approach. Intraoperative observation showed the adenoma was located entirely within the right cavernous sinus, and separated from the normal pituitary gland by the medial wall of the cavernous sinus. There was no communication between the tumor and the pituitary. Histological examination showed a growth hormone-releasing adenoma. Including our case, only eight of 86 reported ectopic adenomas have occurred in the cavernous sinus. Such ectopic presentation may be responsible for failed transsphenoidal surgery for endocrinologically active tumors.

Patología de los adenomas hipofisarios / Pathology of pituitary adenomas

Los adenomas hipofisarios son neoplasias benignas originadas en células de la adenohipófisis. Representan el tumor más habitual en la silla turca y constituyen un 10-15 por ciento de las neoplasias intracraneales. Inicialmente fueron clasificados, según sus características tintoriales, en adenomas acidófilos, basófilos y cromófobos. La aplicación de técnicas morfológicas como la microscopía electrónica y la inmunohistoquímica, y la integración de los hallazgos morfológicos con los datos clínicos, de laboratorio y de las técnicas de imagen han permitido el desarrollo de la nueva clasificación de los adenomas hipofisarios. Las técnicas genéticas y moleculares están proporcionando información que ayuda a entender la patogénesis de algunas variedades de adenoma hipofisario. Por otra parte, la aplicación de determinados marcadores puede aportar información pronóstica y ayudar a predecir la respuesta a modalidades terapéuticas específicas (AU)

Treatment of an acromegalic cat with the dopamine agonist L-deprenyl.

Acromegaly was diagnosed in a 14-year-old domestic short hair cat presented for investigation and management of apparently insulin-resistant diabetes mellitus. Treatment with L-deprenyl and high doses of insulin was commenced. The L-deprenyl did not reduce the requirement for high doses of insulin and did not appear to reduce the clinical signs associated with the disease. The cat was euthanased one year after initial presentation.

Blastoma adrenal en adulto / Adrenal blastoma in an adult

El blastoma adrenal es una neoplasia excepcional de la que hay una única publicación en un niño de 21 meses. Se presenta un caso similar desarrollado en un varón de 68 años. Se revisan los criterios anatomopatológicos e inmunohistoquímicos y el diagnóstico diferencial. En conclusión, el blastoma adrenal no es un tumor exclusivo de los niños (AU)

[Acromegaly--diagnosis and treatment]. / Akromegali--diagnostikk og behandling. Norsk Neuroendokrin Interessegruppe.

BACKGROUND: Acromegaly is a rare but clinically important disease caused by growth hormone hypersecretion, usually from a pituitary adenoma. The condition is associated with increased morbidity and mortality. MATERIAL AND METHODS: The prevalence of acromegaly is estimated to be 4-6 cases per million per year. The diagnosis is based on glucose-suppressed plasma growth hormone. When the diagnosis is confirmed, MR imaging of the pituitary gland is performed. RESULTS: Standard treatment is transsphenoidal microsurgery; however, radicality is often difficult because of extensive tumour growth. Preoperative administration of somatostatin analogues may improve the surgical outcome. INTERPRETATION: We have initiated a randomized, prospective study to elucidate this adjuvant treatment. Somatostatin analouges are required in the case of postoperative activity. This treatment is without tachyphylaxis and has few side effects. Alternatively, dopamine agonists such as bromocriptine can be used, especially in mixed tumours coproducing prolactin. Newer, more specific dopamine agonists are currently being evaluated. Radiation therapy may be required in large, unresectable tumours, but the effects are slow-acting, and almost all patients develop hypopituitarism. Gamma knife radiosurgery seems promising for stopping tumour growth as well as for decreasing excessive hormone production. However long-time follow-up results are so far lacking.

Goldmann perimetry in acromegaly: a survey of 307 cases from 1951 through 1996.

OBJECTIVE: Because visual pathway lesions are a common complication of pituitary tumors, visual field examinations in patients with acromegaly were studied. Proportion and outcome of visual field defects in patients with acromegaly were evaluated. DESIGN: Large, retrospective case series. PARTICIPANTS: We reviewed 307 cases of acromegaly seen from 1951 through 1996 at a single referral center. METHODS: Kinetic visual field testing had been performed with the Goldmann perimeter, and the frequency of visual field defects and their correlation with other clinical manifestations and characteristics of the adenoma were examined. MAIN OUTCOME MEASURES: Repeat visual field examinations. RESULTS: Of the 307 patients included in the analysis, a visual field defect that could be attributed to the pituitary adenoma was observed in 62 (20.2%) during follow-up. Visual field defects were bilateral in 38 (61.3%) of these cases. Patients with visual field abnormalities were significantly younger (P = 0.04), had larger tumors (P < 0.001), had more suprasellar extensions (P < 0.001), and had higher levels of growth hormone in their serum (P = 0.04) than patients free of visual field defects. At the end of the follow-up period, visual field examination remained abnormal in 32 (10.4%). Return to a normal visual field examination after treatment was more frequently observed in patients who were less than 40 years of age at the time of diagnosis (P = 0.004). Secondary empty sella syndrome was the main cause of visual field defects after treatment. Abnormal visual field, either at the time of diagnosis or during follow-up, decreased from 27% of patients between 1951 and 1975 to 15.4% of patients between 1976 and 1996, when modern neuroimaging techniques became available. CONCLUSIONS: Endocrinologic and neuroimaging follow-up of patients with acromegaly should be accompanied by ophthalmic assessment. Factors predictive of visual field defects have been identified.

Sudden blindness from pituitary apoplexy: a report of two cases.

Two cases of acidophil adenoma of the pituitary causing sudden blindness from pituitary apoplexy are presented. The tumours had been clinically silent, without producing any symptoms of endocrine dysfunction. Radiological evidence was very conclusive. Transfrontal craniotomy with decompression resulted in quick and dramatic visual improvement. The interesting syndrome of clinical manifestations is discussed.

Acidophil stem-cell pituitary adenoma in a prepubescent female. Case report.

Acidophil stem-cell pituitary adenomas account for less than 5% of pituitary tumors. Only 15 cases have previously been reported, with a mean age of occurrence of 38.7 years. A case of this unusual tumor is reported in a prepubertal girl. Clinical symptoms included prominent behavioral disturbance with associated headache and visual disturbance. There was marked elevation of serum growth hormone concentration without clinical features of growth hormone excess, suggesting that this tumor has the capacity to excrete biologically inactive hormones. The clinical and pathological features of this unusual invasive pituitary tumor are reviewed; the age spectrum for this neoplasm must be expanded to include prepubertal children.

Pituitary apoplexy.

Three patients with symptomatic haemorrhagic necrosis and infarction of the pituitary gland are described. They showed a range of clinical presentation, diagnostic pitfalls and diversity of treatment.

[Results of surgical treatment of patients with pituitary adenomas]. / Rezul'taty khirurgicheskogo lecheniia bol'nykh s adenomami gipofiza.

The results of operations performed on 126 patients for adenoma of the hypophysis from 1954 to 1975 by the transfrontal approach are analysed. In most patients the adenoma was marked by suprasellar growth. The clinical symptoms and the specific features of the methods of examination and surgical treatment are described. Postoperative mortality was 18.2%, duration of catamnestic follow-up 5-10 years. Recurrences were found in 14% of cases. They occurred 5-8 years, on the average, after the first operation and 1.3 years after repeated operations. The necessity of early diagnosis of hypophyseal adenoma is emphasized. Modified operative techniques and substitution hormonal therapy in combination with timely diagnosis are decisive in improving the results of treatment of patients with adenoma of the hypophysis.